INFANTILE SPINAL PARALYSIS.
BY MARY P. JACOBI, M.D.
SYNONYMS.—Essential paralysis of childhood (Rilliet and Barthez); Myogenic paralysis (Bouchut); Acute fatty atrophic paralysis (Duchenne); Atrophic paralysis (Ferrier); Acute anterior poliomyelitis (Kussmaul, Erb, Seguin); Regressive paralysis (Barlow); Tephromyelitis (Charcot).
DEFINITION.—Of all the titles which have been given to the disease it is our purpose to describe, two alone may be considered irreproachable. In the present state of our knowledge it is unnecessary to argue that this disease is not essential—i.e. destitute of characteristic anatomical lesions. Neither can the theory of its myogenic origin be maintained; nor even is fatty degeneration invariably present in the paralyzed muscles. Finally, the disease cannot longer be regarded as peculiar to childhood,1 since cases in adults have been in these last years quite numerously reported2—four with autopsies demonstrating the identity of the lesion. But there are two definitions in our list of synonyms which embrace between them the most striking characteristics of the disease, yet contain no error of fact. Atrophic paralysis describes at once the two most salient symptoms; acute anterior poliomyelitis defines at once the seat and nature of the lesion, classes it with the systematic diseases3 of the spinal cord, and notes the peculiarity in the mode of invasion by which it is so remarkably distinguished from nearly all the organic diseases of this centre.
1 W. H. Barlow, On Regressive Paralysis, 1828. See Brain, April, 1879.
2 In Dec., 1873, I quoted 14 cases of adult spinal paralysis, as follows: Duchenne, 4 cases; Charcot and Petitfils, 3; Moritz Meyer, 2; Bernhardt (Archiv Psych., 1873), 1; Cumming (Dublin Quart. Journ., 1869), 1; Lucas Championnière (by Hallopeau, Archives gén., 1861), autopsy, 1; Gombault (Archives de Psych., 1873), 1; personal, 1.
In 1874, Seguin published a summary of all the foregoing cases except the last, and added 6 personal observations, also 3 from Duchenne and 1 from Hammond. In the enlarged edition of his essay in 1877, Séguin increased the list to 45—by new personal cases, 3; cases related by Frey (Berlin. Wochens., 1874), 4; cases by Erb (Arch. f. Psych. u. Nervenkrank., v.), 4; case by Cornil and Lépine (Gaz. méd., 1875), autopsy, 1; case by Soulier (Lyon méd., 1875), 1; case by D. H. Lincoln (Boston Med. and Surg. Journ., 1875), 1; case by Lemoine (Lyon méd., 1875), 1; case by George M. Beard, 1; case by Leyden (Klinik Ruckenmarks Krankheiten) Bd. iv. 1; case by Hammond (6th ed. Treatise), 4; case by Courty (Gaz. méd., 1876), 1; case by Dejerine (Arch. de Phys., 1876), 1.
To these may be added—case by Goltdammer (Berl. klin. Wochen., 1876), 1; case by Webber (Trans. Amer. Neurol. Ass. for 1875, vol. i.), autopsy, 1; case by Klose (Diss. Breslau, 1876), 1; case by Schultze (Virchow's Archiv, Bd. lxviii.; also Bd. lxxiii.), autopsy, 1; case by Bernhardt (Archiv für Psych., Bd. ix., 1879); case by Sinkler (Amer. Journ. Med. Sci., Oct., 1878), 5; case by Althaus (ibid., April, 1878), 2; case by Ross (Dis. Nerv. Syst., vol. ii. p. 139), 1—total, 57 cases.
Morton (St. Bartholomew's Hospital Reports).
Others have doubtless been published since this date, but, as they do not immediately concern our subject, need no further citation.
3 Vulpian, Leçons sur les Myelitis, 1880.
SUMMARY OF CLINICAL HISTORY.—The clinical features of an acute attack of infantile paralysis are well known. The children affected are usually between eighteen months and four years of age (Henoch). The attack is more likely to occur in summer than in winter, as Sinkler4 found that 47 out of 57 cases began between May and September, and Barlow noted 27 out of 53 in July and August.5
4 Amer. Journ. Med. Sci., April, 1875.
5 Loc. cit., p. 75. Among Sinkler's 57 cases, only 6 furnish autopsies, thus:
Case by Cornil and Lepine and case by Webber (quoted and accepted by Erb in Ziemssen's Handbuch, Bd. xi.); case by Gombault (rejected by Erb and Westphal); case by Schultze; cases by Dejerine and Lucas Championnière (quoted by Hallopeau).
The influence of heat is perhaps shown in the case related by Dyce-Duckworth in the Lancet of 1877: a child two and a half years, after exposure to great heat on a steamboat-landing, became paralyzed in all four limbs, but the paralysis was subsequently confined to the lower extremities. Coincidently, the patient became delirious; suffered from anæsthesia and temporary paralysis of the sphincters. The paralyzed muscles wasted rapidly and lost faradic contractility. Treatment by faradization was begun in a month from the date of the attack, and recovery was complete three months later.
The onset of the paralysis is either really sudden, occurring in the daytime, while the child is under competent observation, or apparently sudden, being discovered in the morning after a quiet night, the child having gone to bed in health (West); or is preceded by some hours or days of fever or of nervous symptoms, especially convulsions, or both. The paralysis is almost always at its maximum of extent and intensity when first discovered, and from this maximum begins, within a few hours or days, to retrocede. The improvement may, however, be delayed much longer. A variable number of muscles remain permanently paralyzed, and in these, within a week (thirty-six hours, according to some observers), faradic contractility is first diminished, then abolished; galvanic reaction is exaggerated, ultimately is characterized by the degeneration signs (entartungs reaction). The temperature of the paralyzed limbs falls; the muscles waste; the atrophy may rapidly become extreme. The paralysis and loss of faradic contractility are complete, however, while the atrophy is only incipient and progressing. The absence of lesions of sensibility, of visceral disturbance, of trophic lesions of the skin, or of sphincter paralysis is as characteristic of the disease as are the positive symptoms above enumerated.
In the third or chronic stage the paralyzed limbs often become contracted and deformed. At other times, and with more complete paralysis, the growth of the bones is arrested, the muscles remain flaccid, the entire limb shrivels, and dangles so loosely from its articulations that it may be dislocated by slight effort (membre de Polichinelle). The general health of the patient remains remarkably good, the intelligence clear, the disposition lively. The duration of life seems to be in nowise shortened by the paralysis. Thus, suddenness of development, intimate association of trophic, motor, and electrical disturbance, absence of cerebral or sensory lesion, peculiar localization and grouping of the permanent paralyses,—such are the salient characteristics of this remarkable disease.
SYMPTOMS IN DETAIL.—Three well-defined stages exist—the initial, the paralytic, and the chronic.
Initial Stage.—Seeligmüller6 signalizes three principal varieties in this stage. The first is characterized by fever; the second by nervous symptoms, principally convulsions, sometimes delirium or coma; in the third no symptoms either precede or accompany the local disease—"la paralysie est toute la maladie.”7
6 Gerhardt's Handbuch der Kinderkrankheiten, 1880 (separat Abdruck).
7 Rilliet et Barthez, Traité des Maladies des Enfants, ii. p. 551.
Mode of Invasion.—I have elsewhere8 described eight different modes of invasion: absolutely sudden, coming on in the daytime; morning paralysis (West), discovered after a quiet night, preceded by fever or by vomiting alone (?), or by another typical disease, especially one of the exanthemata, or, finally, by a traumatism, generally slight. An interval of time almost always elapses between the occurrence of the traumatism and the development of the paralysis—a fact which already indicates that a definitely-evolved morbid process must intervene between the two occurrences. An exception is related by Duchenne fils;9 and some apparent exceptions, in which recovery occurred rapidly, seem to belong to the temporary paralysis of Kennedy,10 more recently described again by Frey.11
8 Am. Journ. Obstet., May, 1874.
9 Archives gén., 1864. A father pulled his child from a table by the right arm, and set it rather roughly on the ground. Immediate pain, almost immediate paralysis of arm, which persisted, and was followed by atrophy of its muscles.
10 Dublin Quarterly, 1850.
11 Berlin. klin. Wochens., 1874. Frey considers these cases to be identical in nature with, though differing in severity from, anterior poliomyelitis.
PRODROMATA.—There is rarely any lengthened period of prodromata. Seeligmüller has noticed in some cases an indisposition on the part of the child to stand or walk during several weeks before the occurrence of the paralysis. He does not say whether such children were rachitical. In marked contrast with cases of cerebral paralysis is the habitual absence of generalized nervous symptoms. Thus in only 1 case of Seeligmüller's (total of 75) did the child suffer, and that during six months preceding the paralysis, from intermittent muscular contractions, and also from attacks of laryngismus stridulus.
The fever is usually of moderate severity (Seeligmüller), but sometimes extremely high (Erb)12—as much as 41° C. (Henoch),13 or 104° F. (Barlow).14 Duchenne fils observed 7 cases alleged to be entirely without fever, and Laborde counts 10 cases out of 50 as apyretic.15
12 Ziemssen's Handbuch, Bd. xi. Abh. 12.
13 Vorlesung. über Kinderkrank., 2d Aufl., 1881. Seeligmüller (Jahrb. für Kinderheilk., 1878, p. 345) quotes another case from Henoch's clinic where the fever lasted thirty-six hours, the temperature on the first evening being 39.2°, the following morning 39.0°, the second evening 39.5°; the second morning, at which date the paralysis was discovered, it was normal. The author states this to be the only case known to him in which thermometric measurements were taken.
14 Loc. cit.
15 De la Paralysie de l'Enfance.
The duration of the fever usually varies from a single night to forty-eight hours; much more rarely does it last six, eight, twelve, or fourteen days, or even, but quite exceptionally, three or four weeks. According to Duchenne, its intensity and duration increase with the age of the child, perhaps indicating greater resistance on the part of the nerve-tissues which are the seat of the morbid process of which it is symptomatic. Rarely does it last after the paralysis has once occurred, but ceases then with an abruptness which recalls the defervescence of pneumonia when the exudation process is once completed.16
16 See p. [1144] for pathogenic inferences to be drawn from this fact. Seguin (New York Med. Record, Jan. 15, 1874) seems to throw some doubt on the existence of apyretic cases; but, as Seeligmüller remarks, there is too much testimony to this possibility to render it really doubtful.
There is no proportion between the intensity of the fever and the extent of the subsequent paralysis; nor is there any marked contrast between the fever in children and that in adults in those rare cases in which the disease, instead of being subacute, is sudden as in children.
Erb considers the fever to be purely symptomatic of an inflammatory process in the spinal cord.17 But Vogt regards it rather as an essential factor in the development of a spinal lesion, and thus explains the occurrence of this in the course of febrile diseases which at the outset have no special relation to the cord.
17 Loc. cit., p. 279.
Convulsions, usually accompanied by fever, were observed in 11 of Seeligmüller's cases out of 67; Duchenne had 13 out of 70; Heine, 9 out of 86;18 thus a total of 33 cases of convulsions in 223 cases of infantile paralysis—nearly 15 per cent. The paralysis may set in after a single brief convulsion, or this may be repeated several times at variable intervals before the paralysis is definitely declared (Ross).19 The convulsive movements are apt to be particularly intense in the limbs destined to become paralyzed (Vogt).
18 Die Spinale Kinderlahmung.
19 Loc. cit., p. 107. The author is quoting Laborde.
The convulsion may be very slight—an isolated spasm of a limb or even a single group of muscles. Whether, on the other hand, it can ever be so intense that the child succumbs to it before the development of paralysis, is a question which could only be decided by repeatedly examining the cord in the cases of convulsion which have terminated fatally. In a case of Seeligmüller's the child was affected for eight days preceding the paralysis by tremblings generalized through all his muscles.
The convulsion is usually followed by a soporous or even comatose condition, or this may replace the convulsion. Delirium may take the place of either.
Special interest attaches to those cases where the paralysis develops in the course of an acute specific disease; for then becomes most plausible the suggestion of Vogt, that a fever excited by some cause remote from the spinal cord may itself become a cause of lesion in this centre. In Roger's first and most celebrated case, paraplegia developed suddenly during the course of a fatal scarlatina in a child already suffering from paralysis of the left deltoid of two months' standing.20 The scarlatina was hemorrhagic, and, as will be shown farther on, the autopsy showed traces of a hemorrhagic extravasation in the cord. Thus a double influence was presumably exerted by the scarlatina, while, moreover, the previous and recent occurrence of a deltoid paralysis indicated a morbid predisposition in the spinal cord. Of Seeligmüller's 75 cases, 1 occurred during scarlet fever, 1 with measles, 1 in the course of an erysipelas, and 1 of pneumonia.
20 Gaz. méd., 1871.
Apyretic diseases, especially of the gastro-intestinal tract (Brown-Séquard), also seem to have an influence on the development of infantile paralysis. Two of my own cases occurred during an attack of cholera infantum; another in a child who had been for several weeks in bed with a purulent conjunctivitis. Study of these varied antecedents is of interest in connection with the obscure question of the etiology of infantile paralysis. In this latter connection we will refer to them again.
Vomiting, or even the entire symptom-complex of gastric fever, not infrequently ushers in the paralysis. Fever is then usually present, but I have recorded one case of vomiting where, according to the mother's assurance, no fever at all existed.
At the moment that the symptoms of the invasion subside, and the child seems to enter upon convalescence, the terrifying discovery is made that an arm or a leg or all four limbs, or even they and the muscles of the trunk, are paralyzed.
In the severest form the child lies motionless, unable to stir hand or foot, or even a finger or toe. Yet, singularly enough, this extensive paralysis is sometimes overlooked, especially in very young children, as the immobility of the patient is attributed merely to weakness caused by previous illness. General paralysis, during at least the first few hours of the paralytic stage, is probably more common than appears from our present statistics. Not only, as has just been noted, may this condition be overlooked, but it may exist during the hours of sleep which precede the cases of morning paralysis. Seguin21 speaks as if the paralysis were at first always generalized, but this statement seems to me somewhat exaggerated. Referring merely to the statements of the parents, a considerable number of paralyses would be found limited from the beginning. Heine's third table of partial paralysis is entirely composed of cases so limited. In 16 out of the 19 cases of hemiplegia (monoplegia) the original limitation of the paralysis is also specified; similarly with 7 out of the 20 cases of paraplegia contained in the first table.
21 Loc. cit.
Paralysis of one or both lower extremities is often first detected when the child gets out of bed and attempts to walk; or in children too young to walk the flaccid immobility of the limb attracts attention as soon as they are again carried on the mother's arm. Paralysis of the upper extremities is discovered early in proportion to the liveliness recovered by the child, leading him to occupy himself with his toys as usual. In unilateral paralysis of the trunk the child will fall over to one side when placed in the sitting position; in bilateral paralysis it cannot be made to sit up at all.
From lack of competent observation during the initial stage it is really not quite certain whether any degree of paresis precedes the paralysis; but from the testimony at present accumulated the paralysis is nearly always complete when first observed. This is in striking contrast with adult spinal paralysis. In some few cases the paralysis has been observed to creep on slowly, and not reach its maximum for several days (Ross). Laborde relates a case where recovery from a first attack of paralysis was followed by two relapses in the same limbs at intervals, each ushered in by fever. After the second relapse the paralysis remained permanent.
Significance of Original Extent of Paralysis.—The question of the original distribution of the paralysis is of special interest in connection with that of the original distribution of the morbid process in the spinal cord. The real effect of the latter cannot be adequately measured by the permanent paralyses; for, as will be seen, it is not unusual to find traces of an extensively diffused process in the cord in cases of quite partial paralysis.
It is interesting to notice that certain muscles are always exempt from paralysis. With the exception of a single case of paralysis of one temporal muscle, cited by Seguin,22 the muscles of the head, eyeballs, ears, larynx, and pharynx are always exempt, as are also the diaphragm and intercostals. The arrest of the spinal lesion below the medulla explains the immunity of muscles supplied by the vagus and spinal accessory nerves. But since the cervical plexus is often involved, the constant escape of the diaphragm, innervated by the phrenic nerve which comes from this plexus, is remarkable. Still more so the immunity of the intercostal muscles, whose nerves arise in the dorsal region—a position of the cord frequently affected. This fact tends to confirm Ross's hypothesis, that the nuclei of the intercostal nerves lie in the vesicular columns of Clarke—columns confined to the dorsal region of the cord, and which are invariably found intact at autopsies of atrophic paralysis.
22 Loc. cit.
The immunity of these respiratory nerves explains the absence of the dyspnœa which is so marked in Landry's ascending paralysis. In the adult case described by Schultze and Erb23 dyspnœa was present for a short time. The disease terminated fatally twenty months from the time of invasion. In this case traces of myelitis were found extending through the dorsal region of the cord, and including not only the anterior nerves, but, to a less degree, the columns of Clarke.
23 Arch. Virch., Bd. lxviii.
The facial nerve (itself a respiratory nerve) shares the immunity of the phrenic and intercostals. In the cases in which facial paralysis has been noted the limb paralysis has been hemiplegic, as in Seeligmüller's twentieth case. A cerebral origin is then always to be at least suspected.
Barlow24 has seen 6 cases of paralysis of the facial, but the histories render a cerebral paralysis more probable in 4 out of those 6. Henoch25 gives a case of paralysis of left arm, accompanied by paralysis of corresponding facial nerve. The latter rapidly recovered, but the paralysis of the arm persisted and was followed by atrophy. Ross26 implies that the sides of the neck, face, and tongue are always at first implicated in spinal hemiplegic paralysis, but do not remain permanently affected.
24 Loc. cit.
25 Loc. cit., p. 205.
26 Loc. cit., p. 108.
That the facial should be affected while the other medullary nerves escape probably depends on the more anterior position of its nucleus.
