SYPHILITIC AFFECTIONS OF THE NERVE-CENTRES.
BY H. C. WOOD, M.D., LL.D.
Introduction.
Syphilitic affections of the nerve-centres are best studied by separating those of the spinal cord from those of the cerebrum, and in the present article this natural division of the subject is adopted. Further, cerebral syphilis in its most characteristic or gummatous form usually attacks the brain-membranes, or perhaps in some cases the perivascular sheaths of the vessels, and only secondarily affects the tissue of the brain itself. The question of the occurrence of specific disease of the brain-cortex is so important that it shall have a separate discussion. It is perfectly well proven that with or without other brain lesion the vessels of the brain may undergo an atheromatous degeneration as the direct result of a syphilitic dyscrasia; but such disease links itself on the one hand with the subject of syphilitic disease of the general vascular system, and on the other hand with cerebral apoplexies, softenings, and other degenerations. Moreover, the space here allotted to brain syphilis is very insufficient. I, therefore, shall not enter upon the further discussion of syphilitic degeneration of the brain-vessels. The etiology of brain and spinal syphilis is best discussed under one heading.
GENERAL ETIOLOGY.—We do not know why in any individual case syphilis selects one portion of the nervous centres rather than another for attack; indeed, it is only rarely that any exciting cause can be discovered.
It is not unnatural to expect that any agency which is capable of exciting an inflammation of a nerve-centre may, when present in a syphilitic person, provoke a specific disease of such centre. Thus, thermic fever is a very common cause of chronic meningitis, and in the Journ. de Méd. et Chir. (Paris, 1879, p. 191) a case is reported in which cerebral syphilis followed a sunstroke; I have myself seen one similar instance, and in Roberts's case of precocious cerebral syphilis (see p. 804) the first convulsion came whilst the man was fishing on a very hot day, and may have been precipitated by the exposure.
Blows and other traumatisms would be expected to figure largely as exciting causes of nervous syphilis, but they, in fact, are only rarely present. I have seen one or two cases of specific brain disease attributed to violence by the patient, and several cases of possibly specific spinal disease—one in which a poliomyelitis followed a fall on the ice; one in which, after a fall from a cart and marked spinal concussion, a local myelitis developed;1 and one of a general myelitis following an injury by a horse. The only records of such cases are those of Broadbent2 and those collected by Heubner.3
1 Univers. Hosp. Dispen. Service-Book, x., 1875, p. 58.
2 Lond. Lancet, 1876, ii. p. 741.
3 Ziemssen's Encyclopædia, xii. 301.
Various authorities attach much influence to over-study and other forms of cerebral strain in exciting brain syphilis. Engelstedt is stated to have reported cases having such etiological relations, and Fournier4 affirms that he has especially seen the disease in professional men and other persons habitually exercising their brains to excess. Neither in private nor public practice have I met with an instance where over-brainwork could be considered a distinct etiological factor, whilst I have seen some hundreds of cases from amongst the laboring classes, in whom the intellectual faculties are chiefly dormant.
4 La Syphilis du Cerveau.
The drift of the evidence in medical literature is so pronounced, and so in accord with my own experience, that I believe it may be positively affirmed that in the vast majority of cases of nervous syphilis no exciting cause can be found.
Inherited syphilis seems to be less prone than the acquired diathesis to attack the nervous system, but is certainly capable of so acting. As early as 1779, Joseph Glenck5 reported a case of a girl, six years old, cured by a mercurial course of an epilepsy of three years' standing and of other manifestations of hereditary syphilis. Graefe found gummatous tumors in the cerebrum of a child nearly two years old.6 O. Huebner7 details the occurrence of pachymeningitis hæmorrhagica in a syphilitic infant under a year old. Hans Chiari8 reports a case in which very pronounced syphilitic degeneration of the brain-vessels was found in a child fourteen months old. Both Barlow9 and T. S. Dowse10 report cases of nerve syphilis in male infants of fifteen months. For other similar cases the reader is referred to an article by J. Parrott,11 and to a paper by M. E. Troisier.12
5 Doctrina de Morbis Venereis, Vienna.
6 Arch. f. Ophthalm., Bd. i. Erst Abth.
7 Virchow's Archiv, Bd. lxxxiv. 269.
8 Wien. Med. Wochenschrift, xxxi. 1881, 17.
9 Lond. Patholog. Soc. Trans., 1877.
10 The Brain and its Diseases, vol. i. p. 76.
11 Archiv. de Physiologie, 1871-72, p. 319; also to his “Leçons sur le Syphilis hered.,” Progrès méd., 1877 and 1878.
12 Arch. de Tocologie, x. 411.
Recorded cases prove decisively that even after puberty specific nervous affections may primarily attack the unfortunate offspring. Thus, Nettleship reports13 the development of a cerebral gumma in a girl of ten years, and J. A.. Ormerod14 of a tumor of the median nerve (probably gummatous) in a woman of twenty-three, both the subjects of inherited syphilis. Thomas S. Dowse15 details a case of cerebral gumma at the age of ten years, and Samuel Wilks16 one of epilepsy, from inherited taint, in a boy of fourteen. J. Hughlings-Jackson reports17 paraplegia with epilepsy in a boy of eight, hemiplegia in a girl of eighteen, and hemiplegia in a woman of twenty-two;18 the nervous affection in each case being associated with or dependent upon inherited syphilis. E. Mendel reports19 a case of a child who had inherited syphilis, and developed in her fifteenth year a maniacal attack with hallucinations. I have seen cerebral syphilis occur at twenty-one years of age as the first evident outbreak of the inherited disorder.
13 Trans. Lond. Path. Soc., xxxii. 13.
14 Ibid., p. 14.
15 Loc. cit., p. 71.
16 Lectures on Dis. of Nerv. Syst., Philada., 1878, p. 333.
17 Journ. Ment. and Nerv. Diseases, 1875, p. 516.
18 Brit. Med. Journal, May 18, 1872.
19 Archiv f. Psychiatrie, Bd. i. 313.
When a nervous affection develops first at a comparatively late period, and no very apparent evidences of the inherited taint are present, there is great danger of the case being misunderstood; indeed, in some instances an immediate diagnosis may be scarcely possible. It is probable that in most of the reported recoveries from alleged tubercular meningitis the disease has been syphilitic.
Some time since I saw, in an orphan of fourteen, a chronic basal meningitis, and in the absence of any history and of any evidences of syphilis gave the fatal prognosis of tubercular disease; but, to my astonishment, under the long-continued and free use of iodide of potassium complete recovery occurred. Another child, reported by a very good practitioner as cured of tubercular meningitis, and afterward for a long time under my own care, I believe suffered from hereditary syphilis. Cases of this character have also been reported by F. Dreyfous.20
20 Revue mensuelle des Malad. des Enfants, 1883, i. 497; see also Gaz. hébdom. Sci. méd. de Montpellier, 1883, v. 89.
It is of course very important to diagnose between a tubercular meningitis and one due to hereditary syphilis. Without a history certainty is not possible, but a general indefiniteness of symptoms and slowness of progression should arouse suspicion, especially if the absence of the pulse-retardation indicated that the vault rather than the base of the cranium was involved.
The relation of inherited syphilis to various nervous affections not distinctly specific cannot yet be determined. Arrested development, and the consequent epilepsy, idiocy,21 early brain sclerosis, are probably sometimes due to the inheritance; and the cases collected by E. Mendel22 show that chronic hydrocephalus is frequently of specific origin.23
21 See Brain, vol. vii. 409.
22 Archiv f. Psychiatrie, Bd. i. 309.
23 See, also, Virchow's Archiv, Bd. xxxviii. p. 129.
Another very important question connected with the etiology of these disorders is as to the time of their development. Nervous diseases following acquired syphilitic infection certainly belong to the advanced stages of the disorder. Huebner reports24 a case in which thirty years elapsed between the contraction of the chancre and the nervous explosion. I have seen a similar period of thirty years. Fournier reports intervals of twenty-five years, and thinks from the third to the tenth year is the period of maximum frequency of nervous accidents.
24 Ziemssen's Encyclopædia, xii. 298, New York ed.
The fact that nervous syphilis may occur many years after the cessation of all apparent evidences of the diathesis is of great practical importance, especially as the nervous system is more prone to be attacked when the secondaries have been very light than when the earlier manifestations have been severe. I have repeatedly seen nervous syphilis in persons whose secondaries have been so slight as to have been entirely overlooked or forgotten, and who honestly asserted that they never had had syphilis, although they acknowledged to gonorrhœa or to repeated exposure, and confessed that their asserted exemption was due to good fortune rather than to chastity.
The following citations prove that this experience is not peculiar. Dowse25 says: “Often have I had patients totally ignorant of having at any time acquired or experienced the signs or symptoms of syphilis in its primary and secondary stages, yet the sequelæ have been made manifest in many ways, particularly in many of the obscure diseases of the nervous system.” Buzzard26 reports a case of nervous syphilis where the patient was unconscious of the previous existence of a chancre or of any secondaries. Rinecker also calls attention27 to the frequency of nervous syphilis in persons who afford no distinct history of secondary symptoms.
