TUMORS OF THE SPINAL CORD AND ITS ENVELOPES.
BY CHARLES K. MILLS, A.M., M.D., AND JAMES HENDRIE LLOYD A.M., M.D.
DEFINITION.—Under Spinal Tumors will be included the growths or adventitious products which arise in the substance of the spinal cord or spring from its envelopes, membranous or bony, in such manner as to directly or by pressure involve the spinal cord. Tumors strictly confined to the cord are extremely rare. First in order of frequency are the new growths which develop from the spinal membranes, either the dura mater or pia mater, most frequently the former. Tumors originating in the bony spine, like those of the substance of the cord, are comparatively rare.1
1 A “Table of Fifty Cases of Spinal Tumor” (which will be frequently referred to) is appended to this article.
ETIOLOGY.—Under the predisposing causes of spinal as of intracranial growths are such diatheses or constitutional affections as cancer, tuberculosis, and syphilis. Under Pathology a table will be given from which it appears that of 50 tabulated cases, 3 were cancerous, 5 syphilitic, and 4 tubercular.
Traumatisms, such as a fall from a height, a blow on the back, a wrench or twist of the spine, or a sudden concussion as in a railway accident, sometimes serve as exciting causes of spinal tumors. Even when a diathetic or infectious predisposition exists, the patient might frequently escape from the special intraspinal localization of the disease were it not for the accidental infliction of direct injury to the axis. When no special predisposition is present, an injury is more likely to produce an osteoma, fibroma, or sarcoma than some of the other forms which will be mentioned, such as a glioma, myxoma, neuroma, or psammoma.
Spinal tumors are said by most authors to occur much more frequently in the male than in the female sex. Our tabulated cases, however, gave 22 cases among males, 21 among females, and 7 in which the sex was not given.
Fifty cases of spinal tumor gave the following result as to age:
| Under 10 years | 4 | cases. |
| From 10 to 20 years | 3 | cases. |
| From 20 to 30 years | 7 | cases. |
| From 30 to 40 years | 12 | cases. |
| From 40 to 50 years | 10 | cases. |
| From 50 to 60 years | 6 | cases. |
| From 60 to 70 years | 1 | case. |
| Age not given | 7 | cases. |
| 50 | cases. |
SYMPTOMATOLOGY.—Can tumors of the spinal membrane be separated by a study of symptoms from intramedullary tumors? While this may be theoretically possible, in practice it will be found difficult, and of little value even when it can be done. The spinal canal is of such narrow calibre that a growth of any size either in the membranes or the cord itself will soon directly or indirectly involve both. On this subject Erb2 speaks as follows: “The attempt has thus far been made in vain to secure, from amongst the individual symptoms, at least a few fixed points on which to base the diagnosis of intramedullary tumors. The following have been claimed as such: A somewhat long antecedent history of active local manifestations of irritation, belt-like pains, eccentric pains, definite paræsthesiæ, local paralysis, as in meningeal tumors; early and well-marked atrophy, which points to a larger involvement of the gray substance; striking fluctuations in the course of the disease, spontaneous improvement, and equally spontaneous growing worse again (Schueppel); and, finally, Schueppel has also tried to connect the occurrence of scoliosis (curvature of the spinal column to the side on which the tumor is situated) with the presence of a tumor. In this he is doubtless wrong, as this manifestation merely depends on unilateral paralysis of the muscles of the back, which may depend on all sorts of causes.”
2 Ziemssen's Cycl. Prac. Med., Am. trans., p. 754.
In the discussion of symptomatology and diagnosis which follows therefore no effort will be made to separate the phenomena of meningeal and intramedullary growth. In most cases the symptoms indicate involvement early of the membranes, and later compression of the cord.