The regression of the original paralysis is characteristic, indeed almost pathognomonic, of the disease. It is on this account that Barlow has proposed the name regressive paralysis.27 This author quotes the case of a boy who at five months was affected with a universal paralysis, even affecting the neck, but entirely recovered except in the extensor longus digitorum of the foot. This improvement constitutes a second apparent convalescence, as deceptive as that which immediately succeeds the pyrexia. Only in rare cases do all the muscles at first paralyzed remain so permanently (Seeligmüller); nor, on the other hand, do all entirely recover (temporary paralysis of Kennedy and Frey). Even when an entire limb appears to be paralyzed, careful examination will usually detect certain muscles that retain their faradic contractility. Thus the order of frequency of paralysis in the different limbs must be distinguished from that observed for different muscles.
27 Brit. Med. Journ., 1882.
Duchenne fils28 and Seeligmüller29 have tabulated, for lists of 62 and 75 cases respectively, the general locality of the permanent paralyses in their order of frequency. The cases of monoplegia are by far the most numerous. Thus in the table quoted below there are 97; in Heine's tables (86 cases), 47; in Sinkler's tables (86 cases), 29—total, 173 from a total of 309, or nearly one-half:
| Duchenne. | Seeligmüller. | Total. | ||
| Left lower extremity | 7 | 27 | 34 | |
| Right lower extremity | 25 | 15 | 40 | |
| Right upper extremity Left upper extremity | 10 | 13 | 9 4 | 23 |
| All four extremities | 5 | 2 | 7 | |
| Both upper extremities | 2 | 1 | 3 | |
| Both lower extremities | 9 | 14 | 23 | |
| Left over and under extremity | 1 | 1 | 2 | |
| Right over and under extremity | 0 | 1 | 1 | |
| Right over and left under extremity | 2 | 1 | 3 | |
| Muscles of trunk and abdomen | 1 | 0 | 1 | |
| 62 | 75 | 137 | ||
This limitation is all the more noteworthy when compared with the frequency of general paralysis at the outset.
28 Archives gén., 1864.
29 Jahrbuch der Kinderheilkunde, N. H. xii. pp. 338-343.
The next peculiarity is the great preponderance of paralysis of the lower over that of the upper extremities. This is noticeable even in the monoplegias. In Sinkler's cases only two of these affected an arm. But in bilateral paralysis the predilection is still more remarkable, paraplegia of the lower extremities being among the most frequent, paraplegia cervicalis the rarest, form of paralysis. This is not because a lesion situated in the cervical spinal cord must interfere with the motor tract going toward the lumbar, and hence nearly always paralyze all four extremities, if any. Because when this does happen the upper extremities alone exhibit the atrophic changes characteristic of anterior poliomyelitis: the lower, though paralyzed, do not atrophy and retain their faradic contractility. Heine denied the existence of paraplegia cervicalis. But in the table of Duchenne-Seeligmüller 3 cases are recorded; Rosenthal30 relates 1; Lockhart Clarke, 1.31 This is the somewhat famous case, described by Clarke as a progressive muscular atrophy, which contributed one of the earlier autopsies.
30 Klinik der Nervenkrankheiten, 2 Aufl. p. 413.
31 Med.-Chir. Trans., li. p. 219.
Seeligmüller's case32 is remarkable in several respects. The paralysis, occurring after a brief fever in a child seventeen months old, exclusively attacked the two arms at the moment of invasion, and never retreated from them, thus offering a double exception to the usual rule. At four rears of age the arms were much atrophied, and faradic contractility was lost in their muscles, the legs being sturdily developed. Nevertheless, the child constantly fell in walking, because, observes Seeligmüller, he was unable to balance himself with his arms, as is habitual with little children learning to walk.
32 Jahrbuch, loc. cit., p. 349.
The hemiplegic variety of paralysis is again very rare. The Duchenne-Seeligmüller table contains (out of 137) 3 cases; Sinkler records (out of 86) 4; West, 5; Heine (out of 86), 1; Leyden, 1; Duchenne, 1. West's 5 cases all present certain peculiarities, at least unusual in spinal paralysis. In 2 the paralysis came out gradually; in 1 succeeded to remittent fever (pigmentary embolism?); in 1 was preceded by heaviness of the head for several days; and in 1 the leg was paralyzed fourteen days after the arm.33 It is probable that in almost all, if not in all cases, hemiplegic spinal paralysis is the residue of a paralysis originally generalized to all four limbs, if only for a few hours.34
33 In three cases of hemiplegia observed by myself, and previously diagnosed as spinal paralysis by other physicians, I doubted the diagnosis from the coincidence of unusual cerebral symptoms. In the first case the hemiplegia appeared after coma, during cerebro-spinal meningitis; in the second, after a violent convulsion the face was drawn to the opposite side, and the patient, a child of seven, remained for a month in a state of intense maniacal excitement. In a third case, developed during convalescence from scarlet fever, the hemiplegia was preceded during two days by hemiparesis, and accompanied for a year by complete aphasia. Finally, in these cases faradic contractility persisted in the paralyzed limbs (Am. Journ. Obstet., May, 1874).
34 Seeligmüller relates one case where hemiplegia, including the facial nerve, was observed in two days from the beginning of the fever.
The question of hemiplegia is closely connected with that of paralysis of the facial nerve, inasmuch as the existence of the latter often serves to suggest a cerebral paralysis—a suggestion confirmed later by the absence of atrophy and of characteristic electrical reactions. However, in some cases of undoubted spinal paralysis the facial does really seem to have become involved. Thus in the case just quoted from Seeligmüller (Case 20 of his table) Henoch35 relates a case of paralysis of the left facial coinciding with paralysis of the left arm. Rapid recovery from facial paralysis: arm atrophied. Barlow36 records temporary facial paralysis in six cases, but only two of these seem to be really spinal. Such temporary paralysis is not altogether infrequent in the poliomyelitis anterior of adults (Sinkler, Seguin). Ross37 implies that the sides of the neck, face, and tongue are always implicated at first in hemiplegic spinal paralysis, but do not remain so.
35 Loc. cit., p. 203.
36 Loc. cit., p. 76.
37 Loc. cit., p. 108.
Crossed paralysis is extremely rare. There are 3 cases in the Duchenne-Seeligmüller table; Leyden38 has one. But paraplegia of the lower extremities, coinciding with paralysis of one upper extremity, is by no means so rare, especially as a residual paralysis.
38 Archiv Psychiatrie, Bd. vi.
Finally, as in cerebral paralysis, the muscles of the trunk, though often paralyzed at the outset, rarely remain so in children—much more often in adults. Eulenburg39 relates one interesting case of complete paralysis and atrophy of the extensors of the back. Even the interspinous muscles were involved, as shown by the divergence of the spinous processes. The paralysis was observed in a girl of fifteen affected since the age of three, and was completely cured in five months by daily faradizations of ten minutes each, and two gymnastic séances, each lasting two hours.
39 Arch. Virch., Bd. xvii., 1859.
Birdsall40 has described one case of unilateral paralysis of the abdominal muscles.
40 Journal of Nervous Diseases.
Study of the precise combinations of the muscles paralyzed has recently acquired peculiar interest in connection with the localization in the spinal cord of the motor or trophic nuclei of their nerves.41 Several facts have been ascertained: 1st, that, in notable contrast with progressive muscular atrophy, atrophic paralysis tends to involve definite groups of muscles; 2d, that this grouping is not effected in accordance with the proximity to each other of the muscles on the limb, but with their functional association. Remak affirms that Charles Bell had already called attention to the fact that in cases of local muscular paralysis of the extremities the paralysis does not spread by muscular continuity, but in accordance with the functional association of muscles. Thus, paralysis of the thumb is more often associated with that of the forearm than with paralysis of the other muscles of the hand. 3d. From such grouping may often be inferred a different localization of certain nerve-nuclei than would be supposed from the position of the muscles alone. 4th. That the fibres contained in a single nerve-trunk, but distributed to different muscles, probably separate from each other within the cord, to be there distributed to variously-situated nuclei.42
41 Ernst Remak, “Localis. der Atroph. lahmung,” Archiv f. Psych., ix., 1879; Ferrier, Brain, vol. iv. No. 3; also, Proceedings Royal Society, No. 212, p. 12.
42 The theory of course assumes the truth of the demonstration by which atrophic paralysis is rendered symptomatic of disease of the spinal cord, and the nutrition of a muscle dependent on the integrity of the muscles of origin of its nerves.
In the arm two mutually correlative cases are observed: (a) Immunity of the supinator longus during paralysis of the forearm muscles; (b) paralysis of the supinator in association with paralysis of the deltoid, biceps, and brachialis anticus. The latter constitutes Remak's upper-arm type of localization, and is exhibited in his first case.43
43 Loc. cit.; also, cases 1st and 2d by Ferrier, in which, however, other shoulder-muscles were involved.
Ferrier has experimentally confirmed this muscular association by means of isolated irritation of the fourth cervical nerve, which threw into contraction the supinator longus, together with the deltoid, biceps, and brachialis internus. At the same time, in the experiment the flexors and extensors of the wrist were excited, while in the special form of paralysis noted they were exempt. This electrical method is a less precise mode of analysis than the pathological, for the double reason that (a) fibres whose nuclei are dissociated may pass together in the same root; (b) because the same muscles receive fibres from more than one root: thus the flexors and extensors of the wrist from the fifth as well as the fourth cervical. Thus when the nucleus of the latter was destroyed paralysis would be averted by means of the fibres coming from the fifth root.
The experiment and the pathological observation, however, concur in indicating that the fibres innervating the supinator longus, though passing to it in the path afforded by the radial nerve, afterward ascend in the cord to a ganglionic nucleus in close proximity to those of the upper-arm muscles specified—liable, therefore, to be affected with them. The purpose effected by such association is the supination of the arm.
It is excitation of the fourth root in Ferrier's experiment which gives results most closely corresponding to Remak's observations. Excitations of the fifth and sixth root reveal other combinations, which Ferrier has found realized in adult's spinal paralysis. Thus in his second case, in addition to the group of muscles already mentioned, the rhomboid, infraspinatus, and serratus magnus were paralyzed, the last muscle indicating complication with the fifth root. In the third and fourth cases muscles supplied from the sixth root were joined to those innervated by the fourth and fifth—namely, the pectoralis major and latissimus dorsi.
In this upper-arm type the muscles affected are supplied by three different nerve-stems—the axillary, musculo-cutaneous, and the radial. In the forearm type the most common variety consists in paralysis of the extensors of the wrist, thus exactly imitating lead palsy.44 The supinator remains intact, the intrinsic muscles of the hand are sometimes intact, sometimes paralyzed. Sometimes, however, the extensors are relatively intact; the interossei are atrophied, and a clawed hand, resembling that characteristic of cervical hypertrophic pachymeningitis, is developed.45
44 It is on this fact, indeed, that Remak has been led to argue the spinal nature of saturnine paralysis (“Zur Pathogenie der Blei lahmung,” Archiv für Psych., Bd. vi., 1876).
45 The march of this disease, together with that of tabes dorsalis, furnishes data for localizing the nervous nucleus for the wrist extensors. In both diseases the lesion is ascending: in tabes disturbance of sensibility occurs first in the distribution of the sensory fibres of the ulnar nerve; in cervical pachymeningitis the flexors and intrinsic muscles of the hand are first paralyzed. Hence it is to be inferred that the central nucleus for the latter muscles lies in the lower, that for the extensor muscles in the middle, segment of the cervical enlargement of the cord.
The much greater frequency of extensor paralysis in the forearm type of anterior poliomyelitis indicates that the lesion of this disease begins about the middle of the cervical enlargement (see note).
The foregoing groupings have been made out almost entirely from cases of adult spinal paralysis or else of lead palsy. In the lower extremity it is much more difficult to establish such definite muscular association. Certain laws, however, can be made out: 1st. The liability to paralysis increases from the thigh toward the foot; thus, the muscles moving the thigh on the pelvis are the least liable to paralysis, then those moving the leg on the thigh, while the muscles moving the foot and leg and thigh are the most frequently paralyzed of any in the body. 2d. Of the upper thigh-muscles, the glutæi are not infrequently paralyzed, the ilio-psoas hardly ever, the adductors rarely except in total paralysis. 3d. Of the muscles moving the leg on the thigh, the quadriceps extensor is very frequently paralyzed—the most often, indeed, after the foot-muscles: the sartorius is almost always exempt; the liability of the hamstring muscles corresponds to that of the thigh adductors. 4th. At the foot the tibialis anticus often suffers from isolated paralysis, sharing in this respect the fate of the deltoid in the upper extremity—a fact already noticed by Duchenne. On the other hand, (5th) the tibialis anticus often remains intact while the other muscles supplied by the perineal nerve, the perineus longus and brevis, are completely paralyzed.46
46 Thus Buzzard relates a case of paralysis involving the quadriceps extensor and peroneal muscles, while the anterior tibial were intact.
The remarkable contrast in the morbid susceptibility of the quadriceps on the one hand, and the sartorius on the other, suggests dissociations of their nuclei. Remak relates one interesting case (Obs. 13) where the sartorius was paralyzed—coincidently with the quadriceps, it is true, but also with partial paralysis of the ilio-psoas muscle, which is as rarely attacked as the sartorius itself. The two facts, taken together, would indicate that the nucleus of the sartorius lies high in the lumbar enlargement, in proximity to that of the ileo-psoas. The inference, continues Remak, is reinforced by functional considerations, since the sartorius, obliquely flexing the leg on the thigh, is generally in action at the moment that the psoas flexes the thigh on the pelvis.
Again: according to Remak the tibialis anticus is generally paralyzed together with the quadriceps extensor, although supplied by a different nerve.47 And this should be expected from the necessity of exciting dorsal flexion of the foot by means of the tibialis anticus at the moment of extending the leg for the act of walking.48
47 Obs. 14, 15, 16, 17, from Remak's essay.
48 At the moment that the foot is thus flexed, however, to allow the leg to be swung forward, the thigh and leg are both slightly flexed.
Ferrier, from his experiments on the roots of the lumbar plexus, is inclined to doubt this association of the tibialis anticus with the quadriceps, and he adduces Buzzard's case, already quoted, to show coincident paralysis of the quadriceps and peroneal muscles. It is not improbable, however, that fibres associated together in nerve-roots may again diverge in the cord, and thus the discrepancy would be explained.
DIAGNOSIS OF SPECIAL PARALYSIS.—Paralysis of isolated muscles may sometimes be concealed by the vicarious action of their synergists: thus of the extensor communis for the tibialis anticus. Paralysis of both legs and feet may even be partly concealed by the energy of the thigh-muscles, which, using the paralyzed segments of the limbs as inert supports, succeeds in effecting locomotion.49 On the other hand, in limbs apparently abandoned to total paralysis persevering search will often discover some muscles or parts of muscles which respond to faradic electricity: these must be considered as susceptible of ultimate recovery.
49 Thus in Cornil's famous case, Soc Biol., 1863.
The following table sums up some special diagnostic marks for the different paralyses50 afforded by the position of the limb and loss of movements:
Upper Extremity. Deltoid. Absence of deformity, which is averted by weight of arm. Inability to raise arm. Sometimes subluxation. Frequent association with paralysis, biceps, brachialis anticus, and supinator longus.
Lower Extremity. Ilio-psoas. Rare except with total paralysis. Associated with paralysis, sartorius. Loss of flexion of thigh. Limb extended (if glutæi intact).
Glutæi. Thigh adducted. Outward rotation lost. Lordosis on standing. Frequent association with paralysis of extensors of back.
Quadriceps extensor. Flexion and adducting of leg (if hamstrings intact). Loss of extension of leg. Frequent association with paralysis of tibialis anticus.
Tibialis anticus. Often concealed if extensor communis intact. If both paralyzed, then fall of point of foot in equinus. Dragging point of foot on ground in walking. Big toe in dorsal flexion (if extensor pollicis intact). The tendons prominent. Hollow sole of foot (if perineus longus intact).
Extensor communis. Nearly always associated with that of tibialis anticus. Toes in forced flexion.
Peroneus longus. Sole of foot flattened. Point turned inward. Internal border elevated.
Sural muscles. Heel depressed. Foot in dorsal flexion (calcaneus). Sole hollowed if perineus longus intact; flattened if paralyzed. Point turned outward (calcaneo-valgus).
Extensors of back. Lordosis on standing. Projection backward of shoulders. Plumb-line falls behind sacrum (unilateral). Trunk curved to side. Trunk cannot be moved toward paralyzed side.
Abdominal muscles. Lordosis, without projection backward of shoulders.
50 See Duchenne, loc. cit., and also Roth, On Paralysis in Infancy, London, 1869.
After the paralysis the most remarkable symptom of anterior poliomyelitis is the rapid wasting of the paralyzed muscles. The atrophy begins within a week after the paralysis, and its progress is even more rapid than that following the section of a nerve. Sometimes all the flesh on a limb is shrivelled down to the bone; at other times the muscular atrophy is concealed by an abnormal development of fat, constituting a pseudo-hypertrophy. When all the muscles surrounding a joint are equally paralyzed and atrophied, no deformity develops,51 unless, indeed, the segment of a limb is used by means of the non-paralyzed proximate segment. In this case deformities may be produced by the effect of weight quite irrespective of muscular action, or in directions opposed to what we should expect from that.
51 Except talipes equinus.
The weight of the limb or a portion of it, by stretching paralyzed muscles, often aggravates their atrophy. This is most likely to occur with the paralyzed deltoid when the arm is unsupported, and with the anterior tibial muscles when the foot is allowed to drop.
Muscular atrophy occurs in the spinal paralysis of adults as well as in children; but in the latter alone does the atrophy extend to the bones and cartilages, tendons, fascia, ligaments, and blood-vessels. The osseous projections to which the muscles are attached waste; so do the epiphyses.52 The long bones are thinner and shorter, the foot is shorter, and the hand is shortened in paralysis of the upper extremity, even where this is limited to the upper arm, and the forearm is scarcely affected (Seeligmüller).
52 Seeligmüller, Centralbl. f. Chirug., No. 29, 1879.
In exceptional cases the limb may become even elongated from passive extension of the ligaments of the articulation. The bones may become soft and flexible, and break if pressure be applied.53
53 Ch. Salomon, “Des Lesions osseuses et articulaires lieés aux Maladies du Système nerveux,” Revue mensuelle, No. 8, 1878.