25 The Brain and its Diseases, London, 1879, vol. i. p. 7.
26 Syphilitic Nervous Affections, London, 1874, p. 80.
27 Archiv f. Psychiatrie, vii. p. 241.
Although syphilis is prone to attack the nervous system many years after infection, it would be a fatal mistake to suppose that nervous disease may not rapidly follow the chancre. What is the minimum possible intermediate period we do not know, but it is certainly very brief, as is shown by the following cases of this so-called precocious nervous syphilis. Alfrik Ljunggrén of Stockholm reports28 the case of H. R——, who had a rapidly-healed chancre in March, followed in May of the same year by a severe headache, mental confusion, and giddiness. Early in July H. R—— had an epileptic attack, but was finally cured by active antisyphilitic treatment. Although the history is not explicit, the nervous symptoms appear to have preceded the development of distinct secondaries other than rheumatic pains.
28 Archiv f. Dermatol. u. Syphilis, 1870, ii. p. 155.
Davaine is said29 to have seen paralysis of the portio dura “a month after the first symptoms of constitutional syphilis.” E. Leyden30 found advanced specific degeneration of the cerebral arteries in a man who had contracted syphilis one year previously. R. W. Taylor details a case in which epilepsy occurred five months after the infection.31 In the case of M. X——, reported by Ad. Schwarz,32 headache came on the fortieth day after the appearance of the primary sore, and a hemiplegia upon the forty-sixth day. S. L——33 had a paralytic stroke without prodromes six months after the chancre. A. P. L——34 had an apoplectic attack seven months after the chancre; A. S——, one five months after her chancre. In a case which recently occurred in the practice of A. Sydney Roberts of this city the chancre appeared after a period of incubation of twenty-six days, and two months and eight days subsequent to this came the first fit; eight days after the first the second convulsion occurred, with a distinct aura, which preceded by some minutes the unconsciousness. An interesting observation in this connection is that of Ern. Gaucher35 of a spinal syphilis occurring six months after the appearance of a chancre.
29 Buzzard, Syphilitic Nervous Affections, London, 1874.
30 Zeitschrift f. klin. Med., Bd. v. 165.
31 Journ. Nervous and Mental Dis., 1876, p. 38.
32 De l'Hémiplegia syphilitique Prêcoce, Inaug. Diss., Paris, 1880.
33 Ibid.
34 Ibid.
35 Revue de Méd., 1882, ii. 678.
This citation of cases might be much extended, but is sufficient to show that nervous syphilis occurs not very rarely within six months after infection, and may be present in two months.
Gummatous Brain Syphilis.
CLINICAL HISTORY.—Brain syphilis of the type now under consideration may declare itself with great suddenness. An apoplectic attack, a convulsive paroxysm, a violent mania, or a paralytic stroke may be the first detected evidence of the disease. In most of these cases the coming storm ought to have been foreseen, and to a greater or less degree averted. The onset of cerebral syphilis is, however, generally more gradual, the symptoms coming on slowly and successively. Proper treatment, instituted at an early stage, is usually successful, so that a careful study of these prodromes is most important. They are generally such as denote cerebral disturbance, and, although they should excite suspicion, are not diagnostic, except as occurring in connection with a specific history or under suspicious circumstances.
Headache, slight failure of memory, unwonted slowness of speech, general lassitude, and especially lack of willingness to mental exertion, sleeplessness or excessive somnolence, attacks of momentary giddiness, vertiginous feelings when straining at stool, yelling or in any way disturbing the cerebral circulation, alteration of disposition,—any of these, and, a fortiori, several of them, occurring in a syphilitic subject, should be the immediate signal of alarm, and lead to the examination of the optic discs, for in some cases the eye-ground will be found altered even during the prodromic stage. Of course if choked disc be found the diagnosis becomes practically fixed, but the absence of choked disc is no proof that the patient is free from cerebral syphilis. In regard to the individual prodromic symptoms, my own experience does not lend especial importance to any one of them, although, perhaps, headache is the most common. There is one symptom which may occur during the prodromic stage of cerebral syphilis, but is more frequent at a later stage—a symptom which is not absolutely characteristic of the disease, but which, when it occurs in a person who is not hysterical, should give rise to the strongest suspicion. I refer to the occurrence of repeated, partial, passing palsies. A momentary weakness of one arm, a slight drawing of the face disappearing in a few hours, a temporary dragging of the toe, a partial aphasia which appears and disappears, a squint which to-morrow leaves no trace, may be due to a non-specific brain tumor, to miliary cerebral aneurisms, or to some other non-specific affection; but in the great majority of cases where such phenomena occur repeatedly the patient is suffering from syphilis or hysteria.
The first type or variety of the fully-formed syphilitic meningeal disease to which attention is here directed is that of an acute meningitis. I am much inclined to doubt whether an acute syphilitic meningitis can ever develop as a primary lesion—whether it must not always be preceded by a chronic meningitis or by the formation of a gummatous tumor; but it is very certain that acute meningitis may develop when there have been no apparent symptoms, and may therefore seem to be abrupt in its onset. Some years ago I saw, in consultation, a man who in the midst of apparent health was attacked by violent meningeal convulsions, with distinct evidences of acute meningitis. He was apparently saved from death by very heroic venesection, but after his return to consciousness developed very rapidly a partial specific hemiplegia, showing that a latent gumma had probably preceded the acute attack. On the other hand, an acute attack is liable at any time to supervene upon a chronic syphilitic meningitis. At the University Hospital dispensary I once diagnosed chronic cerebral syphilis in a patient who the next day was seized with violent delirium, with convulsions and typical evidences of acute meningitis, and died four or five days afterward. At the autopsy an acute meningitis was found to have been engrafted on a chronic specific lesion of a similar character. In the case reported by Gamel,36 in which intense headache, fever, and delirium came on abruptly in an old syphilitic subject and ended in general palsy and death, the symptoms were found to depend upon an acute meningitis secondary to a large gumma.
36 Tumeurs gommeuses du Cerveau, Inaug. Diss., Montpellier, 1875.
In this connection may well be cited the observation of Molinier37 in which violent delirium, convulsions, and coma occurred suddenly. A very curious case is reported by D. A. Zambaco38 in which attacks simulating acute meningitis occurring in a man with a cerebral gummatous tumor appear to have been malarial. In such a case the diagnosis of a malarial paroxysm could only be made out by the presence of the cold stage, the transient nature of the attack, its going off with a sweat, its periodical recurrence, and the therapeutic effect on it of quinine.
37 Revue méd. de Toulouse, xiv. 1880, 341.
38 Des Affections nerveuses-syphilitiques, Paris, 1862, p. 485.
In the cases of chronic brain syphilis which have come under my observation, most usually after a greater or less continuance of prodromes such as have been mentioned, epileptic attacks have occurred with a hemiplegia, or a monoplegia, which is almost invariably incomplete and usually progressive; very frequently diplopia is manifested before the epilepsy, and on careful examination is found to be due to weakness of some of the ocular muscles. Not rarely oculo-motor palsy is an early and pronounced symptom, and a marked paralytic squint is very common. Along with the development of these symptoms there is almost always distinct failure of the general health and progressive intellectual deterioration, as shown by loss of memory, failure of the power to fix the attention, mental bewilderment, and perhaps aphasia. If the case convalesce under treatment, the amelioration is gradual, the patient travelling slowly up the road he has come down. If the case end fatally, it is usually by a gradual sinking into complete paralysis, or the patient is carried off by an acute inflammatory exacerbation, or, as in two of my cases, amelioration may be rapidly occurring and a very violent epileptic fit produce a sudden fatal asphyxia. Death from brain-softening around the tumor is not infrequent, but a fatal apoplectic hemorrhage is rare.
The clinical varieties of cerebral meningeal syphilis are so polymorphic and kaleidoscopic that it is almost impossible to reduce them to order for descriptive purposes. Fournier separates them into the cephalic, congestive, epileptic, aphasic, mental, and paralytic, but scarcely facilitates description by so doing. Heubner makes the following types:
"1. Psychical disturbances, with epilepsy, incomplete paralysis (seldom of the cranial nerves), and a final comatose condition, usually of short duration.
"2. Genuine apoplectic attacks with succeeding hemiplegia, in connection with peculiar somnolent conditions, occurring in often-repeated episodes; frequently phenomena of unilateral irritation, and generally at the same time paralyses of the cerebral nerves.
"3. Course of the cerebral disease similar to paralytica dementia.”
In regard to these types, the latter seems to me clear and well defined, but contains those cases which I shall discuss under the head of Cortical Disease.
Meningeal syphilis as seen in this country does not conform rigidly with the other asserted types, although there is this much of agreement, that when the epilepsy is pronounced the basal cranial nerves are not usually paralyzed, the reason of this being that epilepsy is especially produced when the gummatous change is in the ventricles or on the upper cortex. In basal affections the epileptoid spells, if they occur at all, are usually of the form of petit mal; but this rule is general, not absolute. The apoplectic somnolent form of cerebral syphilis, for some reason, is rare in this city, and it seems necessary to add to those of Heubner's a fourth type to which a large proportion of our cases conform. This type I would characterize as follows:
4. Psychical disturbance without complete epileptic convulsions, associated with palsy of the basal nerves and often with partial hemiplegia.
The most satisfactory way of approaching this subject is, however, to study the important symptoms in severalty, rather than to attempt to group them into recognizable varieties of the disease; and this method I shall here adopt.