The symptomatology will differ according to the stage of the affection. The symptoms can be arranged into those of a first or early, a middle, and a late stage. As a rule, but not invariably, the symptoms of the early stage are those of beginning irritation—such sensory phenomena, for instance, as pain in the parts supplied by certain nerves in the neck, arms, hands, abdomen, legs, or feet. This pain is sometimes associated at an early period with more or less stiffness, which later may become well-marked contracture. Pain in the back is occasionally an early symptom. Paræsthesiæ, as numbness, formication, constriction or girdle sensations, coldness or heat or alternations of hot and cold feelings, are sometimes early symptoms, but occur in a more positive manner in the middle stages of the progress of the spinal growth. Hyperæsthesia is more likely to be pronounced in the middle stage of the disease. A slight paresis, which may vary a little from day to day for a time, slight twitchings or spasms, usually localized to certain muscles or groups of muscles, are also present, in some cases as an incipient manifestation. The presence of these motor symptoms will depend largely upon the location of the incipient growth with reference to the columns of the cord.
One general point of differentiation between intramedullary and membranous tumors is the fact that irritative phenomena, such as pain in the back and along nerve-tracts, spasmodic twitchings, etc., are not likely to appear as early in the intramedullary cases as in the meningeal or mixed forms.
In the middle period of the progress of a spinal tumor the irritative phenomena, such as pain, paræsthesiæ, hyperæsthesia, twitchings, cramps, stiffness, and paresis, will be found to persist and increase, and in addition other manifestations will appear, chiefly those of compression of the cord. Anæsthesia frequently develops, and, when the posterior segment of the cord is the one chiefly implicated, soon becomes profound; it is, however, often variable in distribution for a time, and sometimes shows peculiar areas. One leg or one arm may be partially involved, or both lower or both upper extremities, or certain portions of any one of the limbs. Anæsthesia dolorosa—that is, absence of sensation to objective tests, although the patient suffers pain or distress in the affected part—is common. Hyperæsthesia, best observed in this middle period, is sometimes cutaneous, but in other cases follows certain nerve-trunks, probably indicating a neuritis descending from the seat of the spinal growth. In this and in the last stage the paresis advances to more or less complete paralysis, which is also variable in distribution according to the localization of the growth. Contractures and rigidity come on and changes in the reflexes now become important symptoms. These may be increase of knee-jerk with ankle-clonus, or diminished or abolished knee-jerk, according to the position and the extent of the lesion.
The late symptoms of spinal tumors, especially of those which are slowly developed, are—usually profound anæsthesia and paralysis of the limbs, with rigidity and contractures, atrophy, marked changes in the spinal reflexes, bed-sores, paralysis of bowels or bladders or their sphincters, impotence, œdema, dyspnœa, vomiting, cardiac palpitation, cystitis, and pyelitis, and, when the lesion is near the medulla oblongata, dysphagia and certain intracranial symptoms, as amblyopia, diplopia, deafness, contracted pupils, affections of speech, etc.
The size of the growth and the rapidity of its development will of course influence the character and the time of appearance of certain symptoms. According to the position of the growth, either as to its level in the spinal axis or as to its relative position to the various longitudinal segments of the spinal cord, the symptoms will also differ at special stages. These differences will appear as symptomatology and diagnosis are now further considered.
Pain in the back occurs, but is not as frequent a symptom as eccentric pain. Unlike headache in intracranial tumors, it is not a constant symptom. The headache of brain tumors is due in part at least to the conditions of tension which are produced by the growth interfering with the balance of pressure within the skull. Headache is also, as has been pointed out in the article on Brain Tumors, frequently due to the irritation of the membranes; but in this case the one great nerve through which pain expresses itself is the trigeminal, which has its distribution both within the skull and outside of it to all parts of the head. In spinal tumors the pains are more likely to be eccentric, because of the limited character of the lesion and the almost exclusively peripheral distribution of the nerves. Twisting the trunk or jarring the spinal column by blows on the head will sometimes cause pain along the spine, most frequently when the bone is involved. It sometimes cannot be elicited.
Leyden3 pointed out the fact that the movement of the spinal column is often difficult and painful in a certain direction, because this motion brings a greater pressure upon the tumor.
3 Quoted by Erb.
Pain on percussion over the spinal column might be expected from the character of the affection, but has not been frequently reported. Like spinal hypersensitiveness and inflexibility with muscular rigidity, it is much more to be expected in those cases in which the vertebral bones and cartilages are implicated.