Atrophy of the bones stands in no fixed relation to that of the muscles, now exceeding, now falling short of that in intensity. This naturally progresses more slowly; still, within seven or eight months there may be a centimeter of difference between two limbs.
In marked contrast with this profound trophic disturbance of the bones is the intact nutrition of the skin. The absence of decubitus is indeed an important diagnostic mark from ordinary myelitis. The subcutaneous fat, however, wastes so completely that the skin seems to be closely adherent to the subjacent tissues, and cannot be pinched up into folds.
The temperature of the skin always falls; the limb is perceptibly colder to the touch than its fellow, and is often bluish and cyanotic. Heine has observed that the temperature diminishes gradually from the centre to the periphery, and at the coldest point may sink to 14° R.54 Hammond relates a case where the local temperature was 75° in an atmosphere of 72°. The author says that exact measurements of surface temperature should be taken with Lombard's differential calorimeter, especially when convalescence is expected, as then a rise of temperature, however slight, is of most favorable augury.
54 Loc. cit., p. 16. This is not a difference of 14 degrees between the sound and paralyzed limbs, as is erroneously quoted by Seeligmüller (loc. cit., p. 67).
General factors contribute to the fall of temperature: diminished blood-supply from shrinkage of blood-vessels, or even atrophy of a certain number among these; loss of nerve-influence upon the oxidation processes; loss of muscular contractions, which should attract an afflux of blood. Among these factors the loss of nerve-supply is probably the most important, since the others exist in cerebral paralysis without causing the remarkable coldness characteristic of anterior poliomyelitis.
The atrophy of the blood-vessels is not always confined to the terminal twigs. The entire iliac artery, and even the lower part of the aorta, have been found markedly diminished in calibre.55 This shrinkage is unaccompanied by any change in the walls of the blood-vessels: it is a simple arrest of development. It strikingly illustrates the dependence of the blood-vessels on the vascular demands of the tissues they are destined to supply.
55 Charcot and Joffroy, Archives de Phys., 1870, case by Séguin, loc. cit., p. 9.
The changes which take place in the electrical reactions of the paralyzed limbs rank in importance with their paralysis and their atrophy. They serve to establish the diagnosis, to decide, to a large extent, the prognosis, and to measure the degree of nervo-muscular degeneration.
It is well known that the early diminution, and even entire loss, of faradic contractility was first emphasized by Duchenne as pathognomonic of infantile spinal paralysis. Contractility is diminished in from three to five days after the occurrence of the paralysis, and by the end of a week is completely lost in those muscles in which the paralysis is to be permanent. The muscles which recover spontaneously during the period of early regression recover their faradic with their voluntary contractility. In others, persistently but less profoundly paralyzed and susceptible of cure, the faradic contractility remains simply diminished and in unequal degrees. Progress to recovery under treatment is usually marked by progressive increase in the faradic response; but sometimes the power of voluntary contraction is fully regained, while the faradic response is still permanently lessened. The loss of faradic contractility is more complete and permanent in muscles irretrievably paralyzed by anterior poliomyelitis than in any other disease. These laws have been generally accepted by late observers.56
56 Simon disputes their validity, and declares that the importance of electricity in the diagnosis of spinal paralysis may easily be exaggerated (Union médicale, 7, 28, p. 942, 1879).
In 1868, Salomon discovered that muscles in which faradic contractility had been completely lost were nevertheless capable of contracting under the stimulus of galvanism—that this contraction is exaggerated, and sometimes occurs at the opening as well as at the closing of the circuit.57 The author remarks that the persistence of galvanic reaction after the complete loss of faradic contractility is completely analogous to an observation of Brucke's on muscles poisoned by woorara,58 where the intramuscular termination of the nerve is paralyzed. It is to be inferred, therefore, in both cases that the muscular contraction results from direct irritation of the muscle, and implies the entire loss of influence from the nerve.
57 Jahrb. f. Kinderheilkunde, N. F. i., 1868. According to Erb (loc. cit., p. 984) and to Ross (loc. cit., p. 111), Salomon was the first to make this observation. Seguin, however, attributes priority to Lobb (Lond. Med. Times and Gaz., 1863), to Hammond (New York Med. Journal, 1865), and to J. Netten Radcliffe between 1863 and 1865. These dates precede that of the publication of Salomon's paper, but the latter seems to have been written without knowledge of earlier observations. (See also Onimus, Soc. de Biol., 1878, who argues that muscle-termination of nerve is partly destroyed.)
58 “Ueber den Einfluss der Stromes dauer auf die Elektrische Bewegung der Muskeln,” Sitzber. d. k. Akad. d. Wissensch. in Wien, 1867, Bd. lxi., quoted by Salomon, loc. cit., p. 388.
Erb has greatly extended these observations, and shown that the galvanic reactions of paralyzed muscles indicate their structural degeneration, and are identical with those observed after section of a peripheric nerve. There are three characteristic peculiarities in the contractions thus obtained: 1st, they are slow, tonic, long drawn out; 2d, they are more painful than in normal muscles submitted to an equal amount of electricity; 3d, in complete degeneration the contraction obtained at anode closure equals or exceeds in intensity that excited by cathode closure [AnSZ = or > KSZ]. The excitability of the muscle to the galvanic current remains increased for several months, then gradually diminishes, and finally falls below normal. The qualitative alterations persist somewhat longer: finally, the muscle fails altogether to contract.
Spinal paralysis differs markedly from progressive muscular atrophy in the absence of constant correlation between the degree of paralysis or atrophy and of electrical changes.
The last positive symptom to be noted in the paralytic stage of infantile paralysis is the diminution and ultimate loss of reflex excitability. This is correlative in time and extent with the loss of faradic contractility. This seems to be an exception to the usual rule, which associates loss of tendon reflex with lesion of the posterior columns or nerve-roots. This is a proof that interruption of the reflex arc at any point suffices to abolish the tendon phenomena.59
59 Buzzard tested the tendon reflex in the zygomaticus major in a patient in whom the sensory branch of the fifth nerve had been stretched, and therefore, to a certain extent insulted. The reflex response was decidedly lower than on the opposite side (Lancet, Nov. 27, 1880).
Negative Symptoms.—The negative symptoms of atrophic paralysis are as important for the diagnosis and pathogeny as are the positive characters, which have now been sufficiently detailed. The absence of decubitus or other nutritive lesions of the skin has been already mentioned. The absence of anæsthesia, or, as a rule, of any marked degree of hyperæsthesia, is most important as indicating immunity of the sensory tracts in the cord. Some diffused hyperæsthesia is sometimes noted during the febrile stage: pain is by no means rare in adults. But in children this is altogether absent, or else slight and transitory. On the other hand, the complete preservation of sensibility constitutes, in children, a serious obstacle to electrical investigation.
After subsidence of the cerebral symptoms, if any, of the initial stage, the functions of the brain are always intact60 and the disposition of the children apt to be remarkably lively. The general health is often remarkable for its vigor. The worst, because the most neglected, cases are naturally most often seen among the poor: the ranks of professional beggars are largely recruited from among the victims of infantile paralysis.
60 Practically, it may often be of importance for the physician to ascertain that an intellectual enfeeblement, or even idiocy, existing at the time of examination had preceded the onset of the paralysis by months or years.
The chronic stage is marked by the development of a new set of symptoms—contractions of certain muscles surrounding one or more joints and deformed positions of the limbs. These symptoms do not always appear. If all the muscles surrounding a joint are completely paralyzed and extensively atrophied, and if no weight is imposed on the limb by the action of a non-paralyzed upper segment, and if the paralyzed segment be so supported that its own weight does not approximate the insertion-points of muscles, and thus cause their passive retraction, then there is no deformity, but a dangling limb, a membre de Polichinelle.
Laborde states that contractions appeared at the earliest about two months after the paralysis. Seeligmüller, however, has seen pes equinus and pes calcaneus develop in four weeks. The date is partly, at least, determined by the time at which the children try to walk or otherwise to use the paralyzed limbs; and the deformities are very much more marked in the lower extremities, proportioned to the much greater weight which they are obliged to sustain.
All varieties of club-foot, and most frequently equino-varus, knock-knees, rigid flexions at the knee and hip, cyphosis, lordosis, and colossal scoliosis may develop as manifold consequences of atrophic paralysis. That subluxation of the humerus and the claw-hand may occur in the upper extremities has already been mentioned.
In Seeligmüller's 75 cases, 53, or 71 per cent., exhibited some kind of deformity. Among these, 43, or 56 per cent., were of the foot; 6 were cases of subluxated humeri; 5, easily-reducible luxation of the fingers.
The following table contains a summary of the deformities observed as a consequence of atrophic paralysis. They are distinguishable from congenital deformities dependent on altered relations of articular surfaces through defective development61 by being easily reducible. This remark especially applies to paralytic club-foot:
| Foot. | Equinus. Equino-varus (varus hardly ever alone). Calcaneus. Calcaneo-valgus. Valgus. |
| Knee. | Genu-recurvation. Genu-incurvation. Permanent flexion. |
| Hip. | Luxation. Permanent flexion. Permanent adduction. |
| Hand. | Flexion fingers or wrist (rare). Extension of wrist. Claw-hand. |
| Elbow. | No deformity (Seeligmüller). |
| Shoulder. | Subluxation humeri. |
| Trunk. | Dorsal scoliosis. Lateral incurvation lumbar region. Cyphosis. Lordosis with backward projection of shoulders. Lordosis without backward projection of shoulders. |
61 Volkmann's Handbuch, Billroth und Pitha.
Mechanism of Deformities.—From what has been said on the cases in which deformities are absent it is evident that one at least of three conditions are required for their production: the paralysis must be unequally distributed in the muscles surrounding a joint; pressure must be exerted by the weight of the body or traction by the weight of the limb; effort must be made to utilize the maimed part of the limb by means of other parts, or even by the muscles of the trunk.
The share taken by these different factors in the production of deformities has been differently estimated by different observers. The French surgeon Delpech was the first to explain the phenomenon on the theory of muscular antagonism. The same theory has been most minutely elaborated by Duchenne.62 According to it, the intact or less paralyzed muscles, in virtue of their tonus, constantly tend to draw the segment of the limb on which they act in a direction opposed to that in which it should be drawn by the paralyzed muscles. Since this action is unantagonized, its influence persists; the insertion-points of the contracting muscle being permanently approximated, the nutrition of the muscle is modified: it grows shorter (adapted atrophy). There results finally shortening and retraction of the muscles on one side of the joint, over-stretching of those on the other.
62 De l'Électrisation localisée, 1861.
Duchenne used to illustrate this theory by means of a skeleton supplied with artificial muscles, whose successive section would cause the appearance of the corresponding deformity. Werner63 first protested against this theory, and the protest has been further developed and a different theory built up through the efforts of Hueter64 and Volkmann.65 The latter observes that the muscles and ligaments surrounding a joint normally receive a large amount of the weight falling upon its articular surfaces. Removal of this elastic resistance exposes these surfaces to the full force of the pressure, and thence to almost certain danger of deformity—a danger, therefore, always incurred after paralysis of the muscles. Thus, the weight of the body, pressing, unresisted, on the arch of the foot, is able to displace the bones of the arch from their normal relations and completely flatten the arch.
63 Reform der Orthopædie, 1845.
64 Gelenkkrankheiten.
65 Sammlung klin. Vort., No. 1.
A position of ease is that in which the movement of the joint has been pushed as far as possible until limited by the passive resistance of the ligaments or the conformation of the articular surfaces. The weight of the body must then be so placed that the line of gravity falls on the side of the open angle, while the limiting bands stretch across the base. Thus, a tired man sits with a curved back; the muscles which may extend the spinal column in a straight line are relaxed; the column falls forward until arrested by the anterior vertebral ligaments. Thus, in standing at ease the thigh presses against the leg, so as to form a wide angle open anteriorly. When the quadriceps extensor is paralyzed, this position is inevitable and exaggerated, since the force which might counteract it, contraction of the thigh extensor, has been removed.
Formerly, the rôle of muscles in this elastic resistance was under-estimated and that of ligaments exaggerated.
CRITICISM OF THE THEORY OF MUSCULAR ANTAGONISM.—Three considerations have been urged in objection to the theory of muscular antagonism: First, deformities may develop even in limbs totally paralyzed, provided these limbs be subjected to weight and pressure. Pes equinus, the most common form of paralytic club-foot, develops with total paralysis of the muscles of the leg where the child does not walk, but is carried on the arms of a nurse with its foot dangling. The part of the foot anterior to the ankle-joint being longer and heavier than that behind, the point falls; the tendo Achillis is passively shortened, and by nutritive adaptation to this position may become permanently retracted. Long persistence in this position accustoms the dorsal surface of the bones to a less degree of pressure than the plantar surface: as a consequence, the growth of the bone becomes more active above, while it is arrested below; the arch of the foot is increased until the sole is curved into a deep hollow; and the plantar aponeurosis is correspondingly shortened.
Volkmann relates a case where this same deformity appeared without the least paralysis, but simply from prolonged passive extension of the feet in bed. The patient was an adult, and suffered from a severe typhoid with a double relapse. After recovering from the fever a year of orthopædic treatment was required to restore the feet to their normal position.
The second objection is the absence of any proof of such constant tonus in the muscles as may be sufficiently powerful to determine the position of a limb. Such tonus exists in the involuntary muscles, especially in those of the blood-vessels, but there is no evidence that it exists in the voluntary muscles. To this Seeligmüller has replied by admitting the objection to the theory as thus proposed, but substituting the more plausible influence of repeated contractions on the part of the non-paralyzed muscles. Each contraction draws the limb in a certain direction, and there it tends to remain, because there is nothing to antagonize the force which it has obeyed.
The third objection is that examination of individual cases not infrequently shows displacements in directions opposed to that which should be determined by muscular antagonism. Volkmann has especially illustrated the latter assertion by the mechanism of genu-recurvation. In paralysis of the quadriceps extensor of the tibia the weight of the body is exercised, not merely from above downward, but from without inward, falling, therefore, on the inner malleolus. It thus tends to press the anterior part of the foot outward,66 and a valgus finally complicates the calcaneus. Seeligmüller, however, quotes two cases of pes calcaneus developed in children who had never walked: in one paralysis of the sural muscles had occurred at the age of four weeks, and the other case was observed at the age of fourteen weeks. Seeligmüller remarks that only early examination of the faradic contractility of a group of paralyzed muscles can decide whether any among them preponderate during a time sufficient to fix the limb in a vicious position. Thus in one case of pes calcaneus he found two years after the occurrence of the paralysis that some contractility still persisted in the dorsal flexors, but six months later this had quite disappeared. Had the examination then been made for the first time when all the muscles were equally paralyzed, it would have seemed impossible to explain the deformity by muscular antagonism.
66 Sayre asserts that lateral rotation cannot take place at the ankle-joint, but at the medio-tarsal articulation. Hueter also refers pes varus and pes valgus to the talo-tarsal articulation.
RELATION OF WEIGHT AND MUSCULAR FORCES.—The influences of weight and of muscular action sometimes concur, sometimes are opposed to each other. Thus, the weight of the foot alone always tends to produce equino-varus; it acts therefore to intensify the action of the sural muscles when the anterior tibial are paralyzed, but to diminish the influence of paralysis of the gastrocnemius when the foot is being drawn into dorsal flexion. Hence one reason for the comparative rarity of pes calcaneus.
Paralytic deformities at the hip and knee are much rarer than those of the foot. At the hip this immunity is partly due to the relative rarity of paralysis in the muscles surrounding the joint—still more to the fact that the weight of the limb tends to correct excessive flexions. These are therefore more likely to occur in children allowed to remain in bed than in those who are encouraged to walk by means of suitable apparatus. The use of crutches, however, favors the development of deformity, because, since with paralysis of the thigh- or leg-muscles pes equinus nearly always exists, the thigh is unduly lengthened. To palliate this inconvenience the patient instinctively flexes the knee or hip, or both, and the position tends to become permanent.
When the flexion is rigid and extension becomes impossible, the gastrocnemii are relaxed until they lose their power of tension, and thence of fixing or raising the heel. Further, as by the flexion the limb is moved in front of the body, it is necessary to project the body forward again over the support. Hence a lordosis is developed, to be distinguished from that caused by paralysis of the vertebral extensors (a) by the rigid flexion of the thigh; (b) by the facility with which the patient can extend the back as soon as he is placed in a sitting position.
Although the quadriceps extensor is so frequently paralyzed, rigid flexion at the knee—such as on the theory of muscular antagonism might be expected from the action of the hamstring muscles—is very rare. As already observed, it occurs, if at all, in neglected children allowed to lie or sit with the leg partly flexed. In those who attempt to walk the leg is not flexed, but forced into hyper-extension by the following mechanism: The muscles inserted on the upper part of the thigh swing the leg forward like a passive support. Afterward the body bends forward over the support, and its weight, pressing from above downward and from before backward, and pressing the articular surfaces of the joint together, forces the head of the tibia backward until the movement is checked by the posterior ligaments. The deformity is the same whether the quadriceps or one or all the muscles surrounding a joint are paralyzed (Volkmann).
DISLOCATIONS.—Dangling limbs are, however, much more frequently the consequence of total paralysis, with extraordinary relaxation of the ligaments of the joint permitting dislocation. Reclus67 has published several such cases. Verneuil has even suggested that congenital luxation of the hip-joint always depends on an intra-uterine spinal paralysis.68<
67 Revue mensuelle de Méd. et de Chir., Mars, 1878.
68 Quoted from Seeligmüller.
In the upper extremities rigid contractions are much less frequent, even in proportion to the number of paralyses. Seeligmüller has seen five cases of reducible flexion of the fingers, and one of permanent extension of the wrist and fingers, associated with paralysis of all the flexors. In this case, if the arms were so suspended that the hand hung freely, its weight gradually overcame the action of the extensors and the fingers fell into flexion. Upon any attempt at exertion the hyperextension was reproduced.