Headache is the most constant and usually the earliest symptom of meningeal syphilis; but it may be absent, especially when the lesion is located in the reflexions of the meninges which dip into the ventricles, or when the basal gumma is small and not surrounded with much inflammation. The length of time it may continue without the development of other distinct symptoms is remarkable. In one case39 at the University Dispensary the patient affirmed that he had had it for four years before other causes of complaint appeared. It sometimes disappears when other manifestations develop. It varies almost indefinitely in its type, but is, except in very rare cases, at least so far paroxysmal as to be subject to pronounced exacerbations. In most instances it is entirely paroxysmal; and a curious circumstance is, that very often these paroxysms may occur only at long intervals: such distant paroxysms are usually very severe, and are often accompanied by dizziness, sick stomach, partial unconsciousness, or even by more marked congestive symptoms. The pain may seem to fill the whole cranium, may be located in a cerebral region, or fixed in a very limited spot. Heubner asserts that when this headache can be localized it is generally made distinctly worse by pressure at certain points, but my own experience is hardly in accord with this. Any such soreness plainly cannot directly depend upon the cerebral lesion, but must be a reflex phenomenon or due to a neuritis. According to my own experience, localized soreness indicates an affection of the bone or of its periosteum. In many cases, especially when the headache is persistent, there are distinct nocturnal exacerbations.
39 Book Y., p. 88, 1879.
It will be seen that there is nothing absolutely characteristic in the headache of cerebral syphilis; but excessive persistency, apparent causelessness, and a tendency to nocturnal exacerbation should in any cephalalgia excite suspicion of a specific origin—a suspicion which is always to be increased by the occurrence of slight spells of giddiness or by delirious mental wandering accompanying the paroxysms of pain. When an acute inflammatory attack supervenes upon a specific meningeal disease it is usually ushered in by a headache of intolerable severity.
When the headache in any case is habitually very constant and severe, the disease is probably in the dura mater or periosteum; and this probability is much increased if the pain be local and augmented by firm, hard pressure upon the skull over the seat of the pain.
Disorders of Sleep.—There are two antagonistic disorders of sleep, either of which may occur in cerebral syphilis, but which have only been present in a small proportion of the cases that I have seen. Insomnia is more apt to be troublesome in the prodromic than in the later stages, and is only of significance when combined with other more characteristic symptoms. A peculiar somnolence is of much more determinate import. It is not pathognomonic of cerebral syphilis, yet of all the single phenomena of this disease it is the most characteristic. Its absence is of no import in the theory of an individual case.
As I have seen it, it occurs in two forms: In the one variety the patient sits all day long or lies in bed in a state of semi-stupor, indifferent to everything, but capable of being aroused, answering questions slowly, imperfectly, and without complaint, but in an instant dropping off again into his quietude. In the other variety the sufferer may still be able to work, but often falls asleep while at his tasks, and especially toward evening has an irresistible desire to slumber, which leads him to pass, it may be, half of his time in sleep. This state of partial sleep may precede that of the more continuous stupor, or may pass off when an attack of hemiplegia seems to divert the symptoms. The mental phenomena in the more severe cases of somnolency are peculiar. The patient can be aroused—indeed in many instances he exists in a state of torpor rather than of sleep; when stirred up he thinks with extreme slowness, and may appear to have a form of aphasia; yet at intervals he may be endowed with a peculiar automatic activity, especially at night. Getting out of bed; wandering aimlessly and seemingly without knowledge of where he is, and unable to find his own bed; passing his excretions in a corner of the room or in other similar place, not because he is unable to control his bladder and bowels, but because he believes that he is in a proper place for such act,—he seems a restless nocturnal automaton rather than a man. In some cases the somnolent patient lies in a perpetual stupor.
An important fact in connection with the somnolence is that it may develop suddenly without marked premonition. Thus in a case reported by J. A. Ormerod40 a man who had been in good health, save only for headache, awoke one morning in a semi-delirious condition, and for three days slept steadily, only arousing for meals; after this there was impairment of memory and mental faculties, but no more marked symptoms.
40 Brain, vol. v. 260.
Apathy and indifference are the characteristics of the somnolent state, yet the patient will sometimes show excessive irritability when aroused, and will at other periods complain bitterly of pain in his head, or will groan as though suffering severely in the midst of his stupor—at a time, too, when he is not able to recognize the seat of the pain. I have seen a man with a vacant, apathetic face, almost complete aphasia, persistent heaviness and stupor, arouse himself when the stir in the ward told him that the attending physician was present, and come forward in a dazed, highly pathetic manner, by signs and broken utterances begging for something to relieve his head. Heubner speaks of cases in which the irritability was such that the patient fought vigorously when aroused; this I have not seen.
This somnolent condition may last many weeks. T. Buzzard41 details the case of a man who after a specific hemiplegia lay silent and somnolent for a month, and yet finally recovered so completely as to win a rowing-match on the Thames. I have seen a fair degree of recovery after a somnolence of four months' duration.
41 Clinical Lectures on Dis. Nerv. Syst., London, 1882.
In its excessive development syphilitic stupor puts on the symptoms of advanced brain-softening, to which it is indeed often due. Of the two cases with fatal result of which I have notes, one at the autopsy was found to have symmetrical purulent breaking down of the anterior cerebral lobes; the other, softening of the right frontal and temporal lobes, due to the pressure of a gummatous tumor, and ending in a fatal apoplexy.
This close connection with cerebral softening explains the clinical fact that apoplectic hemorrhage is very apt to end the life in these cases of somnolent syphilis. But a prolonged deep stupor in persons suffering from cerebral syphilis does not prove the existence of extensive brain-softening, and is not incompatible with subsequent complete recovery. As an element of prognosis it is of serious but not of fatal import.
Paralysis.—When it is remembered that a syphilitic exudation may appear at almost any position in the brain, that spots of encephalic softening are a not rare result of the infection, that syphilitic disease is a common cause of cerebral hemorrhage, it is plain that a specific palsy may be of any conceivable variety, and affect either the sensory, motor, or intellectual sphere. The mode of onset is as various as the character of the palsy. The attack may be instantaneous, sudden, or gradual. The gradual development of the syphilitic gumma would lead us, a priori, to expect an equally gradual development of the palsy; but experience shows that in a large proportion of the cases the paralysis appears suddenly, with or without the occurrence of an apoplectic or epileptic fit. Under these circumstances it will be usually noted that the resulting palsy is incomplete; in rare instances it may be at its worst when the patient awakes from the apoplectic seizure, but usually it progressively increases for a few hours, and then becomes stationary. These sudden partial palsies probably result from an intense congestion around the seat of disease or from stoppage of the circulation in the same locality; whatever their mechanism may be, it is important to distinguish them from palsies which are due to hemorrhage. I believe this can usually be done by noting the degree of paralysis.
A suddenly-developed, complete hemiplegia or other paralysis may be considered as in all probability either hemorrhagic or produced by a thrombus so large that the results will be disorganization of the brain-substance, and a future no more hopeful than that of a clot. On the other hand, an incomplete palsy may be rationally believed to be due to pressure or other removable cause; and this belief is much strengthened by a gradual development. The bearing of these facts upon prognosis it is scarcely necessary to point out.
Although the gummata may develop at almost any point, they especially affect the base of the brain, and are prone to involve the nerves which issue from it. Morbid exudations, not tubercular or syphilitic, are rare in this region. Hence a rapidly but not abruptly appearing strabismus, ptosis, dilated pupil, or any paralytic eye symptom in the adult is usually of syphilitic nature. Syphilitic facial palsy is not so frequent, whilst paralysis of the nerve from rheumatic and other inflammation within its bony canal is very common. Paralysis of the facial nerve may therefore be specific, but existing alone is of no diagnostic value. Since syphilitic palsies about the head are in most instances due to pressure upon the nerve-trunks, the electrical reactions of degeneration are present in the affected muscles.
There is one peculiarity about specific palsies which has already been alluded to as frequently present—namely, a temporary, transient, fugitive, varying character and seat. Thus an arm may be weak to-day, strong to-morrow, and the next day feeble again, or the recovered arm may retain its power and a leg fail in its stead. These transient palsies are much more apt to involve large than small brain territories. The explanation of their largeness, fugitiveness, and incompleteness is that they are not directly due to clots or other structural changes, but to congestions of the brain-tissues in the neighborhood of gummatous exudations. Squint due to direct pressure on a nerve will remain when the accompanying monoplegia due to congestion disappears.
Motor palsies are more frequent than sensory affections in syphilis, but hemianæsthesia, localized anæsthetic tracts, indeed any form of sensory paralysis, may occur. Numbness, formications, all varieties of paræsthesia, are frequently felt in the face, body, or extremities. Violent peripheral neuralgic pains are rare, and generally when present denote neuritis. Huguenin, however, reports42 a severe trigeminal anæsthesia dolorosa, which was found, after death from intercurrent disease, to have depended upon a small gumma pressing upon the Gasserian ganglion. A somewhat similar case has also been reported by Allen McLane Hamilton.43
42 Schwiez. Corr. Blät., 1875.
43 Alienist and Neurologist, iv. 58.
The special senses are liable to suffer from the invasion of their territories by cerebral syphilis, and the resulting palsies follow courses and have clinical histories parallel to those of the motor sphere. The onset may be sudden or gradual, the result temporary or permanent. Charles Mauriac44 reports a case in which the patient was frequently seized with sudden attacks of severe frontal pain and complete blindness lasting from a quarter to half an hour; at other times the same patient had spells of aphasia lasting only for one or two minutes. I have seen two cases of nearly complete deafness developing in a few hours in cerebral syphilis, and disappearing abruptly after some days. Like other syphilitic palsies, therefore, paralyses of special senses may come on suddenly or gradually, and may occur paroxysmally.