Constriction or girdle sensations are of comparatively frequently occurrence. When the cervical cord is involved, choking sensations or a sense of constriction about the neck are common. Tumors located in the lower cervical and dorsal region give girdle sensations most frequently in the chest or abdomen. So far as the assistance afforded by such sensations toward localizing the exact level of the growth is concerned, however, it must not be forgotten that curious and unexpected conditions sometimes occur. Thus, in one case (Case 16) a myxoma at the level of the sixth and seventh cervical vertebræ caused constriction sense about the legs and abdomen, and in another (Case 44), a glioma of the filum terminale, constriction of the chest.
The paralytic phenomena of spinal tumors have certain peculiarities which are not exhibited by any other spinal or by cerebral affections. A glance at the clinical history of a number of cases shows that many of them began with paresis of a single limb or part of a limb, in addition to the irritative phenomena. This paresis deepens after a time into complete paralysis, or before this occurs one or more of the other extremities become paretic. The progress toward bilateral paralysis may be comparatively rapid. The appearance and progress of the paresis or paralysis vary somewhat according to the level of the cord at which the tumor is located. In tumors of the cervical cord the paresis usually, but by no means invariably, first attacks the upper extremity. The fact that the arms are first the seat of irritative phenomena and paresis is in a case of spinal tumor indicative of a cervical location or a location in the upper dorsal region; but, on the other hand, not a few cases are recorded in which in tumors in these locations the loss of power first exhibited itself in one or both of the lower extremities. These cases are to be explained by the manner in which the descending motor tracts are affected directly or by pressure. In mid-dorsal tumors and those below this level the paresis shows itself first in the legs, and generally becomes before long a complete paraplegia.
To Brown-Séquard, more than to any other observer, we owe our accurate practical knowledge of unilateral lesions of the spinal cord, both in the cervical and other regions. When the lesion is localized in one lateral half of the cord and is situated in the cervical region, we have the affection known as spinal hemiplegia. The main symptoms of this affection are motor paralysis of the arm and leg on the side of the lesion and anæsthesia of the opposite limbs. Sensory fibres decussate in the cord soon after entering it, while the motor tracts cross at the anterior pyramids of the medulla oblongata; in which physiological facts we have a simple explanation of the peculiar motor and sensory phenomena presented by such a case. When the lesion is below the cervical portion of the cord, instead of spinal hemiplegia we have the affection known as spinal hemiparaplegia, in which the paralysis and hyperæsthesia in one lower extremity stand out in strong contrast to the anæsthesia and retained muscular power in the other. With a lesion so strictly localized as a spinal tumor it might be expected that these crossed phenomena would present themselves in some cases. They are recorded, more or less distinctly, in Cases 4, 16, and 38, and it is probable that they would have been more frequently observed if they had been anticipated and looked for carefully.
Ataxia does not seem to have been a frequent symptom in reported cases. It has probably been sometimes overlooked or confounded with paresis. In a case of myxoma of the dura mater in the left dorsal region ataxia of both leg and arms was present; but in this case, however, the dura mater of the brain contained fluid and lymph. In the light of the commonly accepted views as to the physiology of the spinal cord regarding the posterior columns, as related in function both to co-ordination and sensation, ataxic manifestations might be frequently expected. Owing, however, to the narrowness of the spinal canal, compression of the entire cord takes place so early as to make paretic symptoms displace those of ataxia.
Atrophy which varies in distribution according to the extent of the destructive involvement of the cord is frequently present. In a few instances the atrophy will be of certain muscles or muscular groups. When true atrophy is present the anterior horn will be involved directly or indirectly, and accompanying changes in the electrical reactions will also be found.
The electrical condition of the atrophy of the paralyzed parts will vary with the extent of the trouble produced by the tumor. In a case of tumor of the cervical enlargement, for instance, producing more or less paralysis both of the upper and lower extremities, reactions of degeneration will be present only in the muscles supplied by the nerves which spring directly from the seat of lesion.