DEFORMITIES OF THE TRUNK.—Scoliosis will be caused when, with unilateral paralysis of the extensors of the vertical column, the lower part of the trunk is drawn to the non-paralyzed side, and the upper half is bent over the paralyzed side in order to restore the balance. In bilateral paralysis of the extensors both shoulders are projected backward, so that a plumb-line dropped from between them falls behind the sacrum, and lordosis is developed, although the lumbar column is not projected forward. The same form of lordosis occurs when the glutæi are paralyzed. When, however, the abdominal muscles are paralyzed, the lumbar column is really projected forward, and then a plumb-line dropped from the shoulders passes over the sacrum.69
69 Duchenne, loc. cit., 1861.
PARALYSIS OF THE ABDOMINAL MUSCLES.—Unilateral paralysis of the extensors of the back is often difficult to detect in young children. Seeligmüller recommends that the child be laid across the mother's knees and told to move the trunk from one side to the other while the pelvis is held firm. If too young to obey the direction, the movement can be excited by pricking or by electric irritation. It will be seen that the trunk can be turned only to one side.
In these paralyses of the trunk it is clear that the deformity does not develop under the influence of muscular antagonism alone, but only when the non-paralyzed muscles attempt to sustain the superincumbent weight of the body or a portion of it.
SUMMARY OF MECHANISMS OF THE DEFORMITIES.—We may indeed conclude, as stated at the beginning, that this complex etiology exists in almost all cases. When the limb is at rest in bed or the weight of the body is transferred to crutches, then repeated contraction of the flexus will suffice to bring the limb into a vicious position (contraction at knee- and hip-joint). When the foot or hand hangs unsupported, its weight is sufficient to cause deformity, even when all the muscles are paralyzed, and sometimes in opposition to the direction of intact muscles. Thus the weight which is passively borne by the limb, and the efforts of intact muscles to effect the function of the limb in spite of the paralysis, both concur in the production of the deformity.
ANATOMICAL LESIONS.—The theory of the anatomical basis of infantile paralysis constitutes one of the most interesting portions of its history. It is indeed one of the most instructive chapters of modern pathology from the rapidity with which in a short time precise knowledge has accumulated, and for the degree in which this has revolutionized previous ideas.
Autopsies.—Until 1863 only five autopsies had been made upon persons affected with infantile paralysis.70 As the disease was never, of itself, fatal, opportunity for pathological investigation did not present itself, while attention was still vividly attracted to the paralysis. When this had become a chronic infirmity, and patients had succumbed to intercurrent disease, the opportunity must have occurred, but was not then utilized.
70 I believe the first list of modern autopsies was made by myself in a paper read before the New York County Medical Society, Dec. 22, 1873. The paper was not published until May, 1874, and in the mean time Seguin's lecture on “Infantile Paralysis” was published in the N. Y. Med. Record, Jan. 15, 1874, with a tabulated list of 25 autopsies, necessarily almost identical with mv own—cases 9 and 10 are really identical. A case by Roth is included; 4 negative autopsies are omitted. In 1880, Seeligmüller, in his elaborate essay in Gerhardt's Handbuch, published a list of 32 cases, including the above, and adding to them the following: Case by Müller, 1871; 4 cases by Leyden, 1875 (Arch. de Psych.); 1 case by Raymond, 1874 (Gaz. méd. de Paris); 1 case by Demme, 1876 (B. med. Bericht über das Jennerischt Kinderspital zu Bern); 1 case by Eisenlohr, 1876 (Tageblatt des Hamburger Naturforscher Versammlung); 1 case by Schultze, 1877 (Virch. Arch., Bd. lviii.).
To this list we may now add 3 cases by Turner, Humphrey, Taylor, 1881 (Path. Trans. London); 2 cases by Dejerine, 1878 (Progrès méd.), giving a total of 37 cases with detailed histories and autopsies.
The first cases affording autopsies in which the spinal cord was thoroughly investigated, belonged, however, precisely to this class of extremely chronic lesions, which should be regarded as showing rather the results of the morbid process than that process itself. Omission to observe this distinction has been the occasion of several misinterpretations of the pathological appearances.
Premature Theories.—In the entire absence of evidence it is a little remarkable that such high authorities as Rilliet and Barthez,71 West,72 Vogel,73 Eulenburg,74 should have pronounced dogmatically that the disease was essential—i.e. unaccompanied by any structural lesion whatever; and that Bouchut,75 on the strength of most incomplete examination, should have built up a theory of myogenic paralysis. It is still more remarkable, after the published autopsies of Rinecker,76 Laborde,77 Cornil,78 Prévost,79 Charcot and Joffroy,80 Parrot and Joffroy,81 Vulpian,82 Roger and Damaschino,83 that Politzer in 1866,84 Brown in 1871,85 Barwell in 1872,86 Kétli,87 Adams in 1873,88 should still adhere to this doctrine. They are, however, entirely in the minority, and all recent monographs and works published with a view to presenting the state of science assume the spinal nature of infantile paralysis to be established beyond possibility of controversy.89
71 Traité des Maladies des Enfants.
72 Diseases of Children, Am. ed., 1860.
73 Diseases of Children, trans. from 4th ed., 1870.
74 Arch. Virchow, Bd. xvii. 1859.
75 Deutsche Klinik, 1863.
76 Gaz. méd., 1864, Soc. de Biol., 1864.
77 De la Paralysie de l'Enfance.
78 Gaz. méd., 1866, Soc. de Biol., 1866.
79 Arch. de Phys., 1870, p. 134.
80 Ibid., p. 310.
81 Ibid., p. 316.
82 Bouchut is said by Simon (loc. cit.) to be alone in his theory; Kétli, however, agrees with him. Eulenburg, in 1872, in his systematic treatise, assigns a central origin to infantile paralysis, but offers no opinion in regard to its nature.
83 Gaz. méd., 1871.
84 Jahrbuch für Kinderkrankheiten, 1866.
85 Compend. für Kinderkrank., p. 161.
86 Lancet, 1872.
87 Jahrbuch für Kinderkrank., 1873.
88 Treatise on Club-foot.
89 See Seguin, loc. cit., 1874; Erb, Ziemssen's Handbuch, Bd. xi.; Seeligmüller, Gerhardt's Handbuch der Kinderkrankheiten; Ross, Treatise on Diseases of Nervous System, vol. ii.; Hammond, Diseases of Nervous System, 6th ed., 1881, etc. etc.
The following table contains a summary of the seven autopsies in which the spinal cord is said to have been examined with negative results. Of these, the only really important case is the third, in which a microscopic examination, made by so competent an histologist as Robin, was said to have discovered no lesion of the cord.
The foregoing autopsies may be tabulated as follows:
TABLE I.—NEGATIVE AUTOPSIES.
| No. | Year. | Name of Author and Patient. | Age at time of Paralysis. | Age at time of Autopsy. | Nature of Symptoms. | Limbs Paralyzed. | Electric Reaction. | Appearances at Autopsy. | Reference in Literature. |
| 1 | 1850 or '51? | Rilliet and Barthez. | ? | ? | ? | l. o. | ? | Negative. | Gaz. méd. de Paris, 1850 (or '51?) p. 681. |
| 2 | 1850 or '51? | Rilliet and Barthez. | ? | ? | ? | b. u. | ? | Negative. | Ibid. |
| 3 | 1867 | Bouchut and Robin. (Angélique Lermain). | 1½ | 3 | Suddenly after 3 days' fever. | both u. | Negative micro- scopically. | Union méd., 1867, No. 130, p. 187. | |
| 4 | 1873 | Adams. | — | — | — | — | — | Negative. | Treatise on Club-foot, p. —. |
| 5 | 1873 | Elischer and Kétli. | — | — | — | — | — | Negative. | Jahrb. Kinderheilk., 1873. |
| 6 | 1873 | Elischer and Kétli. | — | — | — | — | — | Negative. | Ibid. |
Heine, in the absence of autopsies, but arguing from clinical symptoms alone, already inferred the existence of a spinal lesion as cause of the paralysis, and believed that it consisted in congestion, or even in hemorrhagic exudation, capillary or massive, which should compress the cord and result in partial atrophy. The same opinion is advanced in 1844 by Brunnière,90 also by Vogt,91 in 1868 by Salomon92 and Radcliffe.93 The autopsies contained in the following table, in all of which vascular lesions are prominent, might be invoked in support of this view:
TABLE II.—AUTOPSIES SHOWING VASCULAR LESIONS OF CORD.
| No. | Year. | Name of Author and Patient. | Age at time of Paralysis. | Age at time of Autopsy. | Nature of Symptoms. | Limbs Paralyzed. | Electric Reaction. | Appearances at Autopsy. | Reference in Literature. |
| 7 | 1829 | Klein. | 5 | 5 | Persistent cerebral symptoms. | l. o. | ? | Congestion of pia around roots of left brachial plexus. | Quoted by Heine. |
| 8 | 1855 | Brund. | 1 | 5 | Meningitis? | r. u. | ? | Chronic spinal klepto-meningitis. | |
| 9 | — | Hammond. | 4 yrs. stand. | — | l. u. | ? | Encysted clot in left ant. column, lower dorsal cord. | Journ. Psych. Medicine, 1867. | |
90 Krankheiten des Gehirns und Ruckenmarkes.
91 Lahmung der Kinder, p. —.
92 Jahrb. f. Kinderheilk., 1865.
93 Reynolds's Syst. of Medicine.
The first really modern autopsy, that made by Cornil in 1863, agrees with the two earliest on record in disclosing only an atrophy of the cord. Some of the other cases, contained in Table III., note in addition sclerosis of the lateral columns.
In all the remaining autopsies on record are noted atrophy of the anterior gray cornua and more or less extensive destruction of the ganglionic cells.
In a certain number of cases the atrophy seemed to be the unique lesion. But it never was confined to the ganglionic cells, but included the reticulum of gray fibres in which these were imbedded, and which was replaced by a reticulum of connective tissue. These cases were all examined many years after the occurrence of the paralysis.
TABLE III.—AUTOPSIES SHOWING ATROPHY OR SCLEROSIS OF WHITE COLUMNS OR ROOTS.
| No. | Year. | Name of Author and Patient. | Age at time of Paralysis. | Age at time of Autopsy. | Nature of Symptoms. | Limbs Paralyzed. | Electric Reaction. | Appearance at Autopsy. | Reference in Literature. |
| 10 | 1825 | Hutin. | 7 | 49 | Convulsions. | b. u. | ? | Atrophy of cord from below 8th dorsal nerve to thickness pencil, and of nerves. | Quoted by Heine, p. 151. |
| 11 | 1842 | Lunget. | ? | 8 | ? | r. u. | ? | Atrophy and brown discol. of ant. roots of lumbar and sacral nerves going to sciatic. | Anat. et Phys. du Syst. nerveux, i. p. 358. |
| 12 | 1863 | Reckling- hausen. | ? | ? | ? | b. u. | ? | Atrophy of ant.-lateral columns. | Deutsche Klinik, 1863, Jan. 3. Seel. says also atrophy of cells; Seguin and J., tubercles. |
| 13 | 1863 | Cornil. | 2 | 49 | ? | b. u. | ? | Atrophy antero-lateral columns. Seeligmüller says also atrophy of ganglion-cells, as if quoting Charcot. | Soc. de Biol., Comptes Rendus, 1863. Infiltration of gray and white corpuscles, with abundant amyloid corpuscles, especially in anterior gray cornua. These through entire extent of cord—cells—intact. Paraplegia, but walks by muscles of thigh and pelvis; leg and foot atrophied. |
| 14 | 1864 | Laborde and Bouvier. | 1 | 2 | ? | all four. | noted. | Atrophy antero-lateral columns; nerve-fibres atrophied and varicose. | Laborde, De la Paralysie de l'Enfance, pp. 109-119. |
| 15 | 1864 | Laborde and Cornil. | ⅔ | 2 | ? | b. u. | ? | Sclerosis of ant.-lateral columns. Ganglion-cells normal; sciatic neuritis. | Laborde, loc. cit., p. 104. |
The first group is contained in the following table:
TABLE IV.—AUTOPSIES SHOWING ATROPHY OF THE ANTERIOR CORNUA AND GANGLION-CELLS.
In the remaining cases the ganglion-cells of the anterior cornua had also disappeared; but in addition to this atrophy excited distinct evidence of more or less extensive inflammation. This table includes one case of autopsy at two months (Roger's); one at six weeks after paralysis (Turner's).
TABLE V.—AUTOPSIES SHOWING EVIDENCE OF MYELITIS, TO WHICH THE ATROPHY OF THE GANGLION-CELLS WAS CONSECUTIVE.
It will be useful to add another table, which will group together the cases in which the autopsies were made within two years after the occurrence of the paralysis. Of these, all but the two made by Laborde, in which the cornua are declared to be healthy and the lesion limited to the white columns, show traces of destructive morbid processes in the gray substance of the cord, greatly predominating in the anterior cornua, but not absolutely limited to them, nor even to the part of the cord which corresponds to the paralyzed limb:
TABLE VI.
| Case No. | Name of Author. | Date of Autopsy after Paralysis. | Year of Publication. | |
| 41 | Damaschino. | 6 | weeks. | 1883 |
| 40 | Turner. | 26 | days. | 1879 |
| 28 | Roger and Damaschino. | 2 | mos. | 1871 |
| 42 | Money. | 4 | mos. | 1884 |
| 27 | Echeverria. | 6 | mos. | |
| 39 | Eisenlohr. | 6 | mos. | |
| 29 | Roger. | 6 | mos. | 1871 |
| 31 | Roth. | 9 | mos. | 1873 |
| 35 | Leyden. | 11 | mos. | 1876 |
| 14 | Bouvier and Laborde. | 12 | mos. | 1864 |
| 30 | Roger. | 13 | mos. | 1871 |
| 15 | Cornil and Laborde. | 16 | mos. | 1864 |
| 12 | Recklinghausen. | 24 | mos. | 1863 |
| 38 | Demme. | 24 | mos. | 1876 |
| 23 | Taylor. | 18 | mos. | 1879 |
| 25 | Humphrey. | 24 | mos. | 1879 |
AUTOPSIES OF RELATIVELY RECENT CASES.—In cases relatively recent all macroscopic changes in the cord may be entirely wanting. There may be some degree of asymmetry in the surface of section, patches of white coloration in the anterior gray substance, or of gray or yellow color in the white columns; the anterior roots may be congested or even already atrophied.94 On the other hand, there have several times been found foci of visible red softening, much more frequently at a point corresponding to the origin of the paralyzed nerves, but not absolutely confined to them, and sometimes existing at points where they have given rise to no symptoms whatever.95
94 Roger's first case, No. 28 of Table V.
95 Case 1st of Roger, Tab. V.
Microscopic Lesions.—In striking contrast with this paucity of macroscopic lesions are the interesting structural changes revealed under the microscope. These lesions are usually comprised within circumscribed foci whose size may vary from a long diameter of 2 mm.96 to one of from 10 to 30 mm.97 Sometimes bilateral foci are found with monoplegic paralysis; thus one side or the other preponderates in the morbid process.
96 Case Roth, Tab. V.
97 Case Schulze, Tab. V.
In recent cases (Damaschino's, at twenty-six days; Roger's, at two months; Turner's, at six weeks) patches of red softening existed at the portions of the cord containing the nuclei of origin of the paralyzed nerves. In Turner's case the focus contained hemorrhagic extravasation, and the traces of this were clearly perceptible in Roger's first case. The blood-vessels are dilated; their lymphatic sheaths infiltrated with leucocytes and with granular corpuscles; their walls are thickened, pigmented, or fatty.
Leucocytes are often disseminated through the diseased area, and in one case (Demme) were accumulated into a focus of pus. Besides the leucocytes, the foci are often infiltrated with large round granular cells that seem to be transformed neuroglia-cells (Leyden). In one case neuroglia nuclei were accumulated in a ring around the focus, seeming to indicate the beginning of encapsulation.
The most striking lesion, however, and the one which is common to the most recent as well as to old cases, is the deformation, atrophy, and final disappearance of the large ganglionic cells of the anterior cornua. The first change consists in granular pigmentation;98 then the prolongations disappear, leaving the body of the cell shrunken and deformed; at last the whole cell disappears. Sometimes all the cells of an anterior horn have disappeared throughout the entire depth of the focus; quite as often, in certain sections at least, the atrophy is limited to certain groups, as the external,99 or the external in one focus, the antero-lateral in another, situated on the opposite side of the cord.100
98 Case of Echeverria, Tab. V.
99 Case by Schultze (this is a case of ancient lesion), Tab. V. (Virch. Arch., Bd. lviii.).
100 Case by Taylor, Tab. IV. (Path. Trans., London, 1879.)
In a case rendered celebrated by Charcot it is stated that in many sections of the cord atrophy of ganglionic cells constituted the unique alteration, the tissue immediately surrounding the place whence they had disappeared being perfectly healthy. It is on this appearance that has been built up the theory of a primary idiopathic atrophy of the ganglionic cells as the characteristic lesion of infantile paralysis. But in other portions of the same cord Charcot himself describes destruction of the gray reticulum imbedding the cells; and this destruction is insisted upon in many other observations. In other words, there is a general disintegration of the gray nervous tissue of the anterior cornua which contain the focal lesion. The normal tissue is then replaced by a reticulum of conjunctive fibres, more or less dense according to the age of the case.
These focal lesions of the cord explain admirably, as will be seen, the permanent symptoms of the disease. But of great importance for understanding its initial period is the fact that structural changes—similar to, but less severe than, those just described—have beer found diffused throughout the cord. In several cases hyperæmia, partial atrophy of ganglionic cells and nerve-fibres, infiltration with exudation corpuscles, in the anterior cornua and even central gray canal from the lumbar to the cervical region.101 In Damaschino's case, besides the focal lesions which corresponded to the paralyses of the right arm and left leg, were others corresponding to the left arm and right leg where no paralysis existed.
101 See cases by Leyden, Roth, Schultze, Clarke, Damaschino.
This case (1883) is also interesting in bringing out another lesion not usually noticed. This is the breaking up into balls of the myeline in the medullated tubes, both of the anterior intraspinal nerve-roots and of such fibres as traversed the anterior nerves. In balsam preparations these myeline drops are dissolved; but in osmic acid and glycerin preparations they appear as black balls all over the field. The lesion is identical with that already described by Dejerine (1875) in both nerves, roots, and white columns. The columns of Clarke have always been found intact.