44 Loc. cit., p. 31.
Among the palsies of cerebral syphilis must be ranked aphasia. An examination of recorded cases shows that syphilitic aphasia is subject to vagaries and laws similar to those connected with other specific cerebral palsies. It is usually a symptom of advanced disease, but may certainly develop as one of the first evidences of cerebral syphilis. Coming on after an apoplectic or epileptic fit, it may be complete or incomplete: owing to the smallness of the centre involved and the ease with which its function is held in abeyance, a total loss of word-thought is not so decisive as to the existence of cerebral hemorrhage as is a total motor palsy. Like hemiplegia or monoplegia, specific aphasia is sometimes transitory and paroxysmal. Buzzard45 records several such cases. Mauriac46 details a very curious case in which a patient, after long suffering from headache, was seized by sudden loss of power in the right hand and fingers, lasting about ten minutes only, but recurring many times a day. After this had continued some time the paroxysms became more completely paralytic, and were accompanyed by loss of the power of finding words, the height of the crises in the palsy and aphasia being simultaneously reached. For a whole month these attacks occurred five or six times a day, without other symptoms except headache, and then the patient became persistently paralytic and aphasic, but finally recovered. To describe the different forms of specific aphasia and their mechanism of production would be to enter upon a discussion of aphasia itself—a discussion out of place here. Suffice it to say that every conceivable form of the disorder may be induced by syphilis.
45 Loc. cit., p. 81.
46 Aphasie et Hemiplégia droite Syphilit., Paris, 1877.
Owing to the centres of speech being situated in the cortical portion of the brain, aphasia in cerebral syphilis is very frequently associated with epilepsy. Of course right-sided palsy and aphasia are united in syphilitic as in other disorders. If, however, the statistics given by Tanowsky47 be reliable, syphilitic aphasia is associated with left-sided hemiplegia in a most extraordinarily large proportion. Thus in 53 cases collected by Tanowsky, 18 times was there right-sided hemiplegia, and 14 times left-sided hemiplegia, the other cases being not at all hemiplegic. Judging from the autopsy on a case reported in Mauriac's brochure, this concurrence of left-sided paralysis and aphasia depends partly upon the great frequency of multiple brain lesions in syphilis, and partly upon the habitual involvement of large territories of the gray matter secondarily to diseased membrane. An important practical deduction is that the conjoint existence of left hemiplegia and aphasia is almost diagnostic of cerebral syphilis.
47 L'Aphasie syphilitique.
Probably amongst the palsies may be considered the disturbances of the renal functions, which are only rarely met with in cerebral syphilis, and which are probably usually dependent upon the specific exudation pressing upon the vaso-motor centres in the medulla. Fournier speaks of having notes of six cases in which polyuria with its accompaniment, polydipsia, was present, and details a case in which the specific growth was found in the floor of the fourth ventricle. Cases have been reported of true saccharine diabetes due to cerebral syphilis,48 and I can add to these an observation of my own. The symptoms, which occurred in a man of middle age, with a distinct specific history, were headache, nearly complete hemiplegia, and mental failure, associated with the passage of comparatively small quantities of a urine so highly saccharine as to be really a syrup. Under the influence of the iodide of potassium the sugar in a few weeks disappeared from the urine.
48 Consult Servantié, Des Rapports du Diabète et de la Syphilis, Paris, Thèse, 1876; also, case reported by L. Putzel, New York Med. Record, xxv. 450.
Epilepsy.—Epileptic attacks are a very common symptom of meningeal syphilis, and are of great diagnostic value. The occurrence in an adult of an epileptic attack or of an apoplectic fit, or of a hemiplegia after a history of intense and protracted headache, should always excite grave suspicion.
Before I had read Fournier's work on Nervous Syphilis I taught that an epilepsy appearing after thirty years of age was very rarely, if ever, essential epilepsy, and unless alcoholism, uræmic poison, or other adequate cause could be found was in nine cases out of ten specific; and I therefore quote with satisfaction Fournier's words: “L'épilepsie vraie, ne fait jamais son premier dêbut à l'âge adulte, à l'âge mûr. Si un homme adulte, au dessus de 30, 35, à 40 ans, vient, à être pris pour la première fois d'une crise épileptique, et cela dans la cours d'une bonne santé apparente, il y a, je vous le répète, hui ou neuf chances sur dix pour que cette épilepsie soit d'origine syphilitique.”
Syphilitic epilepsy may occur either in the form of petit mal or of haut mal, and in either case may take on the exact characters and sequence of phenomena which belong to the so-called idiopathic or essential epilepsy. The momentary loss of consciousness of petit mal will usually, however, be found to be associated with attacks in which, although voluntary power is suspended, memory recalls what has happened during the paroxysm—attacks, therefore, which simulate those of hysteria, and which may lead to an error of diagnosis.
Even in the fully-developed type of the convulsions the aura is only rarely present. Its absence is not, however, of diagnostic value, because it is frequently not present in essential epilepsy, and it may be pronounced in the specific disease. It is said that when in an individual case the aura has once appeared the same type or form of approach of the convulsion is thereafter rigidly adhered to. The aura is sometimes bizarre: a severe pain in the foot, a localized cramp, a peculiar sensation, indescribable and unreal in its feeling, may be the first warning of the attack. An aura may affect a special sense. Thus, I have at present a patient whose attacks begin with blindness.
In many, perhaps most, cases of specific convulsions, instead of a paroxysm of essential epilepsy being closely simulated, the movements are in the onset, or more rarely throughout the paroxysm, unilateral; indeed, they may be confined to one extremity. This restriction of movement has been held to be almost characteristic of syphilitic epilepsy, but it is not so. Whatever diagnostic significance such restriction of the convulsion has is simply to indicate that the fit is due to a cortical organic lesion of some kind. Tumors, scleroses, and other organic lesions of the brain-cortex are as prone to cause unilateral or monoplegic epilepsy when they are not specific as when they are due to syphilis.
Sometimes an epilepsy dependent upon a specific lesion implicating the brain-cortex may be replaced by a spasm which is more or less local and is not attended with any loss of consciousness. Thus, in a case now convalescent in the University Hospital, a man aged about thirty-five offered a history of repeated epileptic convulsions, but at the time of his entrance into the hospital, instead of epileptic attacks, there was a painless tic. The spasms, which were clonic and occurred very many times a day, sometimes every five minutes, were very violent, and mostly confined to the left facial nerve distribution. The trigeminus was never affected, but in the severer paroxysms the left hypoglossal and spinal accessory nerves were profoundly implicated in all of their branches. Once, fatal asphyxia from recurrent laryngeal spasm of the glottis was apparently averted only by the free inhalation of the nitrite of amyl. The sole other symptom was headache, but the specific history was clear and the effect of antisyphilitic remedies rapid and pronounced.
It is very plain that such attacks as those just detailed are closely allied to epilepsy; indeed, there are cases of cerebral syphilis in which widespread general spasms occur similar to those of a Jacksonian epilepsy, except that consciousness is not lost, because the nervous discharge does not overwhelm the centres which are connected with consciousness.49 On the other hand, these epileptoid spasmodic cases link themselves to those in which the local brain affection manifests itself in contractions or persistent irregular clonic spasms. Contractures may exist and may simulate those of descending degeneration,50 but in my own experience are very rare.51
49 Case, Canada Med. and Surg. Journ., xi. 487.
50 Case, Centralbl. Nerv. Heilk., 1883, p. 1.
51 A case of syphilitic athetosis may be found in Lancet, 1883, ii. 989.
The clonic spasms of cerebral syphilis may assume a distinctly choreic type, or may in their severity simulate those of hysteria, throwing the body about violently.52 It is, to my mind, misleading, and therefore improper, to call such cases syphilitic chorea, as there is no reason for believing that they have a direct relation with ordinary chorea. They are the expression of an organic irritation of the brain-cortex, and are sometimes followed by paralysis of the affected member; in other words, the disease, progressing inward from the brain-membrane, first irritates, and then so invades a cortical centre as to destroy its functional power.53
52 See Allison, Amer. Med. Journ., 1877, 74.
53 Case, Chicago Med. Journ. and Exam., xlvi. 21.
Psychical Symptoms.—As already stated, apathy, somnolence, loss of memory, and general mental failure are the most frequent and characteristic mental symptoms of meningeal syphilis; but, as will be shown in the next chapter, syphilis is able to produce almost any form of insanity, and therefore mania, melancholia, erotic mania, delirium of grandeur, etc. etc. may develop along with the ordinary manifestation of cerebral syphilis, or may come on during an attack which has hitherto produced only the usual symptoms. Without attempting any exhaustive citation of cases, the following may be alluded to.
A. Erlenmeyer reports54 a case in which an attack of violent headache and vomiting was followed by paralysis of the right arm and paresis of the left leg, with some mental depression; a little later the patient suddenly became very cheerful, and shortly afterward manifested very distinctly delirium of grandeur with failure of memory. Batty Tuke reports55 a case in which, with aphasia, muscular wasting, strabismus, and various palsies, there were delusions and hallucinations. In the same journal56 S. D. Williams reports a case in which there were paroxysmal violent attacks of frontal headache. The woman was very dirty in her habits, only ate when fed, and existed in a state of hypochondriacal melancholy. Leiderdorf details a case with headache, partial hemiplegia, great psychical disturbance, irritability, change of character, marked delirium of grandeur, epileptic attacks, and finally dementia, eventually cured by iodide of potassium.57 Several cases illustrating different forms of insanity are reported by N. Manssurow.58
54 Die luëtischen Psychosen.
55 Journ. Ment. Sci., Jan., 1874, p. 560.
56 April, 1869.
57 Medicin Jahrbucher, xx. 1864, p. 114.
58 Die Tertiäre Syphilis, Wien, 1877.
That the attacks of syphilitic insanity, like the palsies of syphilis, may at times be temporary and fugitive, is shown by a curious case reported by H. Hayes Newington,59 in which, along with headache, failure of memory, and ptosis in a syphilitic person, there was a brief paroxysm of noisy insanity.