Spontaneous twitchings or spasms due to irritation of motor-centres or tracts are comparatively frequent. More or less permanent contracture in a limb or part is of frequent occurrence, particularly after the growth has advanced. Forms of torticollis or retraction of the head, strong flexures of the arms or legs, and, late in the history of many cases, complete and extremely painful drawing up of the limbs upon the body, may occur. Persistent subsultus was observed in one case, a glio-myxoma involving the gray columns from the medulla oblongata to the cauda equina. Fibrillary twitching is rare, and in our tabulated cases was observed only in one instance, a glioma of the filum terminale. General convulsions with unconsciousness are exceedingly uncommon. In one case, however, a round-celled sarcoma at the level of the seventh, eighth, ninth, and tenth cervical vertebræ, the patient is recorded as having died in a fit. Nystagmus was observed once, but probably had no significance so far as the spinal affection was concerned, as the cerebral dura mater exhibited evidences of inflammation.
The condition of the reflexes, both cutaneous and tendinous, is often peculiar and almost diagnostic, but varies greatly according to the position and extent of the lesion. Increase of reflex action is sometimes a marked symptom. The slightest irritation of the soles of the feet, the calves of the legs, palms of the hands, or other special regions will often produce decided contractions, and sometimes that symptom which has been designated by Brown-Séquard as spinal epilepsy, in which both lower extremities are thrown into violent clonic spasms, which may last for many seconds or even minutes. In tumors of the dorsal region causing marked compression and preventing cerebral inhibition, clonus and knee-jerk are also markedly exaggerated. The so-called diplegic contractions—that is, contractions in one extremity from irritation of the other—are observed, especially when a transverse area of the cord, large or small, is involved in an irritative or inflammatory process. They probably result from the abnormal facility of transmitting impressions which has been acquired by the cord. When the cervical or the lumbar enlargement of the cord is completely compressed or destroyed by a tumor, reflex activity is diminished or abolished in the region supplied by nerves originating at the seat of lesion.
Alterations in the body-temperatures have been frequently noted in lesions of the spinal cord, such as fractures of the vertebræ and inflammatory changes in the cord and its membranes. Some of the observations are almost incredible, as that of J. W. Teale,4 who records an axillary temperature of 122° F. The subject has been somewhat obscured by physiological speculations. Thus, it has been asserted that paralysis of motor centres and strands causes an increase of temperature, while paralysis of sensory tracts produces a diminution. The accepted clinical facts apparently are as follows: after crushing the cervical cord a uniform rise in temperature occurs if peripheral cooling is prevented. This rise is caused by a paralysis of the vaso-motor nerves, which permits a paralysis of the vessels and floods the parts with blood. This assumes, of course, that mere increase of blood in a part means increase of heat. After a variable period this increase is followed by a decrease which is permanent. Hutchinson records5 a case of fracture of the cervical spine at the fifth vertebra in which the patient's body felt almost as cold as a corpse and the rectal temperature was only 95° F. In Cases 4 and 16 the affected parts are recorded as cold, just as in atrophic and other lesions of the cord. The tabulated cases do not show many exact thermometric observations, but in Case 8 the average temperature for two weeks before death is given as slightly below normal, while Case 7 shows a sudden rise on the day preceding death. It is probable that the permanent decrease following crushing and compressing lesions would also be found in cases of spinal tumors. The following exact observations, made by one of us6 upon a case of injury to the cervical cord, are directly illustrative of this subject. The case was of several months' standing. The patient could walk imperfectly, and the right arm was more paretic than the left. The observations were made at 10 o'clock A.M. on six successive days:
| Right Axilla. | Left Axilla. | |
| First observation | 98.2° | 97.3° |
| Second observation | 99.8° | 98.2° |
| Third observation | 96.4° | 96.2° |
| Fourth observation | 95.6° | 96.4° |
| Fifth observation | 97.2° | 96.8° |
| Sixth observation | 95.2° | 93.8° |
It will be seen that the temperatures range higher in this series on the more paretic side; but this did not hold in a series taken a few weeks later. The fact is to be noted that all these temperatures, with one exception, are below the normal.