In the recent cases no lesions of the white columns have been observed—a fact upon which reposes the doctrine that such lesions, when existing, are secondary to those of the cornua.
SECOND GROUP OF CASES.—The next group of cases contains 16, where the autopsy was made more than two years after the début of the paralysis.
TABLE VII.—AUTOPSIES SHOWING LESIONS OLDER THAN TWO YEARS.
| Case No. | Name of Author. | Date of Lesion. | Year of Publication. | |
| 11 | Longet. | 7 | yrs. | 1842 |
| 27 | Echeverria. | 7 | yrs. | 1866 |
| 43 | Money. | 7 | yrs. | 1884 |
| 16 | Lancereaux. | 16 | yrs. | 1873 |
| 38 | Leyden. | 17 | yrs. | 1876 |
| 33 | Schultze. | 19 | yrs. | 1876 |
| 17 | Charcot and Joffroy. | 25 | yrs. | 1870 |
| 20 | Müller. | 30 | yrs. | 1871 |
| 22 | Clarke. | 31 | yrs. | 1868 |
| 10 | Hutin. | 42 | yrs. | 1825 |
| 13 | Cornil. | 47 | yrs. | 1863 |
| 36 | Leyden. | 54 | yrs. | |
| 34 | Leyden. | 58 | yrs. | 1876 |
| 19 | Vulpian. | 64 | yrs. | 1870 |
| 22 | Raymond. | 70 | yrs. | 1875 |
| 16 | Prévost. | 76 | yrs. | 1866 |
The date of these lesions varies from seven to seventy-six years. In two or three cases, where the autopsy was made on very old people, the early history of the disease was unknown, but the probable date of the paralysis was calculated.
In this group of cases patches of atrophy, semi-transparent and grayish in color, focal or diffused, are clearly perceptible to the naked eye. As a rule, the atrophy is unilateral, and sensibly affects the entire half of the cord. In some cases of paraplegia, however, there is a bilateral, symmetrical shrinkage of the entire lumbar cord, which has been reduced to the size of a quill.
The atrophy involves, first and most markedly, one or both anterior cornua; second, the anterior nerve-roots arising from them; third, the antero-lateral columns.
In both the latter localities the microscope will often find individual nerve-tubes wasted and deprived of their myeline. The atrophied patches are generally sclerosed as the seat of a proliferated neuroglia, coloring deeply with carmine. In Laborde's cases, published at the very beginning of what may be called the anatomical period, the atrophy and sclerosis were said to be limited to the antero-lateral columns and the nerve-roots, while the cornua remained intact. In all more recent observations, however, the lesion of the white columns and roots has been found strictly proportioned to that of the gray horns. The sclerosis extended into the latter, constituted by a reticulum of connective-tissue fibres, sometimes fine, sometimes so matted together as to form a dense felt-like substance, sometimes offering the ordinary aspect of sclerosis.
Amyloid corpuscles have been found infiltrated in great numbers through both the gray and white substance in these old cases (case by Cornil),102 seeming to replace the infiltration with exudation corpuscles observed in the more recent ones.103 When the lesion is distinctly circumscribed the focus is often surrounded by a zone of embryonic cells, seeming to indicate a reactive proliferation on the periphery.104
102 Loc. cit., Soc. Biol.,1863.
103 Seguin (loc. cit.) observes that the opinion is gaining ground which ascribes these to a transformation of the neuroglia corpuscles.
104 Case by Schultze, loc. cit.
As in the relatively fresh cases, the circumstance which has attracted the most attention is the atrophy of the ganglionic cells from the sclerosed patches of the anterior cornua. The completeness with which these have disappeared in any focus seems to be proportioned to the completeness of the paralysis in the corresponding limb. Partial atrophy or disappearance of spinal groups of cells from the cornua may sometimes be correlated with paralysis of special muscles.105
105 Thus in Schultze's case, already quoted, the external group of cells had disappeared from the focus in one gray horn, and the extensors of the foot were alone paralyzed. This seems to confirm the opinion advanced by Spitzka, that this external group of motor-cells corresponds to the extensor, the internal groups to the flexor muscles.
Study of the pathology of infantile paralysis is not completed when the above series of spinal lesions has been enumerated. Most various interpretations have been made of these lesions as they have been successively discovered. Thus, after the theory of congestion came the theory of primary sclerosis, built upon Laborde's two autopsies;106 then the theory of primary atrophy of ganglionic cells;107 then the theory of myelitis;108 finally, a theory of complex and variable lesion.109
106 Laborde, loc. cit.; Cornil, loc. cit.
107 Charcot, Leçons sur les Maladies du Syst. nerveux; Prévost, Soc. Biol., 1864; Joffroy, Arch. de Physiol., 1870; Petitfils, “De l'Atrophie aigue des Cellules matrices,” Thèse de Paris, 1873.
108 Schultze, Virch. Arch., Bd. lxviii.; Roth, Ibid., Bd. lviii.; Henoch, loc. cit., p. 208; Ross, loc. cit., p. 125; Seguin, loc. cit., 1877; Erb, Ziemssen's Handbuch; Seeligmüller, Gerhardt's Handbuch; Roger and Damaschino, Gaz. méd., 1871; Turner, Path. Trans. Lond., 1879; Hammond, loc. cit.
109 Leyden, Archiv für Psych., Bd. vi., 1876.
It was Prévost who first ascribed a predominant importance to the atrophy of the ganglionic cells of the anterior cornua; but it was in the hands of Vulpian, Joffroy, and more especially Charcot and his pupils, that the theory was fully developed. Infantile paralysis was ranked in a newly-formed group of diseases, all characterized by atrophy of these same cells, and differing from each other principally in the acuteness of the process and in its complications.110 Seguin, in his original lecture in 1874, supported the same views, but in 1877 fully adopted that of myelitis. The objections to this theory are: 1st, that by it two diseases so different in their course, localization, electrical reactions, and form of paralysis as atrophic paralysis and progressive muscular atrophy are essentially identified on account of the identity of one lesion, the atrophy of the anterior ganglionic cells;111 2d, the presence of other lesions or of traces of them peremptorily proves the pre-existence of a complex morbid process which involves the ganglionic cells, but is neither limited to them, nor, necessarily, originates in them.
110 Thus, acute anterior poliomyelitis, subacute anterior poliomyelitis, progressive muscular atrophy, amyotrophic lateral sclerosis, bulbar paralysis.
111 When this objection is accepted, Barlow's remark falls to the ground, that “the similarity of lesion found in two such different diseases as infantile paralysis and progressive muscular atrophy proves the failure of anatomical characters, taken alone, to serve as a basis of nosology” (Brain, April, 1879, p. 74).
This inference was drawn by Roger from the hemorrhagic softening, dilatation, and degeneration of blood-vessels, infiltrations with exudation-corpuscles, and hyperplasia of conjunctive nuclei present in his case. Similarily, Schultze, in a case examined nineteen years after the occurrence of the paralysis, found traces of an extensive myelitis in the diffusion of the lesions,112 in the exquisite cellular infiltration, the proliferation of the neuroglia, and the atrophy of axis-cylinders of nerve-fibres together with the cells; and inferred an anterior myelitis, diffused in the long axis of the cord, but limited to the antero-posterior region. Schultze defines Charcot's theory to be an hypothesis of such an acute atrophy of ganglionic cells as leads to a rapid melting down of these bodies, whereby reactionary inflammation is excited in the surrounding tissue. This implies that the dying cells are able to act like a virulent substance on the imbedding tissue, and of this, declares Schultze, “Charcot has offered no proof.”113
112 In this case of paraplegia without lesion of the upper extremity, to which we have several times alluded, there was bilateral atrophy of the lumbar cord, atrophy of the right anterior nerve in the dorsal and lower cervical region, also in the cervical enlargement.
113 It might be said that the fall of the fever as soon as the paralysis is declared and the motor cells presumably melted down should contradict the idea that their dying substance acts as an irritant upon surrounding tissues.
A third objection has been brought forward by Leyden, and is really an enlargement on the second. It is, that various lesions or morbid processes may underlie the same clinical history. In four autopsies of cases presenting all the clinical history of acute anterior poliomyelitis this author has found three different lesions. In one an extensive lepto-meningitis, together with irregular focal sclerosis of the white columns, evidently depended upon the latter, and in turn caused sclerosis of the anterior cornua with consequent destruction of their cells.114 In two other cases an anterior poliomyelitis was accompanied by diffused lesions of the central canal. Finally, in a fourth case the lesions were limited to the anterior cornua, as is most usual.
114 This case of Leyden's throws light on the two autopsies by Laborde with sclerosis of the white columns and intact cornua. It seems probable that a process originating in the cornua had then been arrested or had receded, while continuing its evolution in the white columns.
The theory of acute atrophy of ganglionic cells is not sensibly different from that of a parenchymatous myelitis.115 But all the objections which can be urged against the former theory apply to the latter also, with the exception that the hypothesis of inflammation suggests a cause for the otherwise inexplicable atrophy. Observation of the pathological appearances alone could not decide whether the irritation started in the parenchymatous or interstitial tissues. Reference to the etiology of the disease shows that of the two most frequent apparent causes, blood-poisoning and traumatisms, the first would indicate that the inflammation started in the connective tissue supporting the blood-vessels; the second suggests that the irritation began in the spinal elements constituting the origin of the nerves.
115 Hammond assumes such a form of myelitis in his classification of inflammations limited to the anterior part of the gray matter of the spinal cord:
1. Inflammation of motor and trophic nerve-cells: (a) Infantile spinal paralysis; (b) Spinal paralysis of adults; (c) Pseudo-hypertrophic spinal paralysis.
2. Inflammation of motor cells: (a) Glosso-labia-laryngeal paralysis.
3. Inflammation of trophic cells: (a) Progressive muscular atrophy; (b) Progressive facial atrophy (Dis. Nerv. Syst., 6th ed., p. 464).
We think this classification open to several fundamental criticisms.
Whatever be the starting-point, however, it is very evident that the morbid process soon involves all the tissues contained in the gray matter of the anterior horns, and constitutes, therefore, a real anterior poliomyelitis.
A question of much interest is the relation to this of the lesions of the anterior roots and of the white columns. Is the atrophy of nerve-tubes a passive consequence of their separation from the ganglionic cells, the sclerosis a secondary consequence of this? or is the sclerosis the cause of the atrophy, itself the result of an irritation propagated downward from the myelitic focus, according to the usual law for secondary degenerations in motor tracts? or, finally, is it a residuum of a leucomyelitis (or of the white substance), complicating by simple extension the inflammation of the gray substance?
Review of the autopsies recorded would indicate that the lesions in question are brought about sometimes in one, sometimes in another, of these ways—sometimes even, as in Leyden's case, by extension from a meningitis. That sclerosis of the white columns is most frequently a secondary degeneration is indicated by the frequency with which it appears below the lesion of the cornua, by the rarity with which it is found above, and also by the general proportion between its intensity and that of the disease of the gray matter.
We have devoted so much space to consideration of spinal-cord lesions, because they are by far the most constant and the most important; after these rank the structural alterations of the muscles, which received for a while such a preponderance of attention.
Hammond has studied the progress of these changes on the living subject by fragments of fibre successively removed with Duchenne's harpoon. In an incipient stage of degeneration the fibrillæ are found to be irregular and torn,116 the transverse striæ dim; oil-globules are seen arranged according to the long axis of the fibre. In a more advanced stage the transverse striæ nearly disappear, the oil-globules are in large numbers, and fat-corpuscles are also abundant. Finally, the whole specimen is seen as a mass of air-globules. Six weeks later, however, these had in turn disappeared, and there remained a mass of connective tissue.
116 Though, from the method of removal, this appearance cannot be considered as certainly pathological.
This series of changes, however, does not always take place, as Hammond himself recognizes. Laborde117 first described a granular form of muscle atrophy, where the muscular substance gradually wastes away without ever becoming fatty, and leaving a transparent and hyaline sheath. The two forms of fatty and of simple atrophy can be distinguished by the naked eye. In the latter the muscle begins by being thinner or lighter and softer than usual, ultimately turning light brown. The fatty muscle becomes a homogeneous yellowish-white, diversified by occasional remnants of reddish fibres.
117 Loc. cit., p. 131.
Proliferation of the interstitial connective tissue may be combined with either simple or fatty atrophy. A combination of abundant sclerosis and abundant fatty infiltration may lead to a pseudo-hypertrophy of the muscles.
“There cannot be the slightest doubt,” observes Erb, “that the lesions described constitute a degenerative atrophy similar to what may be caused by section or sense traumatism of a peripheric nerve.”
The peripheric nerves have been much less thoroughly studied than the spinal cord. Leyden first directed special attention to the nerves. He found the sciatic altered in two cases,118 in the first by an interstitial neuritis; in the second by partial atrophy. In 1880 the same writer, in an extensive article on poliomyelitis and neuritis,119 greatly extends his views as earlier expressed. Not only does he claim the coexistence of neuritis with spinal-cord disease in atrophic paralysis, but thinks that many cases of this, and also of other forms of paralysis, “lately supposed to originate in the spinal cord, may really begin in any part of the motor apparatus,” thence sometimes generalize throughout the whole apparatus, sometimes remain limited to the original portion affected. Thus, progressive muscular atrophy may sometimes begin in the nerves, sometimes in the muscles, and sometimes in the ganglionic cells of the cord; and this variety of origin explains the discrepancies of opinion which have been held upon the nature of this disease. Similarly, all forms of acute or chronic atrophic paralysis in either children or adults may begin in either the nerves or cord, thence become generalized to both, or remain limited to one part of the spinal motor system. Cases of atrophic paralysis which recover are probably not cases of poliomyelitis at all, but of multiple neuritis, rheumatic, traumatic, or infectious in nature. The regeneration of peripheric nerves is a well-demonstrated possibility, but not that of the cells of the cord. Lead-paralysis is usually confined to the nerves, but sometimes extends to the cord. In diphtheritic paralysis Buhl has found injection, thickening, and granular infiltration of nerves at the union of their anterior and posterior roots;120 and as long ago as 1876, Dejerine, in a case of atrophic paralysis in a syphilitic woman, found varicose swelling of the medullary sheath in the nerves of the paralyzed lower extremities, together with heaping up of the myeline into large drops, colored black in glycerin and osmic-acid preparations. Coincidently, in the cord, at the origin of the same nerves, the number of motor-cells was diminished, and of those that remained the prolongations, and even the body, of the cell were atrophied.121
118 Cases 34 and 35 of Table V., quoted from Arch. de Psychiatrie, Bd. vi., 1876.
119 Zeitschrift für Klin. Med., 1880.
120 Zeitschrift für Biol., 1867.
121 Arch. de Phys., 1876.
These views of Leyden's are extremely interesting, and should stimulate future research into the condition of nerves in all cases of atrophic paralysis. It is quite incorrect to say, as Archambault and Damaschino have recently done,122 that Leyden denies the existence of anterior poliomyelitis in such cases, especially in such as prove permanent. He only insists on the frequent coincidence of neuritis, on a varying point of departure for the morbid process, and on the probability that in cases of recovery this process has always remained peripheric.
122 Le Union méd., 1883, 7, 35, case quoted in Table V. It is much to be regretted that Damaschino, who strongly controverts Leyden's views, did not examine the nerves in his own most interesting case.
The strongest objection to Leyden's theory is the absence in most recorded cases, either infantile or adult, of the usual signs of nerve inflammation, local pain, or tenderness. Autopsies of old cases are not able to differentiate an inflammation from an atrophic process in the nerves, followed by a secondary thickening of the endoneurium. This thickening was found in three cases examined by Edmonds in 1882, whose subjects had suffered from infantile paralysis in early life, and had had the paralyzed limb amputated at the age of fifteen or sixteen. Transverse sections were made from the internal popliteal nerves. The specimens showed some healthy nerve-fibres, presumed to be sensory; others much smaller, with the axis-cylinders wasted or degenerated; while strands of connective tissue traversed the nerve-bundles, resulting from hypertrophy of the endoneurium. The vessels showed inflammation of their coats, with proliferation of the endothelium.123
123 Trans. Path. Soc. London, 1883.
The brain is usually normal, unless indeed the paralysis has affected children previously rendered idiotic by congenital atrophia cerebri. Sandie, however, examined one brain with an interesting positive result.124 The brain was taken from a boy of fifteen paralyzed since the age of three in almost all his muscles, with even paresis of the muscles of the trunk and neck. The paralysis was more marked upon the right than on the left side. At the autopsy, in addition to atrophy of the muscles and of the motor nerves, with exquisite atrophy of the anterior columns and anterior cornua, was found a decided atrophy of the left central convolution, and, less marked, of the paracentral lobule. This was shown by comparative measurements with the opposite side of the same brain, and also with the corresponding convolution and lobule in two other brains. The child's intelligence had not been affected.
124 Centralblatt f. d. Med. Wissensch., No. 15, 1875.
The arrest of development of the bones has been already mentioned, as well as that of their epiphyses and apophyses. The compact osseous tissue is atrophied: the medullary, on the contrary, abundantly developed and rich in fat.
PATHOGENY OF INFANTILE PARALYSIS.—In the pathological anatomy of infantile paralysis there are two principal facts to be correlated with its clinical phenomena—namely, the limitation of the myelitis to the anterior gray horns of the spinal cord; the destruction of the ganglionic nerve-cells in these gray horns. That the other lesions observed are subordinate to these is shown by their variableness as compared with the constancy of the anterior poliomyelitis. These lesions are, in the cord, the atrophy and sclerosis of the anterior nerve-roots and white columns; in the muscle, the fatty degeneration or simple atrophy of the fibre; in the nerve, breaking down, and finally atrophy of the myeline sheath, sometimes of the axis-cylinder; proliferation of the endoneurium.