59 Journ. Ment. Sci., London, xix. 555.
DIAGNOSIS.—In a diagnosis of cerebral syphilis a correct history of the antecedents of the patients is of vital importance. Since very few of the first manifestations of the disorder are absolutely characteristic, whilst almost any conceivable cerebral symptoms may arise from syphilitic disease, treatment should be at once instituted on the appearance of any disturbance of the cerebral functions in an infected person.
Very frequently the history of the case is defective, and not rarely actually misleading. Patients often appear to have no suspicion of the nature of their complaint, and will deny the possibility of syphilis, although they confess to habitual unchastity. My own inquiries have been so often misleading in their results that I attach but little weight to the statements of the patient, and in private practice avoid asking questions which might recall unpleasant memories, depending upon the symptoms themselves for the diagnosis.
The general grounds of diagnosis have been sufficiently mapped out in the last section, but some reiteration may be allowable. After the exclusion of other non-specific disease, headache occurring with any form of ocular palsy or with a history of attack of partial monoplegia or hemiplegia, vertigo, petit mal, epileptoid convulsions, or disturbances of consciousness, or attacks of unilateral or localized spasms, should lead to the practical therapeutic test. Ocular palsies, epileptic forms of attacks occurring after thirty years of age, morbid somnolence, even when existing alone, are sufficient to put the practitioner upon his guard. It is sometimes of vital importance that the nature of the cephalalgia shall be recognized before the coming on of more serious symptoms; any apparent causelessness, severity, and persistency should arouse suspicion, to be much increased by a tendency to nocturnal exacerbations or by the occurrence of mental disturbance or of giddiness at the crises of the paroxysms. Not rarely there are very early in these cases curious, almost indefinable, disturbances of cerebral functions, which may be easily overlooked, such as temporary and partial failures of memory, word-stumbling, fleeting feelings of numbness or weakness, alterations of disposition. In the absence of hysteria an indefinite and apparently disconnected series of nerve accidents is of very urgent import. To use the words of Hughlings-Jackson, “A random association or a random succession of nervous symptoms is very strong warrant for a diagnosis of a syphilitic disease of the nervous system.” Cerebral syphilis occurring in an hysterical subject may be readily overlooked until fatal mischief is done. When any paralysis occurs a study of the reflexes may sometimes lead to a correct diagnosis. Thus in a hemiplegia the reflex on the affected side in cerebral syphilis is very frequently exaggerated, whilst in hysteria the reflexes are usually alike on both sides. When both motion and sensation are disturbed in an organic hemiplegia, the anæsthesia and motor paralysis occur on the same side of the body, whilst in hysteria they are usually on opposite sides.
In all cases of doubtful diagnosis the so-called therapeutic test should be employed, and if sixty grains of iodide of potassium per day fail to produce iodism, for all practical purposes the person may be considered to be a syphilitic. No less an authority than Seguin has denied the validity of this, but I believe, myself, that some of his reported cases were suffering from unsuspected syphilis. I do not deny that there are rare individuals who, although untainted, can resist the action of iodide, but in ten years' practice in large hospitals, embracing probably some thousands of cases, I have not met with more than one or two instances which I believed to be of such character. Of course in making these statements I leave out of sight persons who have by long custom become accustomed to the use of the iodide, for although in most cases such use begets increase of susceptibility, the contrary sometimes occurs. Of course the physician who should publicly assert that a patient who did not respond to the iodide had syphilis would be a great fool, but in my opinion the physician who did not act upon such a basis would be even more culpable.
PROGNOSIS.—Cerebral meningeal syphilis varies so greatly and so unexpectedly in its course that it is very difficult to establish rules for predicting the future in any given case. The general laws of prognosis in brain disease hold to some extent, but may always be favorably modified, and patients apparently at the point of death will frequently recover under treatment. The prognosis is not, however, as absolutely favorable as is sometimes believed, and especially should patients be warned of the probable recurrence of the affection even when the symptoms have entirely disappeared. The only safety after the restoration of health consists in an immediate re-treatment upon the recurrence of the slightest symptom. The occurrence of a complete, sudden hemiplegia or monoplegia is sufficient to render probable the existence of a clot, which must be subject to the same laws as though not secondary to a specific lesion. If a rapid decided rise of temperature occur in an apoplectic or epileptic attack, the prognosis becomes very grave. An epileptic paroxysm very rarely ends fatally, although it has done so in two of my cases.
The prognosis in gummatous cerebral syphilis should always be guardedly favorable. In the great majority of cases a more or less incomplete recovery occurs under appropriate treatment, and I have seen repeatedly patients who were unconscious, with urinary and fecal incontinence, and apparently dying, recover. Nevertheless, so long as there is any particle of gummatous inflammation in the membrane the patient is liable to sudden congestions of the brain, which may prove rapidly fatal, or he may die in a brief epileptic fit. On the one hand there is an element of uncertainty in the most favorable case, and on the other so long as there is life a positively hopeless prognosis is not justifiable.
PATHOLOGY.—Gummatous inflammation of the brain probably always has its starting-point in the brain-membranes, although it may be situated within the brain: thus, I have seen the gummatous tumors spring from the velum interpositum in the lateral ventricle. The disease most usually attacks the base of the brain, and is especially found in the neighborhood of the pons Varolii and the optic tract. It may, however, locate itself upon the vault of the cranium, and in my experience has seemed to prefer the anterior or motor regions. The mass may be well defined and roundish, but more usually it is spread out, irregular in shape, and more or less confluent with the substance of the brain beneath it. It varies in size from a line to several inches in length, and when small is prone to be multiple. The only lesion which it resembles in gross appearance is tubercle, from which it sometimes cannot be certainly distinguished without microscopic examination.
The large gummata have not rarely two distinct zones, the inner one of which is drier, somewhat yellowish in color, opaque, and resembles the region of caseous degeneration in the tubercle. The outer zone is more pinkish and more vascular, and is semi-translucent.
On microscopic examination the most characteristic structures are small cells, such as are found in gummatous tumors in other portions of the body. These cells are most abundant in the inner zone, which, indeed, may be entirely composed of them. In the centre of the tumor they are more or less granular and atrophied; in some cases the caseous degeneration has progressed so far that the centre of the gumma consists of minute acicular crystals of fat. In the external or peripheral zone of the tumor the mass may pass imperceptibly into the normal nerve tissue, and under these circumstances it is that it contains the spider-shaped cells or stellate bodies described by Jastrowitch, and especially commented upon by Charcot and Gombault and by Coyne. These are large cells containing an exaggerated nucleus and a granular protoplasm, which continues into multiple, branching, rigid, refracting prolongations, which prolongations are scarcely stained by carmine. Alongside of these cells other largish cells are often found without prolongations, but furnished with oval nuclei and granular protoplasm. Amongst these cells will be seen the true gummatous cells, as well as the more or less altered neuroglia and nerve-elements. In the perivascular lymphatic sheaths in the outer part of the gumma is usually a great abundance of small cells. The spider-shaped cells are probably hypertrophied normal cells of the neuroglia, and have been considered by Charcot and Gombault as characteristic of syphilitic gummata of the brain. In a solitary gumma, however, of considerable size from the neighborhood of the cerebellum, studied by Coyne and Peltier, there were no stellated cells. Coyne considers that their presence is due to their previous existence in the normal state of the regions affected by the gumma. Exactly what becomes of syphilitic gumma of the brain in cases of recovery it is difficult to determine. It is certain that they become softened and disappear more or less completely, and it is probable that the cicatrices or the small peripheral cysts which are not rarely found in the surfaces of the brain are sometimes remnants of gummatous tumors. In a number of cases collected by Gros and Lancereaux there were small areas of softened tissue or small calcareous and caseous masses or cerebral lacunæ corresponding to the cicatrices of softening or imperfect cysts, coincident with evidences of syphilis elsewhere. V. Cornil also states that he has found small areas of softening with well-established syphilitic lesions of the dura mater and cranium, but believes that the lacunæ or cysts depend rather upon chronic syphilitic lesions of cerebral arteries than upon gummatous inflammation.
When a gummatous tumor comes in contact with an artery, the latter is usually compressed and its walls undergo degeneration. The specific arteritis may pass beyond the limit of the syphilome and extend along the arterial wall. Not rarely there is under these circumstances a thrombus, and if the artery be a large one secondary softening of its distributive brain-area occurs.
TREATMENT.—The treatment of cerebral syphilis is best studied under two heads: First, the treatment of the accidents which occur in the course of the disease; second, the general treatment of the disease itself.
It must be remembered that in the great majority of cases in which death occurs in properly-treated cerebral syphilis the fatal result is produced by an exacerbation—or, as I have termed it, an accident—of the disease. Under these circumstances the treatment should be that which is adapted to the relief of the same acute affection when dependent upon other than specific cause. In a large proportion of cases the acute outbreak takes the form either of a meningitis or else of a brain congestion. In either instance when the symptoms are severe free bleeding should be at once resorted to. The amount of blood taken is of course to be proportionate to the severity of the symptoms and the strength of the patient. I have seen life saved by the abstraction of about a quart of blood, whilst in other cases a few ounces suffice. Care must be, of course, taken not to mistake a simple epileptic fit for a severe cerebral attack; but when this fit has been preceded by severe headache and is accompanied by stupor, with marked disturbance of the respiration, measures for immediate relief are usually required; and if the convulsions be perpetually repeated or if there be violent delirious excitement, the symptoms may be considered as very urgent. In taking blood the orifice should be large, so as to favor a rapid flow, and the bleeding be continued until a distinct impression is made upon the pulse. In some cases which I have seen in which the action of the heart continued to be violent after as much blood as was deemed prudent had been taken, good results were obtained by the hypodermic injection of three drops of the tincture of aconite-root every half hour until the reduction of the pulse and the free sweating indicated that the system was coming under the influence of the cardiac sedative.