4 Lancet, March 6, 1875.
5 Ibid., August, 1875.
6 Hospital Gazette, Nov. 7, 1879.
Cystitis, pyelitis, and pyelo-nephritis are usually secondary symptoms, due to retention of urine, distension of the bladder, etc. in consequence of paralysis of this viscus. Bed-sores result in two ways: in the first place, they may be due to emaciation and immobility and the uncleanliness which it is almost impossible to prevent; or, in the second, trophic eschars may arise because of the involvement of nutritive regions of the cord. The so-called sacro-ischiatic eschars are of this character. Febrile phenomena, such as chills, increased temperature, increased respiratory action, are frequently secondary phenomena due to bed-sores, cystitis, continued pain, exhaustion, or septic infection.
Priapism was recorded in two of the fifty cases, both tumors of the cervical cord. Impotence was only recorded once, a dorsal myxoma. It is probable that both priapism and impotence, particularly the latter, were present, but overlooked in other reports.
Headache was present in three of fifty cases. One of these was a fibro-sarcoma at the level of the fourth cervical nerve; the other two were low down in the spinal axis, one in the lumbar enlargement, and the third, strange to say, in the filum terminale. Vertigo is a rare symptom in the spinal tumors. In one case in which the growth was located at the position of the third cervical vertebra its presence was recorded.
Out of five cases in which vomiting was present, two were in the cervical regions, one in the dorsal, one in the lumbar, one in the filum terminale.
Tumors involving the upper cervical and bulbar region of course will give rise to special symptoms indicating more or less involvement of cranial nerves.
Unfortunately, very few ophthalmoscopic observations have been made in cases of spinal tumor. Optic neuritis has been found in a few cervico-dorsal cases, and in other cases located in the same region no ophthalmoscopic alterations have been discoverable.
Mental disturbances were only especially recorded in four of the fifty cases. Such symptoms were certainly not of frequent occurrence, except those emotional manifestations which were due to the great suffering which the unfortunate patient was called upon to endure. These emotional disturbances, as in all forms of painful disease, varied according to the mental stamina of the patient. In one case the mental symptoms, in association with other phenomena and a probability of dog-bite, led to the suspicion of hydrophobia.
In one case (31) an interesting observation was made of an anal sphincter reflex, with frequent stools. The tumor in this case is reported at the level of the tenth dorsal vertebra, which would be at the level of the eleventh dorsal segment. The tumor was tubercular, and therefore probably meningeal, so that the irritation to the anal centre, which is in the lumbo-sacral segment, may have been caused by extension of inflammation along the meninges.
PATHOLOGY.—We present in tabulated form the various kinds of tumors as found in the fifty cases which have been collected:
| Aneurism | 1 | Myxoma | 2 |
| Cancer (?) | 2 | Neuroma | 1 |
| Carcinoma | 1 | Organized blood-clot | 1 |
| Cysticercus and hydatids | 3 | Osteoma | 1 |
| Cyst (Dermoid?) | 1 | Phlegmon | 1 |
| Fibroma | 5 | Psammoma | 2 |
| Glioma | 5 | Sarcoma | 7 |
| Gumma | 5 | Tubercle | 4 |
| Myo-lipoma | 2 | Unclassified | 6 |
It will be observed that the predominance in this list is decidedly in favor of the sarcomata and structures which are likely to be associated with or to graduate into them, such as the gliomata, myxomata, and the psammoma. In one instance the resemblance (Case 15) to psammoma is referred to by the reporter. The comparatively large number of unclassified, and the two cases referred to vaguely as cancer, would probably, on more exact report, have added several more to the group of the sarcomata. The table shows that next in frequency come the fibromata and gummata, while the carcinomata have but a single representative in the group. Tubercular tumors occurred with comparative frequency, no less than 8 per cent. being recorded. Although the exact origin of only somewhat more than one-half (29) of all the tumors is given, it is stated of this fraction that 17 sprang from the membranes, while of the remainder 8 were located in the cord itself and 4 in the vertebræ. Of the 3 oases of parasitic invasion, it is recorded of one (Case 43) that a hydatid cyst was also found in the liver; and it is probable that in any given case the spinal cord would not be the only part to suffer. Cobbold's work refers to one case of hydatid of the spinal cord. Erb refers to 13 cases, all but 2 external to the dura mater. In Case 43 of the table pains in the back and hip, simulating rheumatism, were present early.