Consequence of Limitation of Myelitis.—Limitation of the morbid process to a portion of the motor tract, the anterior cornua, and exclusion of the posterior horns and roots, readily explain the predominant positive symptom of motor paralysis, together with the absence of sensory disturbance. The absence of muscular rigidity, spasm, active contraction, and of exaggerated reflexes is similarly explained by the immunity from the morbid process of the posterior white columns and the portion of the lateral columns immediately adjacent to them. The motor paralysis resulting from destruction of the anterior ganglionic cells of the cord is much more complete than that which depends on simple interruption of the motor tracts passing from the brain. The manner in which the motor tracts are connected by a succession of arching fibres with these cells already indicates that the latter are dépôts for the reinforcement of the motor impulses. We must believe, indeed, that the centrifugal impulses reaching the anterior cornua are not yet motor in character, but to become so must sustain a new elaboration in the ganglionic cells of this region. Evidently, the network of gray fibres connecting the arcuate strands of the antero-lateral columns with the cells become, in virtue of that fact alone, essential to the process. But it is also probable that the multiplied transmission of impressions, which lies perhaps at the basis of the process of their higher elaboration in ganglionic centres, is carried on in the larger network of gray fibres as well as in the smaller network contained in the ganglionic cells. Destruction of a portion of this network would therefore interfere with the elaboration of the motor impulse, in the same manner, though to a relatively less extent, as destruction of the ganglionic cells themselves.
Trophic Lesions.—The rapid wasting of the paralyzed muscles, with their degenerative electrical reactions, seems, however, to be an effect altogether peculiar to lesions of the ganglionic bodies.125 According to Charcot, who has so especially formulated the laws of amyotrophic paralysis, all the ganglionic cells essential to the elaboration of motor impulses exercise a trophic influence upon muscles. The spinal cell, nerve-fibre, and muscle-fibre combine into a complex indissoluble unity or element. One part of this lesion of complex elements is necessarily followed by proportionate lesion of all its other parts.
125 The amyotrophic lateral sclerosis of Charcot exhibits in an exquisite manner the difference between paralysis without atrophy, caused by sclerosis of the antero-lateral columns, and paralysis with atrophy when the morbid process has extended to the anterior cornua.
According to Erb, however, who extends Samuel's doctrine of special trophic nerves, it is not the motor cells which influence the nutrition of the muscle-fibres with which they are connected, but special trophic cells lying among the others in the anterior cornua. This theory is principally based on the existence of muscular atrophies of central origin (progressive muscular atrophy, bulbar paralysis), unaccompanied for a long time by paralysis.126 Hammond cites as a converse example the anterior poliomyelitis “where the peripheric disturbance is, in the first place, solely one of motility; this is paralysis without atrophy. After a time, which may be as much as six months or even more, the trophic changes begin.”127
126 Ziemssen's Handbuch.
127 Loc. cit., p. 429.
But surely this is an exaggerated emphasis on the exception, rather than the true inference from the rule of rapid wasting in anterior poliomyelitis—a rule so general as to have originated the title atrophic paralysis. Erb gives an ingenious scheme (Fig. 55) of the mental relations of motor and trophic cells with cerebral and spinal nerve-fibres. It will be seen that isolated lesions of one or the other trophic apparatus might occur without paralysis of motor tracts, while simultaneous lesion of the trophic apparatus and of the ganglion-cells, or of the latter, involving the tracts coming from the trophic cells, would cause, as in anterior poliomyelitis, motor paralysis, muscular atrophy, loss of the reflexes, degenerative reaction in nerves and muscles.
FIG. 55.
c, trophic cell for nerve; a, cerebral fibre; b, trophic cell for muscle; d, ganglionic cell; s, sensory fibre; f, trophic path to muscle; m, muscle. (From Ziemssen's Handbuch der Speciellen Pathol., Bd. xi. Zweite H., Zweite Abtheil, p. 313.)
Duchenne and Joffroy128 also argue the existence of special trophic nerve-cells. The absence129 of the nutritive lesions of the skin and cellular tissue which are so conspicuous when the gray matter around the central canal or posterior to it is involved,130 the dependence of the nutrition of the motor apparatus, nerves, muscles, bones on the integrity of the anterior horns, are facts which, taken together, seem to indicate that the maintenance of nutrition depends on the unbroken continuity of the motor or sensory apparatus from the periphery to the ultimate central element, rather than on any special central cells endowed with trophic functions.131 Erb's hypothesis, as his own scheme moreover denotes, demands not only trophic cells distinct from motor cells, but separate trophic cells for the muscles, for the motor, and for the sensory nerves.
128 “De l'Atrophie aigue et chronique des Cellules nerveuses,” Arch. de Phys., No. 4, 1870.
129 Money, and also Gowers, have signalized a condition of the skin resembling myxœdema (Tr. Path. Soc. London, 1884, and Brit. Med. Journ., 1879).
130 Mayer (Herman's Handbuch Physiol.) sums up the great mass of evidence now accumulated, which demonstrates the trophic influence of the central gray mass of the cord upon the tissue.
131 Nepveu (La France médicale, 1879) mentions some cases of infantile paralysis complicated with trophic lesions of the skin. The facts, if accepted, could only indicate an extension of the myelitis to the central and posterior regions of the gray columns. The relations between non-atrophic paralysis caused by interruptions of the motor tracts and muscular atrophy dependent on lesion of the anterior cornua are exquisitely shown in a case reported by Sander. An adult suffered from chronic motor paralysis, gradually increasing, in the right arm, with paresis of the lower extremities. In the hand, arm, and shoulder the paralysis was followed by gradual atrophy and diminution of the faradic contractility; in the lower extremities no atrophy occurred. At the autopsy was found a gliomatous tumor seated in the anterior cornua predominating on the right side, extending from the level of the sixth dorsal to that of the eighth cervical vertebra. The ganglion-cells were pigmented and compressed, not altogether destroyed. The lumbar cord was intact, and the non-atrophic paresis of the lower extremities evidently resulted from the interruption of the motor tract above.
The peculiar grouping of nerve-centres within the cord that seems to be indicated by some of the groupings of infantile paralysis shows, as has been said, a probable divergence within the cord of nerve-fibres which run together in the same nerve-stem. The associations to be expected from the data of functional association and of clinical history are by no means fully decided. It is even a matter of dispute whether the tibialis anticus is functionally more associated with the flexors or with the extensors of the thigh, and whether its experimental irritation or clinical paralysis really coincides with that of the first or of the second group. This entire field of observation is new and promises fertile results.132
132 It is from this field that has come a new argument for the spinal nature of lead-paralysis, from its peculiar grouping, and from analogy with that of anterior poliomyelitis of the upper extremities (Remak, “Ueber die Local. Atropa. Spinal Lahm.,” Archiv für Psych., Bd. ix.; also, Ferrier, loc. cit.).
Relation between Limitation of Myelitis and Age.—From the relative frequency of anterior poliomyelitis in childhood, as compared with its much greater rarity in adult life, we must infer the existence of some special conditions in childhood which tend to limit the morbid process to such a portion of the cord. The theory of a primitive spontaneous atrophy of the motor cells would serve, indeed, to explain this limitation. The reasons already alleged for regarding the morbid process as a systematic myelitis decisively hinder the acceptance of such an explanation as it stands. On the assumption, however, that the myelitis is usually of functional origin, and starts, therefore, in the elements of the anterior cornua essentially involved in the motor functions, the morbid susceptibility of these elements may be ranked with the liability to disease of the entire locomotor system which is known to be so predominant in children. From pathological evidence, even without anatomical proof, we may reasonably infer an incompleteness of development in the anterior cornua of the cord correlative with that well demonstrated in the bones and functionally inexperienced muscles. If the antero-posterior fibres which connect the anterior cornua with the central and posterior gray masses be also incomplete, the radiation of irritations, and consequent vascular irritation, would also be arrested within the boundaries of the original lesion. Thus a peculiarly circumscribed, instead of the common diffused, myelitis of adults.
Money133 points out that for the gray matter of the cord, as of the brain, the centre or maximum force of the circulation is on the periphery, and the nutritive supply of the centre is thus easily cut off. Moreover, while the blood-vessels of the cervical and dorsal regions of the cord pass to it transversely, the vessels of the lumbar region are compelled to describe a somewhat prolonged vertical course before reaching their point of distribution. From these circumstances, even transitory congestions in the circulation of the cord are easily followed by irreparable injury of its delicate elements.
133 Loc. cit., Path. Trans., 1884.
Finally, in all discussions on pathogeny must not be forgotten the doctrine of Leyden134 that infantile paralysis, also progressive muscular atrophy, is a disease which may begin at the periphery and extend to the centres, as well as the reverse. It must also be remembered that, as yet, only very scanty evidence exists to support this, in itself, plausible theory.
134 See loc. cit., ut supra.
COURSE OF INFANTILE PARALYSIS.—The most ordinary course of infantile paralysis is that already described as typical—namely, extremely rapid development to a maximum degree of intensity, then apparent convalescence, retrocession of paralysis, atrophy, and ultimate deformities in limbs in which paralysis persists.
Several variations from this typical course are observed. Complete recovery may take place, as in the so-called temporary paralysis of Kennedy135 and of Frey.136 These cases are very rare. But their possibility seriously complicates the estimate we may make of the efficacy of therapeutic measures.137
135 Dublin Quarterly Journal, 1840.
136 Berlin. Klin. Wochensch., 1874. I have described one such temporary case in the article already quoted. These cases seem about as frequent in adults. (See Frey, loc. cit.; also case of Miles, etc. etc.)
137 As of the case of complete recovery, the only one the author had seen, related by Dally, Journal de Thérap., 1880, 1, vii.
On the other hand, there may be a complete absence of regression; and this is observed sometimes in cases where the paralysis is originally limited; sometimes where it is extremely extensive, involving nearly all the muscles of the trunk or limbs;138 or muscles or limbs originally spared may become involved in a fresh attack. Laborde relates cases of this kind. In Roger's first case paraplegia occurred under the influence of scarlatina two months after paralysis of one arm.
138 Thus in Eulenburg's case, quoted ut supra.
The form of anterior poliomyelitis most frequent in adults is the subacute, and after that the chronic. Both are extremely rare in children, the latter excessively so. Seeligmüller and Seguin139 both admit the possibility of a chronic form in children, and the latter has kindly communicated to me one case from his private practice:
Miss N. D——, æt. 15, paresis in both legs, first at age of nine, increased at age of twelve, when weakness of vision first noted. At fourteen both feet in rigid pes equinus, and both tendons achilleis cut, without benefit. Hands became tremulous, without paresis. On examination at age of fifteen found moderate atrophy of muscles of both legs. Tendo Achillis united on both sides, and equinus persists. Voluntary movement exists, both in anterior tibial and in gastrocnemius muscles, but diminished in anterior tibial. Faradic contractility diminished in both sets of muscles; examination difficult from extreme sensibility of patient. In both hands interossei, muscles of thumb, and little finger show tremors and fibrillary contractions. Thenar eminences small, abductor pollicis nearly absent, not reacting to faradic current. Optic nerves slightly atrophied. Mind enfeebled, memory poor; articulation not affected. Five years later the motor paralysis and mental enfeeblement had still further progressed, but no exact notes exist of this period.
139 Loc. cit. (ed. 1877).
Erb140 relates a case that he considers unique at the time in a girl of six. The paralysis began insidiously in the right foot in July; a fortnight later had extended to the left foot; complete motor paralysis existed in August, without any lesion of sensibility: after electrical treatment, then instituted, first return to motility to peroneal muscles in November; by January child able to walk again and electrical reactions nearly normal.141
140 Brain, 1883.
141 In the same number of Brain, A. Hughes Bennett quotes cases of so-called chronic paralysis in very young children which are evidently cases of general paresis from congenital cerebral atrophy. The children were defective in intelligence, could not sit up nor hold up the head; the electrical reactions were preserved. I have seen a great many such cases: they are indeed not at all uncommon. Much more so is Bennett's diagnosis.
COMPLICATION WITH PROGRESSIVE MUSCULAR ATROPHY.—Raymond142 and Seeligmüller describe some rare cases where progressive muscular atrophy declared itself in persons previously affected with infantile paralysis in other limbs. Both observers infer a gradual and chronic extension along the cord of the originally acute anterior poliomyelitis.143 Similar cases have much more recently (1884) been quoted by Ballet as tending to modify the prognosis which has usually been pronounced favorable quoad life and further spinal accidents. (See infra.)
142 Gaz. méd., 1875. No. 17.
143 It seems to me that Seguin's case, above quoted, might be an example of such complication(?). But I have not seen the patient myself, and describe the case according to the views of the author.
PROGNOSIS.—The prognosis of atrophic paralysis, quoad vitam, is, as is well known, extremely good. The prospect of recovery from the paralysis is variable. It cannot be estimated either by the extent of the initial paralysis or by the severity of the fever or attendant nervous symptoms. The electrical reactions alone are of value in the prognosis, and their value is very great. Duchenne first formulated their law: “All the cases of infantile paralysis which I have seen where the faradic contractility was diminished but not lost, and which could be treated by faradic electricity within two years after the onset of the paralysis, have completely recovered.”144 This encouraging statement must be read as applying rather to individual muscles than to cases as a whole. Few complete recoveries of patients are claimed even by so enthusiastic an electrician as Duchenne; who nevertheless affirms his not unfrequent success in re-creating entire muscles out of a few fibres saved from degeneration.
144 Loc. cit.
The persistence of galvanic irritability in muscles which fail to contract to the faradic current has been shown by Erb to belong to the degenerative reactions. Hammond, however, without alluding to the qualitative changes in the galvanic contractions, sees in them the elements of a relatively favorable prognosis, even when faradic contractility is lost. Thus, out of 87 cases, in 39 of which the paralyzed muscles contracted to the galvanic but not the faradic current, 14 were entirely cured, 28 greatly improved, 30 slightly improved, 15 discontinued treatment very early.145
145 Loc. cit., p. 482.
Examination of fragments of living muscle obtained by Duchenne's harpoon, though useful, should not be allowed to exaggerate an unfavorable prognosis. Much fat may be found in such fragments when the muscle is as yet by no means completely degenerated and can be made to contract to one or the other current. Erb, however, admits that the results of treatment have not, in his hands, been brilliant; but adds that he has had no opportunity to treat any cases which were not of long standing.146
146 Loc. cit.
Volkmann147 considers the paralysis entirely hopeless, and advises the concentration of all effort upon the prevention or palliation of deformities.
147 Loc. cit.
It seems probable that at the present moment sufficient data do not exist for formulating a fair prognosis; nor will they until a much larger number of cases than hitherto have been submitted to all the resources of a complex and persevering system of therapeutics from the earliest period of the disease.
SPECIAL PARALYSES.—Among the paralyses, some exercise a more unfavorable influence on locomotion than others. Thus, paralysis of the muscles of the trunk is more difficult to palliate, either by apparatus or by the efforts of the patient, than any paralysis of the limbs. Similarly, paralysis of the upper segments of a limb is more crippling than when confined to the lower. Partial paralysis of the muscles surrounding a joint is often (but not always) more liable to lead to deformity than total paralysis.
Influence of Neglect.—Apart from the influence of treatment in curing the paralysis, must be estimated in the prognosis the effect of care and watchfulness in limiting the disease and in averting many consequences, even of those which are incurable. The rescue of muscles only partially degenerated may often serve to compensate the inaction of those which are irretrievably ruined.
Ballet148 has recently called attention to the fact that in certain cases persons who had been attacked with an anterior poliomyelitis in childhood became predisposed to different forms of spinal disease. Four have been observed: (1) transitory congestion of the cord, causing paralysis of a day or two's duration; (2) an acute spinal paralysis of the form usually seen in adults; (3) subacute spinal paralysis; (4) progressive muscular atrophy. The author relates cases under each of these heads, and further quotes one related by Dejerine in 1882.149 The patient, a carpenter aged fifty-five and with an atrophic deformity of the foot, became suddenly paralyzed in the four limbs, trunk, and abdomen. The paralysis was complete in a month, was stationary for three months, then began to improve, and at the end of six months from the onset of the disease recovery was complete.
148 Revue de Médecine, 1884.
149 Revue de Médecine, 1882.
The observations of progressive muscular atrophy in persons bearing the stigmata of an infantile paralysis are quite numerous.150
150 Charcot, Soc. Biol., 1875, and Gaz. méd.; Seeligmüller (4 cases), in Gerhardt's Handbuch, 1880; Hayem, Bull. Soc. de Biol., 1879; Vulpian, Clinique méd. de la Charité, 1879; Pitres, new observation, quoted by Ballet in 1884.
The prognosis cannot be the same for cases where everything is done to avert malpositions and for those where all precautions are neglected. Thus, prolonged rest in bed favors pes equinus; the use of crutches necessitates flexion of the thigh and forced extension of the foot; locomotion without support tends to displace articulations by superincumbent weight, causing pes calcaneus, genu-recurvatum. Finally, compensatory deformities must be averted from sound parts, as scoliosis from shortening of the atrophied leg, equinus from passive shortening of the gastrocnemii through flexion of the leg, etc.
ETIOLOGY.—Concerning the etiology proper of infantile paralysis little definite is known. It is probable, as has been already noticed, that traumatisms have a much more decided influence than is generally assigned to them. Leyden particularly insists on this influence, and on the facility with which a traumatism relatively severe for a young child may be overlooked, because it would not be recognized as such for an adult. It must be noticed, however, that children are much more liable to have the arms wrenched and pulled violently than the lower extremities; yet in a great majority of cases the lesion is situated in the lumbar cord.
It has been shown that the myelitis, though so limited transversely, is often far more diffused in the longitudinal axis of the cord than might be supposed from the permanent paralyses. This fact corresponds to the initial generalization of the motor disturbance. It seems possible that the traumatic irritation, starting from the central extremity of the insulted nerve, diffuses itself through the cord until it meets with its point of least resistance, and here excites a focal myelitis. That this point should most frequently be found in the lumbar cord would be explained by its relatively less elaborate development, corresponding to the imperfect growth and function of the lower extremities.
A second cause of anterior poliomyelitis is, almost certainly, the presence of some poison circulating in the blood. The frequent occurrence of the accident in the course of one of the exanthemata is one indication of this; other indications are found in such cases as that related by Simon, where three children in one family were suddenly attacked—two on one day, one, twenty-four hours later.151 The same author relates a case of motor paralysis in an adult, followed by atrophy of left lower extremity, and which occurred during a fit of indigestion caused by eating mussels.152 The acute ascending paralysis of Landry, with its absence of visible lesion, has been said to strikingly resemble the effects of poison. Hydrophobia and tetanus are again examples of the predilection exhibited by certain poisons for the motor regions of the cord.