Of course, I do not mean to encourage the improper or too free use of the lancet in these cases, but in the few fatal cases which I have seen I have almost invariably regretted that blood had not been taken at once very freely at the beginning of the acute attack. In most of these cases the symptoms had progressed too far for good to be achieved before I reached the patient. After venesection, or in feeble cases as a substitute for it, the usual measures of relief in cerebral congestion should be instituted. I shall not occupy space with a discussion of these measures, as they are in no way different from those to be employed in cases not syphilitic.
The most important part of the treatment of cerebral syphilis itself is antisyphilitic, and the practitioner is at once forced to select between the iodide of potassium and the mercurial preparations. In such choice it must be remembered that even a very small amount of syphilitic deposit in the brain may at any time cause a sudden congestion or other acute attack, and is therefore a very dangerous lesion. I have seen a cerebral syphilis which was manifested only by an epileptic attack occurring once in many months, and in which after death the affected membrane was found to be not larger than a quarter of a dollar, and the deposit not more than an eighth of an inch in thickness, suddenly produce a rapidly fatal congestion; and I have known a case fast progressing toward recovery suddenly ended by the too long continuance of the arrest of respiration during an epileptic fit. I have, myself, no doubt of the superiority of the mercurials over the iodide of potassium as a means of producing absorption of gummatous exudates; and as these exudates in the brain are so very dangerous, a mercurial course should in the majority of cases of cerebral syphilis be instituted so soon as the patient comes under the practitioner's care. When, however, there is a history of a recent prolonged free use of the mercurial, or when there is marked specific cachexia, the iodide should be chosen. Cachexia is, however, a distinctly rare condition in cerebral syphilis, the disease usually developing in those who have long had apparent immunity from the constitutional disorder. In my opinion the best preparation of the mercurial for internal use is calomel. It should be given in small doses, one-quarter of one grain every two hours, guarded with opium and astringents, so as to prevent as far as possible disturbance of the bowels, and should be continued until soreness of the teeth, sponginess of the gums, or other evidences of commencing ptyalism are induced. After this the dose of the mercurial should be so reduced as simply to maintain the slight impression which has been created, and the patient should be kept under the mercurial influence for some weeks.
A very effective method of using the mercury is by inunction, and where the surroundings of the patient are suitable the mercurial ointment may be substituted for the calomel. It should be applied regularly, according to the method laid down in my treatise on therapeutics. I have sometimes gained advantage by practising the mercurial unction and at the same time giving large doses of iodide of potassium internally.
After a mercurial course the iodide of potassium should always be exhibited freely, the object being not only to overcome the natural disease, but also to bring about the complete elimination of the mercury from the system. There is no use in giving the iodide in small doses; at least a drachm and a half should be administered in the twenty-four hours, and my own custom has been to increase this to three drachms unless evidences of iodism are produced. The compound syrup of sarsaparilla covers the disagreeable taste of the iodide of potassium better than any other substance of which I have knowledge. Moreover, I am well convinced that there is some truth in the old belief that the so-called “Woods” are of value in the treatment of chronic syphilis. I have seen cases in which both the iodide of potassium and the mercurials had failed to bring about the desired relief, but in which the same alteratives, when given along with the “Woods,” rapidly produced favorable results. The old-fashioned Zittmann's decoction, made according to the formula of the United States Dispensatory, may be occasionally used with very excellent effect. But I have gradually come into the habit of substituting a mixture of the compound fluid extract and the compound syrup of sarsaparilla in equal proportions. The syrup itself is too feeble to have any influence upon the system, but is here employed on account of its flavor. A favorite method of administration is to furnish the patient with two bottles—one containing a watery solution of the iodide of potassium of such strength that two drops represent one grain of the drug, and the other the sarsaparilla mixture above mentioned. From one to two drachms of the solution of the iodide may be administered in a tablespoonful of the sarsaparilla well diluted after meals. When the patient has been previously mercurialized, or there is any doubt as to the propriety of using mercurials, corrosive sublimate in small doses may be added to the solution of the iodide, so that one-tenth to one-fifteenth of a grain shall be given in each dose. I have never seen especial advantage obtained by the use of the iodides of mercury. They are no doubt effective, but are not superior to the simpler forms of the drug.
Syphilitic Disease of the Brain-Cortex.
The psychical symptoms which are produced by syphilis are often very pronounced in cases in which the paralysis, headache, epilepsy, and other palpable manifestations show the presence of gross brain lesions. In the study of syphilitic disease of the brain-membranes sufficient has been said in regard to these psychical disturbances, but the problem which now offers itself for solution is as to the existence or non-existence of syphilitic insanity—i.e. of an insanity produced by specific contagion without the obvious presence of gummatous disease of the brain-membranes. Very few alienists recognize the existence of a distinct affection entitled to be called syphilitic insanity, and there are some who deny that insanity is ever directly caused by syphilis. It is certain that insanity often occurs in the syphilitic, but syphilis is abundantly joined with alcoholism, poverty, mental distress, physical ruin, and various depressing emotions and conditions which are well known to be active exciting causes of mental disorder. It may well be that syphilis is in such way an indirect cause of an insanity which under the circumstances could not be properly styled syphilitic.
If there be disease of the brain-cortex produced directly by syphilis, of course such disease must give rise to mental disorders; and if the lesion be so situated as to affect the psychic and avoid the motor regions of the brain, it will produce mental disorder without paralysis—i.e. an insanity; again, if such brain disease be widespread, involving the whole cortex, it will cause a progressive mental disorder, accompanied by gradual loss of power in all parts of the body, and ending in dementia with general paralysis; or, in other words, it will produce an affection more or less closely resembling the so-called general paralysis of the insane, or dementia paralytica.
As a man having syphilis may have a disease which is not directly due to the syphilis, when a syphilitic person has any disorder there is only one positive way of determining during life how far said disorder is specific—namely, by studying its amenability to antisyphilitic treatment. In approaching the question whether a lesion found after death is specific or not, of course such a therapeutic test as that just given is inapplicable. We can only study as to the coexistence of the lesion in consideration with other lesions known to be specific. Such coexistence of course does not absolutely prove the specific nature of a nutritive change, but renders such nature exceedingly probable.
What has just been said foreshadows the method in which the subject in hand is to be here examined, and the present article naturally divides itself into two sections—the first considering the coexistence of anatomical alterations occurring in the cerebral substance with syphilitic affections of the brain-membranes or blood-vessels, the second being a clinical study of syphilitic insanity.
In looking over the literature of the subject I have found the following cases in which a cerebral sclerotic affection coincided with a gummatous disease of the membrane. Gros and Lancereaux60 report a case having a clear syphilitic history in which the dura mater was adherent to the skull. The pia mater was not adherent. Beneath, upon the vault of the brain, was a gelatinous exudation. The upper cerebral substance was indurated, and pronounced by Robin after microscopic examination to be sclerosed. At the base of the brain there were atheromatous arteries and spots of marked softening.
60 Affec. Nerv. Syphilis, 1861, p. 245.
Jos. J. Brown61 reports a case in which the symptoms were melancholia, excessive irritability, violent outbursts of temper, very positive delusions, disordered gait, ending in dementia. At the autopsy, which was very exhaustive, extensive syphilitic disease of the vessels of the brain and spinal cord was found. The pia mater was not adherent to the brain. The convolutions, particularly of the frontal and parietal lobes, were atrophied, with very wide sulci filled with bloody serum. The neuroglia of these convolutions was much increased, and “appeared to be more molecular than normal, the cells were degenerated, and in many places had disappeared, their places being only occupied by some granules.” These changes were most marked in the frontal convolutions.
61 Journ. Ment. Sci., July, 1875, p. 271.
H. Schule reports62 a very carefully and meritoriously studied case. The symptoms during life exactly simulated those of dementia paralytica. The affection commenced with an entire change in the disposition of the patient; from being taciturn, quiet, and very parsimonious, he became very excited, restless, and desiring continuously to buy in the shops. Then failure of memory, marked sense of well-being, carelessness and indifference for the future, developed consentaneously with failure of the power of walking, trembling of the hands, inequality of the pupils, and hesitating speech. There was next a period of melancholy, which was in time followed by continuous failure of mental and motor powers, and very pronounced delirium of grandeur, ending in complete dementia. Death finally occurred from universal palsy, with progressive increase of the motor symptoms. At the autopsy characteristic syphilitic lesions were found in the skull, dura mater, larynx, liver, intestines, and testicles. The brain presented the macroscopic and microscopic characters of sclerosis and atrophy; the neuroglia was much increased, full of numerous nuclei, the ganglion-cells destroyed. The vessels were very much diseased, some reduced to cords; their walls were greatly thickened, and full of long spindle-shaped cells, sometimes also containing fatty granules.