The dimensions of the spinal tumors of whatever character are never very great, for the reason that they have but little space in which to enlarge, and that their presence soon causes such grave changes as to be incompatible with life. They rarely exceed an inch in their longest diameter, and not unfrequently are smaller than this. There is usually about them a more or less marked meningitis and an area of vascular fulness. Œdema of the membranes is sometimes noted. The substance of the cord beneath is compressed, atrophied, or softened, and this softening sometimes extends for a considerable distance both above and below the neoplasm. These changes were reported in Case 8, in which there was the addition of an abscess. Secondary degenerations would probably be found in all cases, unless very recent; and these changes, following the Wallerian law, would ascend the posterior and descend the lateral columns. Such degenerations are reported in some of the cases. The spinal nerves are sometimes compressed and atrophied. Old or recent hemorrhages are found, as in Case 18. Among the changes which occur, probably at a late stage, are the formation of cysts, either large or small, either in the substance of the cord or consisting simply of a dilatation of the central canal of the cord. This condition is known as syringo-myelia, and is of exclusive pathological interest. In Case 1 is recorded, apparently, a well-marked dilatation of the central canal (hydromyelus), and cyst-formation is recorded with gliomata, sarcomata, and gummata in other cases. Caries of the vertebræ is recorded in a number of cases, and occurred both in cases of gumma (Case 8) and carcinoma (Case 14). These cases were, however, exceptions to the general rule that the bony envelope of the cord does not furnish external evidence of the location of the tumor. It is worthy of note that the one instance of phlegmon or inflammatory exudate (Case 20) also presented infiltration of the tissues of the throat and mediastinal space. A case of organized blood-clot (Case 39) has been included in the list, although, properly, a spinal hemorrhage, because it became and acted as a tumor. The location of the single case of aneurism (Case 48) is not given. The symptoms were those of tumor in the dorsal spine.
As sequelæ of tumors of the cord may be mentioned especially bed-sores, which sometimes commit frightful ravages, as in a case (37) in which the spinal canal was laid open. The bronzing of the skin and diseased condition of the suprarenal capsules, as recorded in Case 41, were mere coincidences, and not probably at all connected in pathological sequence with the spinal lesion. Cystitis, pyelitis, and pyonephritis are not uncommon in cases of tumor of the cord, just as they are observed in other compressing and destructive lesions of that organ. In those cases in which the tumor is the result of a general taint, as in gummy and tubercular growths, the evidence of this taint is not usually wanting in other organs; thus in Case 37, of tubercle of the cord, tubercles were also found in the lungs, bowels, and uterus. In gummata of the cord it would not be likely to escape careful inquiry that the patient's history or his body presented evidence of the disease.
In a case (47) of congenital sacral neuroma amyilinicum the infant was also hydrocephalic and had a bifid spine—conditions of faulty development with which the patient cannot long survive.
In one case of psammoma (Case 5) a resemblance to endothelioma is noted, while in another (Case 15), already referred to, a sarcoma is said to have resembled a psammoma.
Vascular changes are usually notable. In addition to the congestion already spoken of, it is recorded in one case (No. 14) that the right vertebral artery was obliterated. This was a carcinoma which had partly destroyed one vertebra.
Virchow's case (No. 50) of a stillborn child with a large tumor of the size of the head of a child of two years, and containing bone, has some analogies in three cases, referred to by that author, in which both hair and bone were found.
In several cases a brown or yellow exudate (plastic lymph?) is mentioned as extending along the cord far beyond the immediate neighborhood of the tumor.