151 Journal de Thérap., 7, vii., 1880, p. 16. These children belonged to an American family, but were seen by several distinguished French physicians.
152 P. 357.
The evidence that infectious diseases may constitute the immediate (apparent) causal antecedent of acute poliomyelitis has led, not unnaturally, to the theory that all cases of acute infantile paralysis are due to a specific infecting agent, some as yet unknown member of the great class of pathogenic bacteria. It may be noticed, however, that the occurrence of the spinal accidents after the ordinary infectious diseases, as scarlatina and measles, should as well indicate that a specific agent proper to itself was at least not essential to its development.153
153 Perhaps the occurrence of diphtheria in the course of scarlatina and typhoid should indicate a similar lack of real specificity in the morbid agent of the former disease.
The influence of exposure to cold, which seems to have been sometimes demonstrated, must probably be interpreted, as in the case of rheumatism and pneumonia, as effective by means of some poison generated in the organism when cutaneous secretion, exhalation, or circulation has been suddenly checked.
DIAGNOSIS.—The diagnosis of the acute anterior poliomyelitis of childhood is usually easy, but unexpected difficulties occasionally arise.
Typical cases are markedly different from typical cases of cerebral paralysis, but in exceptional cases these differences disappear. This is shown in the following table:
| SPINAL PARALYSIS. | CEREBRAL PARALYSIS. |
| Paraplegic or monoplegic (rule). | Hemiplegic, (rule). Monoplegic as residuum of hemiplegia or as consequence of solitary tubercle (exception). |
| Hemiplegic as residuum from paraplegia, or original and involving facial nerve (very exceptional). | |
| Intelligence free (rule). | Intelligence depressed (rule). |
| Intelligence depressed (when spinal paralysis has affected imbecile children). | Intelligence free (exception, especially with solitary tubercle). |
| Disposition lively. | Disposition apathetic or cross. |
| Initial convulsion unique; general symptoms of a few hours' duration (rule). | Convulsions repeated; pyrexia prolonged several days or weeks (rule). |
| Convulsion repeated during two to three weeks before paralysis; fever a month (rare exceptions). | |
| Sensibility intact (rule). | Sensibility intact after initial period. |
| Occasional hyperæsthesia (exception). | |
| Reflexes cutaneous, and tenderness lowered or lost (rule). | Reflexes intact. |
| Reflexes preserved when only single muscles in groups paralyzed. | |
| Associated movements of hand absent (Seeligmüller). | Associated movements frequently observed in hand. |
| No rigid contractions of upper extremity. | Extensive and rigid contractions of upper extremity very frequent. |
| Atrophy of paralyzed muscles and arrested development of limb, very marked. | Atrophy very slight. |
| Faradic contractility diminished or lost; degenerative galvanic reaction. | Electrical reactions normal. |
Rather singularly, the diagnosis from transverse myelitis is less liable to error than that from cerebral paralysis:
| ANTERIOR POLIOMYELITIS. | TRANSVERSE MYELITIS. |
| Fever brief or absent. | Persistent fever. |
| Sensibility intact. | Hyperæsthesia, then anæsthesia. |
| Decubitus absent. | Presence decubitus. |
| Reflexes lost. | Reflexes increased. |
| Atrophy of muscles. | Atrophy of muscles sometimes as intense. |
| Electrical muscular contractility lost. | Loss of electrical contractility, but not proportioned to sensory and motor disturbance; less rapidly completed. |
The diagnosis from hæmatomyelitis is almost impossible, and practically useless. For if the hemorrhage be severe, the child dies at once, as in Clifford Albutt's case. If less severe, it excites a myelitis, and the history becomes identical with that of the disease we are considering; or if the clot beyond the anterior cornua, it is identified with a vulgar myelitis of traumatic origin.
Progressive muscular atrophy is extremely rare in childhood, but is occasionally seen under hereditary influence (Friedreich's disease). In adult cases confusion is not only easy to make, but often difficult to avoid, especially with the rare, chronic form of poliomyelitis. The basis of distinction is as follows:
| ANTERIOR POLIOMYELITIS. | PROGRESSIVE MUSCULAR ATROPHY. |
| Onset sudden; maximum of paralysis at the beginning. | March very gradual; maximum of disease not attained for years. |
| Faradic contractility lost almost at once. | Faradic contractility not lost until atrophy complete. |
| Shortening of limbs and atrophy of limbs (in infantile cases). | No arrest of development of limbs. |
| Functionally associated muscles frequently associated in paralysis: hand rarely affected. | Capricious selection of muscles, but frequent wasting of these at eminences. |
Paralysis from lesion of a peripheric nerve closely imitates anterior spinal paralysis.154 It is distinguished by closely following the distribution of the injured nerve, and, usually, by concomitant lesions of the sensibility and of cutaneous nutrition.
154 The importance of this fact has been shown in the section on Pathogeny. (See also quotations from Leyden and remarks on lesions of peripheric nerves.)
The pseudo-paralysis sometimes observed in syphilitic children as a consequence of a gummatous infiltration of the bones at the junction of the epiphysis and diaphysis155 might easily be mistaken for a spinal paralysis. But it is an affection peculiar to the new-born; the electrical reactions of the paralyzed muscles are intact; careful examination will show that the movements of the muscles are not impossible, but restrained by pain; often other syphilitic affections are present.
155 Parrot, Wagner.
The diagnosis from diphtheritic paralysis is embarrassed, from the fact that true anterior poliomyelitis may develop in the course of diphtheria as of other infectious diseases. The paralysis of the soft palate, preservation of faradic reaction, absence of atrophy, and the usually rapid recovery must establish the differentiation.
In spinal paralysis there is loss of the reflexes,156 and also of faradic contractility, both of which are preserved in hysteria. In hysterical paralysis, also, there is no wasting of the affected muscles.
156 See Gowers's monograph on “Spinal-Cord Diseases” for an excellent summary of the spinal reflexes.
Various diseases of the bony skeleton or articulations may simulate spinal paralysis. Congenital club-foot, caused by unequal development of the bones and cuticular surfaces, is to be distinguished from the paralytic variety by the date of its appearance,157 by the deformity of the tarsal bones, and by the extreme difficulty of reduction.
157 Though in some cases paralysis of the muscles of the foot seems to take place during fœtal life, and a club-foot result which is both congenital and paralytic.
Caries of the calcaneum, leading the child to walk on the anterior part of the foot to avoid pressure on the heel, may leave after recovery such a retraction of the plantar fascia as to cause a degree of equinus and varus, with apparent paralysis of the peroneal muscles. I have seen one such case.
Congenital luxation of the hip may simulate paralysis; indeed, by Verneuil, it has been attributed to an intra-uterine spinal paralysis. There is, however, no change in the electrical reactions of the muscles surrounding the joint.
In coxitis, however, Newton Shaffer158 has demonstrated a moderate diminution of faradic contractility in such muscles, and a corresponding degree of atrophy; and this fact might complicate the diagnosis of paralysis from arthritis of the hip-joint. Gibney159 has called attention to the facility with which this confusion may arise, and Sayre160 relates cases of infantile paralysis mistaken for coxitis.
158 Archives of Medicine.
159 Am. Journ. Med. Sci., Oct., 1878.
160 Orthopædic Surgery.
In a case observed by myself, which had been previously diagnosed as coxitis, the mistake was all the more interesting as the paralysis which really existed seemed to have been caused by a meningitis rather than primary myelitis of the cornua.161 It thus corresponded to the meningo-myelitic case related by Leyden.
161 The details of this case are as follows: C. P——, aged 11, ten months previous to consultation suffered from febrile attack, accompanied by retraction of head, severe pains diffused through body and intense at nape of neck; unconsciousness for thirty-six hours; vomiting; no convulsions. Case diagnosed as cerebro-spinal meningitis by attendant physician. Convalescence in a week, but with pain in lumbar region of back, predominating on right side, so aggravated by standing or walking that both acts impossible. Coincidently, pain in right calf; exquisite tenderness to pressure even from stocking. No complaint in recumbent position. Child could not get from floor to bed, nor raise right leg from ground. As pain subsided walking became possible, but right leg dragged. Chronic twitchings on left side, face, arm, leg. These symptoms lasted ten or twelve weeks, but at end of nine weeks patient could walk up stairs. In ten months power of walking almost recovered, but there remained a certain amount of lordosis and oscillation of pelvis, which is jarred on the left side while the right leg is swung forward. Recumbent, all movements executed equally well on both sides and passive motion of the hip-joint perfectly free. Circumference of right thigh and leg diminished from one-half to one inch as compared with the left. Faradic contractility diminished on the right side in the gluteal muscles, vastus externus, and rectus, and in the gastrocnemii. The sacro-lumbalis muscle was, unfortunately, not examined, but from the lordosis was probably affected. The remaining muscles were intact. Pain on pressure persisted over right side of second, third, and fourth lumbar vertebræ. Diagnosis was made of a limited meningeal exudation, with compression of anterior part of cord or of a portion of the lumbar and of the sacral plexus.
Scoliosis, which may be caused by the relatively rare unilateral paralysis of some of the muscles of the trunk, may also be simulated by paralysis with shortening of one lower extremity. To compensate the shortening, the trunk is bent over on the paralyzed side; hence a lateral curvature, easily reducible, but easily leading into error.
It would seem easy to distinguish traumatic cases of subluxation of the humerus from those due to paralysis of the deltoid. Yet sometimes only the history will serve to establish, and that somewhat doubtfully, the diagnosis.162
162 A child of four was brought to me with a stiffness and rigidity of the shoulder-joint which could only very partially be overcome by passive motion, and not at all by voluntary effort. The mother stated that several months previously the child had, without apparent cause, become suddenly unable to move the arm. After two months' delay it was taken to a dispensary, and told that the arm was out of joint, and had it reset under ether. From this date the stiffness had gradually developed. The deltoid was atrophied, with marked diminution of the faradic contractility. Question: Were these signs merely symptomatic of an arthritis consequent on a dislocation, or was the latter the result of a spinal paralysis of the deltoid?
THERAPEUTICS.—The treatment of anterior poliomyelitis embraces two stages. In the first it is directed against inflammation of the spinal cord and the paralysis of the muscles; in the second period the spinal lesion has run its course and the paralysis is considered incurable. Treatment is then directed to the prevention or palliation of deformities or toward facilitating the functions of the limb in spite of them.
These two periods are not, however, rigidly separated from each other in chronological order. From the very outset it is important to take certain precautions to prevent deformities, and while palliating these with orthopædic apparatus it is important for years to continue treatment of the paralyzed muscles in the hope that at least a remnant of them may be saved. To abandon the case to the orthopædic instrument-maker, or to neglect the problem of dynamic mechanics while applying electricity and studying the progress of fatty degeneration, are errors greatly to be condemned.
The treatment of the initial stage is necessarily purely symptomatic for the fever and convulsions, since the diagnosis cannot be made out until these have subsided.
As soon as the diagnosis is clear, however, certain measures should be adopted to diminish the hyperæmia of the spinal cord. Dally163 recommends the ventral decubitus; almost all modern authorities advise ice to the spine and ergot internally or subcutaneously. Thus, Althaus164 makes hypodermic injections of ergotin in doses of one-fourth of a grain for a child between one and two years old; one-third of a grain between three and five; and one half grain from five to ten; and these doses repeated once or twice daily. The only objection to this treatment is the degree of local irritation it can hardly fail to occasion. Hammond, who “affirms ergot to be of great service, the only medicine capable of cutting short the disease or of limiting its lesions,” recommends the internal administration of the fluid extract—ten drops three times a day for infants of six months, half a drachm for children between one and two years.165
163 Journ. Thérap., t. viii., 1880.
164 On Infantile Paralysis.
165 I have elsewhere quoted one case of early recovery under the use of ice and ergot; or was this a case of temporary paralysis?
The belladonna treatment, at one time so warmly praised by Brown-Séquard, retains to-day few adherents.
Simon advises cutaneous revulsives to divert the circulation to the surface; thus, hot-air baths, mustard powder sprinkled on cotton enveloping the limbs. Ross advises mercurial inunction along the spine, followed by iodine and blisters. At the same time, iodide of potassium should be given internally in large doses. The action of this drug upon inflammations of the nerve-centres seems, within certain limits, to be indisputable, but its mode of action is certainly very obscure. Where the lesion can be attributed to a meningo-myelitis,166 the iodide may be expected to facilitate the absorption of the exudation. In these cases it should be continued for a long time.167
166 As in Leyden's first case, and my own.
167 Binz explains the local action of iodine by an exudation of leucocytes which follows the dilatation of blood-vessels. These elements break down the exudation into which they are poured, and thus facilitate its absorption.
Electrical treatment may be begun by the end of the first week after the paralysis. At this stage Erb recommends central galvanization as an antiphlogistic remedy for the myelitis. For this purpose a large anode must be placed over the spine at the presumed seat of the lesion, while the cathode is applied over the abdomen. By a slight modification of the method the cathode is placed over the paralyzed muscles. The application is stabile, and, according to Erb, should last from three to ten minutes; according to Bouchut, several hours daily. Erb's method is intended exclusively as a sedative to the local inflammation. When the cathode is placed on the muscles it is hoped that the descending current, replacing the lost nervous impulses, may avert the threatening degeneration of the muscle and nerve.
Faradization cannot modify the inflammatory lesions of the cord. As a means of averting degeneration in completely paralyzed muscles it is inferior to galvanism, and should not therefore be used in those muscles which refuse to contract under its stimulus. Its immense utility, however, is as a stimulus to muscles imperfectly paralyzed, but liable to degenerate from inaction and to be overborne by their antagonists. The excitation of contractions in such muscles is a powerful local gymnastic, helping to maintain nutrition by artificially-excited function.
For the same purpose, muscles inexcitable to the faradic current should be, when this is possible, made to contract by the interrupted galvanic current. After this treatment has been prolonged during several months, the faradic contractility often returns, and the current then should be changed (Seguin).
The value of electrical treatment has been very differently estimated. Erb remarks that “its results are not precisely brilliant.” Roth, whose testimony perhaps is not above suspicion, since evidently prejudiced, insists that numerous cases fall into his hands which have submitted for months to electrical treatment without the slightest benefit. On the other hand, Duchenne, as is well known, has expressed almost unbounded confidence in the therapeutic efficacy of faradization, declaring that it was capable of “creating entire muscles out of a few fibres.”
The sensitiveness of children to the electrical current, and their terror at its application, seriously interfere with its persistent use; as, if the patience of the physician is maintained, that of the parents is very likely to fail in the presence of the cries and resistance of the child.
It is very probable that some of the failures of electrical treatment are due to the attempt to rely upon it exclusively, instead of suitably combining both electrical methods with each other and with other remedial measures. With our present knowledge it is safe to assert the desirability of persistent electrical treatment during at least the first two years following the paralysis. The currents must never be too strong—the faradic, at least, never applied for longer than ten minutes at a time. The muscles should be relaxed by the position of the limbs (Sayre). If the muscles continue to waste, and especially if they become fatty, the electrical response will grow less and less, and finally cease altogether.168 In the contrary case the galvanic contraction will become normal in quality, and the faradic contractility will return and increase, while the atrophy is arrested and the muscle regains its bulk and voluntary powers. Sometimes, as already stated, the latter is regained, while faradic contractility remains greatly diminished.169
168 Passing through three stages: faradic contractility diminished, galvanic contraction increased; faradic response lost, galvanic degenerative; absence of contraction to either current.
169 Sayre (loc. cit.) has noticed cases in which the muscle would contract several times under faradism, then refuse to do so for a day or two. This observation, if valid and not due to unequal working of the battery, is a most curious one.
A succedaneum to electricity that is highly prized by some authorities is strychnia, especially when subcutaneously administered. Pelione170 relates the cure of two cases in children of four and five years, after three and four years' duration of the paralysis, by strychnia—one-half milligramme daily. None should be given to children under six months, but over that age one-ninety-sixth of a grain may be given (Hammond). It should not be given subcutaneously more than two or three times a week (Seeligmüller).171
170 L'Union médicale, 1883.
171 Duchenne relates a case of a paralysis general at the outset and remaining so for six months. It was then treated by strychnine for five or six months, and at the end of that time had become limited to the lower extremities (Elect. local., ed. 1861, p. 278).
The incidental action of electricity in attracting blood to the paralyzed muscles may be sustained by several other methods.
Among these the external application of heat, either dry or in the form of hot douches, alternating with cold, is an adjuvant remedy of real importance. Beard has suggested tubing, malleable to the limbs, for the conduction of hot water. It is desirable to employ massage immediately after cessation of the hot applications.
On the value of massage and passive gymnastics opinion is even more variable than in regard to electricity. Roth, a specialist in orthopædics, places it at the head of all remedial measures, and denounces electricity in comparison. Many professional manipulators, ignorant of medical science, continually claim wonderful triumphs over regular physicians obtained by means of systematized massage. Volkmann, on the other hand, dismisses the pretensions of the Heilgymnastik with considerable contempt, declaring that faradization is the only method which can really secure exercise to paralyzed muscles.
The Swedish movement cure consists in passive movements imparted to a limb by the manipulator, at the same time that they are strenuously resisted by the patient. From the nature of this method, and its aim in stimulating the voluntary innervation of the muscles, it is admirably adapted to hysterical paralysis. Theoretically, it is difficult to perceive the applicability of this method in organic atrophic paralysis, especially in young children, whose voluntary efforts cannot be commanded. There are, however, several real indications for passive gymnastics in the treatment of infantile paralysis. Surface friction and deep massage have some influence in dilating the blood-vessels and causing an afflux of blood to the cold and wasting muscles. A probably more important effect may be produced upon the contraction caused by malposition and adapted atrophy of certain groups of muscles. It is these contractions which formerly constituted the special objection of the orthopædist, and were treated almost universally by tenotomy. They are in any case the proximate cause of deformities; and, generally existing on the side of the joint opposite to the most severely paralyzed muscles, they keep these over-stretched and prevent them from receiving the benefit of the electrical treatment. Muscles which will not contract to the faradic current while thus stretched will often begin at once to do so when the rigidity of their antagonists has been overcome.