62 Allgem. Zeitschrift f. Psychiatrie, xxviii. 171, 172.
C. E. Stedman and Robt. T. Edes report63 a case in which the symptoms were failure of health, ptosis, trigeminal palsy with pain (anæsthesia dolorosa), finally mental failure with gradual loss of power of motion and sensation. At the autopsy the following conditions were noted: apex of the temporal lobe adherent to dura mater and softened; exuded lymph in neighborhood of optic chiasm; sclerosis of right Gasserian ganglion, as shown in a marked increase of the neuroglia; degeneration of the basal arteries of the brain.
63 American Journ. Med. Sciences, lxix. 433.
These cases are sufficient to demonstrate that sclerosis of the brain-substance not only may coexist with a brain lesion which is certainly specific in its character, but may also present the appearance of having developed pari passu with that lesion and from the same cause.
It has already been stated in this article that cerebral meningeal syphilis may coexist with various forms of insanity, and cases have been cited in proof thereof. It is of course very probable that in some of such cases there has been that double lesion of membrane and gray brain matter which has just been demonstrated by report of autopsies; but if we find that there is a syphilitic insanity, which exists without evidences of meningeal syphilis, and is capable of being cured by antispecific treatment, such insanity must be considered as representing the disease of the gray matter of the brain. Medical literature is so gigantic that it is impossible to exhaust it, but the following list of cases is amply sufficient to prove the point at issue—namely, that there is a syphilitic insanity which exists without obvious meningeal disease, and is capable of being cured by antisyphilitic treatment:
A study of the brief analyses of the symptoms just given shows that syphilitic disease of the brain may cause any form of mania, but that the symptoms, however various they may be at first, end almost always in dementia unless relieved.
Of all the forms of insanity, general paralysis is most closely and frequently simulated by specific brain disease. The exact relation of the diathesis to true, incurable, general paralysis it is very difficult to determine. It seems well established that amongst persons suffering from this disorder the proportion of syphilitics is not only much larger than normal, but also much larger than in other forms of insanity. Thus, E. Mendel64 found that in 146 cases of general paralysis, 109, or 75 per cent., had a distinct history of syphilis, whilst in 101 cases of various other forms of primary insanity only 18 per cent. had specific antecedents. H. Obersteiner has 1000 cases of mental disease,65 175 cases of dementia paralytica; of these, 21.6 per cent. had syphilis; moreover, of all the syphilitic patients 51.4 per cent. had dementia paralytica.
64 Progres. Paral. der Irren, Berlin, 1880.
65 Monatshefte f. prakt. Dermat., Dec., 1882.
Various opinions might be cited as to the nature of this relation between the two disorders, but for want of space the curious reader is referred to the work just quoted and to the thesis of C. Chauvet66 for an epitome of the most important recorded opinions.
66 Influence de la Syph. sur les Malad. du Syst. nerveux, Paris, 1880.
Those who suffer from syphilis are exposed in much greater proportion than are other persons to the ill effects of intemperance, sexual excesses, poverty, mental agony, and other well-established causes of general paralysis. It may be that in this is sufficient explanation of the frequency of general paralysis in syphilitics, but I incline to the belief that syphilis has some direct effect in producing the disease. However this may be, I think we must recognize as established the opinion of Voisin,67 that there is a syphilitic periencephalitis which presents symptoms closely resembling those of general paralysis. Such cases are examples of the pseudo-paralysie générale of Fournier.68
67 Paralysie générale des Alienés, 1879.
68 La Syphilis du Cerveau, Paris, 1879.
The question as to the diagnosis of these cases from the true incurable paresis is of course very important, and has been considered at great length by Voisin,69 Fournier,70 and Mickle.71
69 Loc. cit.
70 Loc. cit.
71 Brit. and For. Med.-Chir. Review, 1877.
The points which have been relied upon as diagnostic of syphilitic pseudo-general paralysis are—
The occurrence of headache, worse at night and present amongst the prodromes; an early persistent insomnia or somnolence; early epileptiform attacks; the exaltation being less marked, less persistent, and perhaps less associated with general maniacal restlessness and excitement; the articulation being paralytic rather than paretic; the absence of tremulousness, especially of the upper lip (Fournier); the effect of antispecific remedies.
When the conditions in any case correspond with the characters just paragraphed, or when any of the distinguishing characteristics of brain syphilis, as previously given, are present, the probability is that the disorder is specific and remediable. But the absence of these marks of specific disease is not proof that the patient is not suffering from syphilis. Headache may be absent in cerebral syphilis, as also may insomnia and somnolence. Epileptiform attacks are not always present in the pseudo-paralysis, and may be present in the genuine affection; a review of the cases previously tabulated shows that in several of them the megalomania was most pronounced; and a case with very pronounced delirium of grandeur, in which the autopsy revealed unquestionably specific brain lesions, may be found in Chauvet's Thesis, p. 31.
I have myself seen symptoms of general paralysis occurring in persons with a specific history in which of these so-called diagnostic differences the therapeutic test was the only one that revealed the true nature of the disorder. In these cases a primary, immediate diagnosis was simply impossible.
Case 14 of the table is exceedingly interesting, because it seems to represent as successively occurring in one individual both pseudo and true general paralysis. The symptoms of general paralysis in a syphilitic subject disappeared under the use of mercury, to return some months afterward with increased violence and with a new obstinacy that resisted with complete success antisyphilitic treatment. Such a case is some evidence that syphilis has the power to produce true general paralysis.
In conclusion, I may state that it must be considered as at present proven that syphilis may produce a disorder whose symptoms and lesions do not differ from those of general paralysis; that true general paralysis is very frequent in the syphilitic; that the only constant difference between the two diseases is as to curability; that the curable sclerosis may change into or be followed by the incurable form of the disease. Whether under these circumstances it is philosophic to consider the so-called pseudo-general paralysis and general paralysis as essentially distinct affections, each physician can well judge for himself.
Spinal Syphilis.
The subject of spinal syphilis is at present a difficult and unsatisfactory one. The recorded cases with well-observed autopsies are comparatively few, and when recovery occurs much uncertainty must rest upon the nature of the lesion. More than this, there is scarcely any chronic degeneration of the spinal cord which has not been attributed to syphilis, and my own experience as well as the records of medical literature lead to the very positive conclusion that all the various spinal scleroses are much more frequent in infected than in non-infected persons. Whether this is due to a direct or indirect influence of the disease is uncertain, but I shall not here discuss the relation of these chronic inflammations of the cord to syphilis.
It seems necessary to briefly consider at this place acute and subacute myelitis in their relations to syphilis. That these affections are not rare in syphilitics is certain. In the Revue de Médecine (Jan., 1884) Dejerine records the case of a person suffering from chronic syphilis in whom there were fulgurant pains with increasing weakness of the legs, and subsequently, after very severe exposure to the weather, a sudden development of complete paraplegia followed by trophic troubles, and death in twenty-eight days. At the autopsy there was found a central myelitis with pronounced lesion of the ganglionic cells, inflammatory changes of the pia mater, capillaries, and neuroglia, extreme alteration of the nerve-roots, and secondary degeneration of the columns of Goll and the lateral columns. In a second case recorded by Dejerine there appears to have been no exposure or apparent immediate exciting cause. The symptoms and lesions were similar to those just spoken of, but death occurred in eight days.
Whether such attacks as these occurring in syphilitic subjects are produced directly by the syphilis or not is at present doubtful. The same is true of subacute myelitis, of which I have reported two rather peculiar fatal cases in syphilitic subjects. The general symptoms of this affection are progressive loss of power with grossly exaggerated reflexes, severe twitchings and jerkings of the legs, rigidity, usually more or less marked pain, and other sensory disturbances in the legs, and finally partial anæsthesia and complete paraplegia, paralysis of bladder, bed-sores, and death from exhaustion. At the autopsy the most important change in the cord has been the presence of great numbers of round neuroglia-cells in both gray and white matter. One of my cases died of a rapidly developed central myelitis supervening upon the subacute disease, and affording lesions similar to those described by Dejerine in addition to the changes of the subacute affection.
In another class of spinal cases occurring in syphilitics the symptoms resemble those of the so-called acute ascending paralysis (Landry's paralysis). The fourth variety of syphilitic diseases of the spinal cord of Huebner72 includes these cases. According to Huebner, they are without anatomical lesions, but in the majority of the recorded cases no proper microscopic study of the cord has been made. Huebner states, however, that Kussmaul failed in one case after such study to detect lesion. As some of these cases may really have been instances of peripheral neuritis, it is essential that in the future the peripheral nerves as well as the spinal cord be carefully studied. I have seen one case which might be placed in this category. The first symptom was some numbness in the legs, with a small deep sharp-cut ulcer on the plantar surface of the great toe; directly after this loss of motion and sensation in the legs and thighs, rapidly becoming almost complete and spreading quickly to the trunk and arms, so that in one week the patient was a flaccid, helpless mass, and the breathing so interfered with that he was believed to be dying. After almost losing the power of swallowing this patient began to get better, and finally so regained power of his hands and feet that he was able to partially dress himself and walk a distance of ten or twelve feet, when he was suddenly seized with a pleural effusion and died. During the first week of his disease his temperature was 100° F. At the autopsy the spinal membranes were found to be normal. But in the cord there were very distinct lesions found; the neuroglia seemed everywhere more granular than normal; the ganglionic cells were not distinctly diseased; the white matter in various places was much changed, the tissue appearing abnormally dense and opaque where most affected; the nerve-tubules appeared to gradually lose their myeline, and in places were reduced to simple axis-cylinders. Finally, the axis-cylinders became smaller and smaller until in the most altered portions of the cord they disappeared. As the autopsy was obtained with great difficulty, it was not possible to get the peripheral nerves for study.
72 Ziemssen's Encyclopædia, vol. xii.
In regard to these very acute cases, it seems to me uncertain whether the disease should be attributed to the syphilis. In my own case twenty years had elapsed since the chancre, alcohol was habitually used in great excess, and the attack was apparently precipitated by great exposure. On the other hand, the man bore well enormous doses of iodide of potassium, and lowly progressed under them.
Finally, there is a class of disease of the spinal cord in which the lesion is undoubtedly the direct outcome of a syphilitic diathesis. In these cases the exudation commences primarily in the membranes of the cord, and may extend into the cord itself. In this class I would include the first two varieties of syphilitic spinal disease of Huebner. The number of recorded autopsies is not great; the only cases with which I am acquainted are those referred to in the note at the bottom of this page.73
73 Winge (Dublin Med. Press, 2d Series, vol. ix., 1863); Moxon (Dublin Quarterly Journ., li., 1870); Charcot and Gombault (Archiv. d. Physiologie, tome v., 143, 1873); Schultze (Archiv. Psychiat., xii. 567); Thos. Buzzard (Diseases of Nervous System, 1882, p. 407); Julliard (Étude Crit. sur les Localis. Spinal de la Syphilis, 1879); Westphal (Arch. Psychi., vol. xi.); Greif (Arch. Psychiat., xii. 579); Homolle (Progrès méd., 1876).
The lesions in these cases are entirely similar to those of brain syphilis. The disease very rarely or never begins in the interior of the cord. I know of no recorded case: Wagner's case, in which a yellow nodule was found within the cervical marrow, was probably not one of syphilis. If a gummatous inflammation does occur inside of the cord, it probably starts from the ependyma. The gummatous exudate may occur in the form of small multiple formations or of an extensive meningitis, with an infiltration of the membranes and their spaces with gummatous material. The membranes are usually agglutinated with one another and with the surface and with the cord. The exudation is usually made up of roundish cells, and in several cases spindle-shaped cells have been noticed, as have also the peculiar Deitres corpuscles already described as they occur in brain syphilis. The changes in the cord itself vary somewhat. In Winge's case the white matter seems to have undergone a rapid myelitis from pressure. It was of a grayish color, with numerous fine granular masses, corpora amylacea, pigment-masses, and fatty globules, the nerve-fibres being broken up. In other cases the change has been a sclerosis. The vessels of the cord have been noticed by various observers in the different stages of the degeneration seen in syphilis of the brain. They are often greatly dilated, their walls thickened, and, together with the lymph-spaces, infiltrated with small cells. Minute hemorrhages have been found.
The so-called syphilitic callus, as described by Heubner, is probably the remnant of a true gummatous inflammation. It consists of a circumscribed induration one to several lines in thickness, originating apparently from the dura mater, and causing sometimes adherence with the vertebræ, in others with the membranes of the spinal cord. In a case described by Virchow of this character the lesion was cervical, and the symptoms were stiffness in the nape of the neck, pains in the neck and arms, and finally paralysis in both arms. A second case is elaborately described by Heubner in his article in Ziemssen's Encyclopædia.
SYMPTOMS.—As the lesion of gummatous spinal syphilis affects primarily membranes of the cord, in the beginning of the attack the symptoms chiefly arise from the implication of the nerve-roots. Of course these symptoms vary with the seat of the lesion, for it must be remembered that the meningeal irritation is at first usually localized in a small region. As in a majority of cases this lesion affects a posterior portion of the cord, and as the posterior nerve-roots seem especially sensitive to irritations of this character, pain is usually a very marked and precocious symptom of spinal syphilis. The seat of the pain varies with the seat of the lesion. At first the pain is slight, but in most cases it soon becomes severe. It is sometimes situated at a fixed spot on the spinal column, where, according to Heubner, it may be increased on pressure. I have seen two or three such cases, but have and still do believe that under these circumstances the patient was suffering not simply from a spinal syphilis, but also from an implication of the vertebral periosteum or of the vertebræ themselves. In one of my cases this diagnosis was confirmed at the autopsy. When the lesion is purely meningeal there is probably no marked local tenderness. The severe pains usually felt in the extremities or in the trunk are often fulgurant; sometimes they are described as resembling the thrust of a knife, and not rarely they closely resemble the pains of locomotor ataxia. In some instances the pains are comparatively slight and are aching in character. Paræsthesiæ are not rare phenomena: such are formications, tingling in the extremities, numbness and feeling as though the limb were asleep, intense sense of coldness on the surface, sensation of water running over the limb. Early in the disorder there is sometimes very marked hyperæsthesia, but later, even though the pain persists, blunting of sensibility is marked, and there may be a complete anæsthesia. This anæsthesia is sometimes localized in certain parts of the limb. Thus, in a case reported by Alfred Mathieu,74 although there was complete anæsthesia of the outer side of the left leg and foot, the inner side retained its normal sensibility. In some cases there is the abdominal cincture of ordinary myelitis. The records show that even in these early stages there may be diplopia, amblyopia, or other disorder of vision, and the pupil may be distinctly affected. In these cases it is probably the upper portion of the cord which is affected.
74 Ann. de Dermatol. et Syph., vol. iii., 1882.
Disturbances of motility in the majority of cases do not develop until some time after sensation has been affected, but may come on very early. Usually, the first symptoms are those of irritation, such as rigidity of the neck, back, and limbs or even of isolated groups of muscles. Tremors have been described as frequently present. These may be convulsive, and are often plainly reflex in their origin; indeed, I am inclined to believe that they are always reflex tremblings, and never true tremors. Heubner describes a case in which a paralyzed limb was thrown into violent tremblings whenever passive motion was attempted. The patella-reflex is usually grossly exaggerated, although it may be lost in the later stages of the disorder. Not rarely there is the condition which has received the misnomer of spinal epilepsy. This exaggeration of the reflexes may be limited to one leg, when it is almost pathognomonic. In some cases severe cramps are excited by movement. Usually there is no tenderness. These symptoms of the meningitic stage may continue for weeks or months without there being pronounced paralysis, although locomotion is not rarely interfered with by the stiffness of the legs. Finally, if the case progresses the patient notices a weakness in one or both legs, or (if the disease be situated high up in the spinal cord) in one arm, which rapidly increases until there is almost complete loss of power. This rapid increase of palsy following long-continued disturbance of sensation is almost pathognomonic. In most cases one side of the body is more affected than the other. The sphincters are prone to be implicated, and in advanced stages of the disease there is usually complete loss of control over the bladder and rectum. The patient may live for months without very distinct change of this condition, or bed-sores and other trophic disturbances may rapidly develop and death ensue in a short time. I have seen under these circumstances marked elevation of temperature, rapid feeble pulse, mental weakness, and the general symptoms of septicæmia last for many weeks. Ammoniacal cystitis is of course prone to be developed during this stage. When motility fails, sensibility is usually blunted, although the pains may even increase. Heubner affirms that an incompleteness of the anæsthesia is characteristic of the disorder.
The typical course of spinal syphilis, such as has been described, may be variously departed from. Sometimes the power of co-ordination is early affected, and the symptoms may resemble those of locomotor ataxia. I doubt, however, whether under any circumstances there is a loss of the patella-reflex in the early stages of the gummatous disease of the spinal cord. In other cases the paralytic symptoms may be very prominent from the beginning: thus, in the case of R. P——, aged 27, which I believed to be gummatous disease of the spinal cord, the first disorder was a feeling of malaise lasting for about a week, followed by the sudden, rapidly-developed paralysis of the bladder, loss of power in the legs, and to a less extent in the arms, the only pain being a dull, steady ache in the arms. The bowels were obstinately costive. Double vision was soon very pronounced. When I first saw the patient, about three weeks after this, there was decided impairment of sensibility in the legs, but not in the arms; marked muscular weakness of both legs and arms; no loss of co-ordinating power; dropping of the right eyelid, with double vision; and only some slight aching pains in the arms. By the use of large doses of iodide of potassium and other appropriate measures a good recovery was secured.
A case illustrating the occasional difficulty of diagnosing spinal syphilis is reported by C. Eisenlohr.75 The first symptom was obstinate constipation, with very great discomfort after defecation; then appeared incontinence of urine with weakness of the legs: finally, a sudden complete palsy of the right leg occurred, with marked anæsthesia in both legs, partial loss of power in left leg, violent boring abdominal pains, and distress in the bladder. In the last stages there were severe neuralgic pains in both legs, with complete loss of sensation, bed-sores, atrophy of the leg-muscles, with reactions of degeneration, and death from exhaustion. At the autopsy an advanced meningitis was found which had apparently commenced in the regions of the cauda equina, and given rise to complete degeneration of the nerves. The only alteration of the cord was an ascending degeneration of the posterior columns.
75 Neurolog. Centralb., 1884, p. 73.
Again, owing to the diseased condition of the vessels, a spinal syphilis may be suddenly interrupted by an apoplectic accident.
In a patient of my own, who was believed to be suffering from gummatous spinal meningitis, there was an abrupt development of violent tearing pains, loss of power and sensibility, and all the other symptoms which are characteristic of meningeal spinal hemorrhage. A. Weber reports a case in which, after doubtful premonitory symptoms, such as vertigo, loss of power on the right side, pressure on the top of the head, and tinnitus aurium, there was a sudden development of convulsions, and death. At the autopsy a syphilome of the right vertebral artery was found with a recent thrombosis of the basilar artery.76
76 American Journ. of Neur. and Psychiat., vol. ii.