Persevering stretching by the hands will often overcome this rigidity as completely, and even more permanently, than will the tenotomy-knife. It is in this part of the treatment that entirely ignorant and even charlatan manipulations do, not unfrequently, achieve remarkable results.172
172 Of course many of those on record, and to some of which I have been a witness, relate to hysterical contractions, hysterical scoliosis, etc.
It is the retracted tendo Achillis and plantar fascia which most frequently require this manipulation. In the paralytic club-foot of young children all authorities agree in the value of repeated manipulations and restorations of the foot as nearly as possible to a position where it may be retained by simple bandaging. While turning the foot out it becomes perfectly white, but on releasing hold of it the circulation is restored, after which the manœuvre may be repeated (Sayre).
This principle of intermittent stretching by seizure of the segments of the limb above and below the joint applies to all forms of paralytic contraction. In the trunk the pelvis should be held by the mother, while the manipulator, seizing the thorax of the child between both hands, moves it gently but forcibly to and fro in the required direction. Great care is required in these manipulations—not merely to avoid exhausting the muscles, but even to avoid fracturing atrophied bones.
It may be laid down as a positive rule that tenotomy should never be performed in the contractions of spinal paralysis until the resources of manipulation have been exhausted. It is to be remembered that the rigidity depends on no active contraction of the muscle, but on its elastic retraction. The manœuvre of stretching does not appeal to the force of contractility, which may have been lost, but to the force of elasticity, which remains and can be made to act in a reverse direction. Finally, in the cases where the retracted muscles have not been originally paralyzed, but have lost the power of contracting during the process of shortening, this power may be restored if the muscle regain its normal length.
The operation of tenotomy, apparently a far more heroic measure, is often a less efficacious means of arriving at the results. Unless followed by the application of apparatus which permits motion in the joint, section of contracted tendons is only of brief utility.
Though the edges of the cut tendon have been kept apart until the intervening space is filled by new tissue, union is finally effected by the latter, and retraction through elasticity is again imminent. Often, therefore, the deformity is repeated in spite of repeated operations; when it is not, the happy issue is due to the fact that, with increased freedom of locomotion immediately after the tenotomy, the patient has been enabled to bring the influence of weight to bear in such a manner as to fix the limb in a new and more convenient position. Thus, after section of the tendo Achillis for pes equinus, if the patient begins at once to walk on the paralyzed foot, the weight of the body, pressing down the heel, may keep the tendon stretched. So walking immediately after section of the hamstring muscles will have a tendency to produce genu-recurvation by the same mechanism which produces it in total paralysis, and the original deformity will not recur.
Besides the tendo Achillis, the parts which may be occasionally submitted to tenotomy are the plantar fascia, the peroneal muscles, very rarely the anterior tibial and extensors, the hamstrings, the thigh adductors. Section of the external rotators of the thigh or of the tensors of the fascia lata could hardly ever be required, and among these operations Hueter173 rejects that on the plantar aponeurosis as inadequate. The excavation in the foot it is designed to remedy depends upon alteration in the form of the tarsal bones, and can only be cured by means of forcible pressure exerted on their dorsal surface. Section of the peroneal muscles, often recommended by Sayre, is considered by Hueter to be superfluous after section of the tendon achilleis. Paralytic contraction of the hamstrings or of the hip flexors is rarely sufficiently severe to demand tenotomy.
173 Loc. cit., p. 416.
From what has preceded it is evident that maintenance of locomotion is of great importance, in order to avoid the deformities which are threatened by prolonged repose. Locomotion, however, can only be safely permitted with the assistance of apparatus capable of restraining the movements liable to be produced by the weight of the body. The supporting instrument which restrains movement in certain directions must, however, facilitate it in others: immovable apparatus, such as is not infrequently applied after tenotomy, is always injurious.
In young children unable to walk, the development of pes equinus may often be prevented by drawing down the foot to a sole splint made of thin wood, gutta-percha, or felt, and fastening it with a flannel bandage. The point of the foot may be drawn up toward the tibia by a strip of diachylon plaster. If the equinus has already developed, a splint of gutta-percha or of felt (Sayre) may be modelled to the leg and foot while the latter is held forcibly in dorsal flexion. The splint is attached by means of strips of adhesive plaster. It should extend as far as the knee, and be suitably padded (Seeligmüller).
In children able to walk a sole splint of thin metal, to which the foot had been previously attached by a flannel band, should be inserted in a stout leather boot. On the outer side of this boot should run a metallic splint, jointed at the ankle and extending to a leather band surrounding the leg just below the knee. A broad leather band, attached to the outer edge of the sole anterior to the talo-tarsal articulation, also passes up on the outside of the foot, gradually narrowing until, opposite the ankle, it passes through a slit in the side of the shoe, to be attached to the leg-splint. This band tends to draw the point of the foot outward, and thus correct the varus (Volkmann). Sayre174 has improved on this shoe by dividing the sole at the medio-tarsal articulation, in which lateral deviation takes place, and uniting the anterior and posterior parts by a ball-and-socket joint, permitting movement in every direction.
174 Loc. cit., p. 88.
The orthopædic boot for the treatment of calcaneo-valgus is constructed on the same principle. But the splint runs up the inner side of the leg, and the leather strap passing to it from the edge of the sole draws the point of the foot inward and raises its depressed inner border (Volkmann). Essential to the treatment of this deformity, however, is the elevation of the heel. This is effected by means of a gutta-percha strap which is attached below to a spur projecting from the heel of the shoe, and above to a band encircling the leg. If, by rare exception, a paralytic calcaneus exists in a child unable to walk, a simple substitute may be found for the shoe in a board sole-splint projecting behind the heel, attached to the foot by a strip of adhesive plaster, which finally passes from the posterior extremity of the board up the back of the leg, and is there secured by a roller bandage.
The device of the gutta-percha elastic band to replace the gastrocnemius muscle illustrates a principle of wide application in orthopædic apparatus. The suggestion to replace paralyzed muscles by artificial ones was first made by Delacroix175 in an apparatus designed for the hand. The suggestion was repeated by Gerdy;176 and in 1840, Rigal de Gaillac proposed to exchange the metallic springs hitherto used for India-rubber straps. Duchenne elaborated the suggestion in a remarkable manner,177 using delicate spiral springs as a substitute for the lost muscles, and taking the greatest pains to make the insertion-points of these to exactly correspond with the insertions of the natural muscles. This was effected by means of sheaths, imitating natural tendinous sheaths, sewed to a glove or gaiter in which the hand or foot was encased.
175 Article “Orthopédie,” Dict. des Sciences médicales, quoted by Duchenne.
176 Traité des Bandages, 2d ed., Paris, 1837, quoted by Duchenne.
177 See chapter on “Prothetic Apparatus” in his treatise De l'Électrisation localisée.
At the present day the prothetic apparatus the most employed is that contrived by Barwell.178 The principle is the same as Duchenne's, but the artificial muscles are made of India-rubber, to which a small metallic chain is adjusted, and they are attached to the limb by means of specially-devised bands of adhesive plaster and pieces of tin bearing loops for the insertion of the muscle. In this apparatus the artificial muscles do not attempt to imitate the situation of the natural muscles with the precision which Duchenne claimed for his. Barwell's own dressing for talipes valgus consists of two rubber muscles which pass from the inner border of the foot, one to the inner, the other to the anterior, part of a band which encircles the leg just below the knee. For talipes calcaneus another band is required behind the leg, passing to the heel, as in Volkmann's apparatus, already mentioned. For talipes varus a rubber band should pass on the outside of the foot; for equinus, one or more from the anterior part of the leg to the sides of the anterior part of the foot.
178 A tolerably minute account of the Barwell dressing is given by Sayre, loc. cit., p. 84.
Sayre endorses Barwell's dressing as entirely adequate for the treatment of any form of club-foot, but modifies it by substituting a ball-and-socket shoe for the adhesive plaster which should encircle the foot. The artificial muscles are then passed from the sides of the shoe to a padded leather girdle encircling the leg. A straight splint, jointed opposite the ankle, runs up from each side of the foot to this girdle, and from it two lateral upright bars, jointed at the ankle, pass to the heel of the shoe; and from below the joint passes forward on each side a horizontal bar reaching the point of origin of the artificial muscles and giving attachment to them.
In equinus it is necessary to bind the heel of the foot down firmly in the heel of the shoe; and this is accomplished by means of two chamois-leather flaps which are attached to the inside walls of the shoe and lace firmly across the foot.179
179 “The aim of the dressing or instrument is simply to imitate the action of the surgeon's hand; accordingly, any apparatus combining elastic force is far superior to any fixed appliance; and, moreover, that is to be preferred which is the most readily removable. Shoes, therefore, are better than bandages or splints. A proper shoe must have joints opposite the ankle and the medio-tarsal articulation; it must permit the ready application of elastic power; and it must not so girdle the limb as to interfere with the circulation” (Sayre, loc. cit., p. 91).
Sayre places so much confidence in the power of this elastic tension to overcome contractions that he rarely resorts to tenotomy in the treatment of paralytic talipes. Hueter, however,180 considers tenotomy much the speediest, and therefore the most desirable, way of removing contractions.181
180 Loc. cit.
181 Loc. cit.
Seeligmüller quotes with approval Böttger's method for the treatment of deformities, where the weight of the body is utilized to stretch the retracted tendons. Thus, for talipes equino-varus an over-reduction is effected under ether, and the foot forced into a position of moderate calcaneo-valgus. In this position it is retained by the immediate application of a plaster or silica bandage. After this has hardened the child should be encouraged to walk in the mould, with the addition of felt shoes having a slanting sole that is thickened like a wedge at the inner side of the foot and strapped on like a skate. Then, during the act of walking the body tends to constantly force down the heel and thus stretch the retracted tendo Achillis, while the bandage and felt sole (acting like a splint) prevent the inner side of the foot from slipping up.
For talipes valgus the method is analogous, but the foot is forced into an equino-varus position, so that the tendo Achillis is artificially shortened, and ultimately becomes a rigid band, capable, in spite of the sural paralysis, of sustaining the heel.
A cause of relapse in talipes not unfrequently overlooked is the presence of even slight contractions of the hip- and knee-joints. These by shortening the limb tend to the production of equinus, since the foot points itself in order to reach the ground. These contractions, whose rigidity is far inferior to that induced by chronic arthritis, may be overcome by forced extension under ether or gradually by manipulations, or by the weight-and-pulley apparatus, applied in the recumbent position, as in morbus coxarius. The obvious objection to the latter method is the confinement in bed which it necessitates in a child enjoying at the time perhaps robust general health.
The contraction once overcome, the limb must be placed in apparatus which shall both maintain suitable extension and assist in supporting the trunk during station and locomotion. The latter purpose is effected, as in apparatus for chronic joint diseases, by transferring the weight of the body to steel splints running up each side of the limb, the outer one as far as a girdle which encircles the hips; the inner to a band surrounding the upper part of the thigh. Thus is extended the support which in paralysis limited to the leg-muscles is given by the steel splints inserted in the side of the club-foot shoes.
In the simplest form of apparatus locomotion is expected to be accomplished by the action of muscles inserted above the seat of the paralysis. Thus, when the muscles passing over the ankle-joint are paralyzed, the foot is moved as a dead weight by means of the quadriceps extensor, popliteus, and hamstring muscles inserted at the upper extremity of the leg. If the quadriceps cruris is paralyzed, the rotators of the thigh, ilio-psoas, sartorius, and adductor muscles, passing from the pelvis to the thigh, and which are so frequently intact in atrophic paralysis, are enabled to move the limb if the weight of the body is borne by steel splints, if these be light and properly jointed at the hip, knees, and ankle.182
182 Or the joint of the knee may be kept locked while the patient walks, when extension of the limb is mainly required, during both the active and passive movements of locomotion, the necessary flexion being supplied at the hip and ankle. By means of a key the knee-joint can be flexed during the sitting positions.
But an important aid to locomotion may be obtained from the artificial muscles, whose elastic tension is of such value in overcoming contractions. The quadriceps extensor, the most frequently paralyzed, may be supplemented by an India-rubber band and chain passing down the front of the thigh from a point on the pelvic girdle corresponding to the anterior iliac spine to a point on a leg-band, imitating the tibial insertion of the quadriceps tendon. Analogous bands stretched on the posterior aspect of the thigh simulate the hamstring muscles. When the external rotators are paralyzed, the artificial muscle must stretch from the pelvic girdle to a band encircling the upper part of the thigh.
The action of these muscles, apart from their elastic tension of repose, is thus explained by Duchenne: When any effort is made to move a paralyzed limb, the intact antagonists to the paralyzed muscles contract; thus, the flexors of the leg. But this contraction, being constantly opposed by the elastic tension of the artificial quadriceps, is restrained and gradual, instead of being brusque, jerking, and excessive, as it otherwise would be. This is the first result obtained. In the second place, contraction of the antagonist having ceased, the artificial muscle which has been stretched returns upon itself in virtue of its elasticity, and restores the limb to the position of normal equilibrium.
For the act of walking, however, the artificial quadriceps would require to be made tense enough to resist flexion, and thus keep the limb in extension. An artificial anterior tibial muscle, however, would require to yield to the intact gastrocnemius while the heel was being raised from the floor; then its elastic force should be sufficient to retract the point of the foot in dorsal flexion during the pendulum movement which passively swings the leg forward. The tension of the artificial muscle should therefore be so adjusted that it can only be overcome by the active contraction of the gastrocnemius, and at the moment of greatest tension, immediately after stretching, it should be able to quite overcome the gastrocnemius, then relatively183 relaxed.
183 We say relatively, believing that the simultaneous contraction of antagonist muscles has been well established as a constant normal phenomenon.
The anterior tibial, gastrocnemius, and many other of the artificial muscles devised by Duchenne are still in use in the modified form given to them by Barwell. On the other hand, the action of the long peroneus in pronating the foot, and which Duchenne imitated by an elaborate artificial tendon following the exact course of the natural one, is to-day generally supplemented by the jointed shoe and laced bandage.
In paralysis of all the muscles surrounding a joint, when the limb is placid and no retractions by adapted atrophy have taken place, the artificial muscles can only serve to oppose the malpositions which are threatened from mechanical influences.
In the upper extremities prothetic apparatus has been principally used for progressive muscular atrophy. Paralysis of the wrist extensors is perhaps the only case in which the artificial muscle is required in anterior poliomyelitis. A string may be necessary to support the arm in paralysis of the deltoid, to avert luxation of the humerus.
Duchenne's ingenuity did not shrink from the difficult task of supplementing the muscles of the trunk. This he did by inserting the elastic spirals in corsets in a direction following that of the muscles paralyzed. Thus, a unilateral paralysis of the sacro-lumbalis may be met by a spiral splint running up one side of the spine; below, to the lateral posterior portion of a pelvic girdle. In bilateral paralysis two springs are used to antagonize the action of the abdominal muscles.
In Barwell's apparatus for the trunk184 India-rubber bands are again substituted for spiral springs. No attempt is made to imitate the direction of muscles, but the force is applied in any direction required to antagonize the pressure producing the deformity.185
184 Especially designed for habitual scoliosis, but applicable also to the paralytic deformity.
185 Volkmann (loc. cit., p. 778) thinks that the force of Barwell's India-rubber straps, whether for scoliosis or club-foot apparatus, is inadequate, and much inferior to metallic springs.
It is always important to remember the rarity of scoliosis caused by spinal paralysis of the trunk-muscles, and the much greater frequency with which this deformity occurs as a consequence of the paralytic shortening of a leg. A high shoe, equalizing the length of the lower extremities, is then the simple and efficient remedy.
In cases of long standing, even when the scoliosis is due to this cause, certain muscles on the concave side of the curve may become so retracted and rigid as to require tenotomy. Before this operation it is necessary to put the rigid muscles on the stretch as much as possible; and this may be done, if necessary, by means of Sayre's hanging apparatus. After this operation the spine may be straightened out with ease—an important distinction from advanced habitual scoliosis, where the alteration in the shape of the vertebræ defeats all attempts at rectification. The position may be maintained by elastic straps or corsets and by removing the condition which has led to the deformity.
Seeligmüller criticises too unfavorably the entire system of elastic tension in the prophylaxis and treatment of paralytic deformities. He quotes Duchenne's admission, that in certain cases traction upon rigidly-retracted tissues becomes insupportably painful, and must be abandoned. It is in these cases that tenotomy becomes an indispensable preliminary to the use of apparatus. Sayre insists that the necessity for tenotomy is indicated when pressure on the rigid muscle is followed by instantaneous spasmodic contraction in the affected or neighboring muscles. He declares that such contractions indicate reflex irritations, show that the muscle has undergone structural change, and that any attempt to stretch or lengthen it would be followed by an excess of irritation and pain.
This explanation can hardly be accepted, since muscles, whether imperfectly or not at all paralyzed, which from position and adapted atrophy have become retracted, have necessarily undergone structural changes. The greater these changes, the greater the diminution of reflex excitability; and in any muscle completely paralyzed and degenerated this is entirely lost. If, however, the afferent nerves retain enough vitality, if the muscle be slightly paralyzed or altogether intact, then irritation of its tendon by stretching may serve to excite contractions in the belly of the muscle. The possibility of such spinal reflexes is demonstrated by the now familiar phenomenon of the tendon reflex in various spinal diseases.186 The contractions must be painful from the impediments offered to the progress of the contracting nerve, and from the exaggeratedly vicious position into which they tend to force the limb. Under these circumstances prothetic apparatus must be deferred until section of the tendons has been made.
186 “Passive muscular tension excites tonic contraction in a muscle, and this action may, in abnormal conditions, be excessive, as in the myelitic contractions (so-called tendon reflexes).... The afferent nerves commence in the fibrous tissues of the muscle, and seem to be especially stimulated by extension” (Gowers, On Epilepsy, 1881, p. 97).