The histology of tumors of the whole cerebro-spinal axis will be found described in the article on Tumors of the Brain.
DIAGNOSIS.—The diagnosis of tumors of the spinal cord presents itself naturally under two heads—the differential, or general, and the local diagnosis. The conclusions reached in this paper are based on a careful study of the cases appended, two of which were personal observations, and the remainder were collected from American, English, French, and German literature. It cannot be denied that much obscurity rests upon the diagnosis of tumors of the spinal cord, and that the doubts expressed by Erb and other writers have much to support them. It is hoped that the systematized study presented in this table will do something to dispel this obscurity.
(1) General Diagnosis.—The differential diagnosis has regard first to certain general phenomena which are broadly indicative of a spinal disorder as distinct from a cerebral or peripheral one. Thus, mental symptoms are absent, or if present are an accompaniment of tumors high up in the spinal axis, are the results of suffering, or appear very late in the disease because of progressive weakness. Briefly stated, the phenomena which point with comparative certainty to the existence of spinal tumors are symptoms of meningeal irritation gradually increasing, and symptoms of slow compression of the cord. These have been sketched at the beginning of Symptomatology. As to duration, the data in the cases studied were somewhat meagre. The usual duration is from six months to three years.
The differential diagnosis of spinal tumors will be considered in reference to the following affections: congestion, hemorrhage, meningitis (simple and specific), caries, traumatisms, sclerosis, aneurisms, neuritis, metallic and infectious disorders, and hysteria. Spinal tumors, it will be recalled, are from constitutional or special causes, as syphilis, cancer, and tuberculosis. The onset is gradual and irregular. The duration is comparatively long. The progress is by irregular advances toward a fatal termination. The symptoms are inclined to be at first unilateral or local; later, bilateral. Special symptoms, as paralysis, spasm, sensory and visceral disorders, occur irregularly as to time. Decubitus and trophic changes are common late in the history. Reactions of degeneration are often present. Gowers refers to the fact that two morbid processes often occur, one consecutive upon the other, as a secondary degeneration or a hemorrhage, after the establishment of the morbid growth, with characteristic increase of symptoms.
In spinal congestion a constitutional cause is not likely to be present. The onset is usually sudden and after exposure. The duration is shorter than in tumors, and is from a few days to four months. The disease is stationary for a while; then retrogression of symptoms toward recovery occurs. The symptoms are more uniformly bilateral, and motor and other symptoms develop about the same time. Decubitus is rare. Reactions of degeneration are rare (?). It is desirable that cases of so-called spinal congestion should be differentiated from the forms of peripheral neuritis above referred to, the most characteristic symptom of which appears to be tenderness of nerve-trunks.
In spinal hemorrhage there is no special history, or a history and signs of cardiac and vascular degeneration may be present. The onset is quite sudden and the progress of the case regular. The first symptoms persist, and secondary degenerations follow, and differ according to the extent and location of the lesion, but are most likely to be uniformly bilateral.
In meningitis the symptoms of localized compression are absent. The girdle symptom is absent. The affection is sometimes curable, and especially so if it has been of syphilitic origin. The reactions of degeneration are not marked.
In caries of the spinal vertebræ deformity is rarely absent, especially if the case has continued a few months. Rigidity of the muscles of the back is an important symptom, which, however, is occasionally found with tumor. Jarring of the spinal column by tapping upon the head or jumping from a chair or stool is more likely to elicit pain in caries than in tumors. Strumous symptoms and evidence of tubercles in the lungs or other organs are often present.
In traumatisms usually a history of the injury can be obtained. The symptoms are those of caries, myelitis, meningitis, or of combinations of these, according to the character of the case.
In sclerosis the symptoms are usually those of progressive systemic affections, with absence of compression symptoms. The duration is longer. The progress is gradual and more regular.
Aneurisms are only to be distinguished when extra-spinal, causing erosion and compression.
In neuritis there is the soreness of the nerve-trunk already referred to, while compression symptoms and visceral disorders are absent. The motor and sensory symptoms are confined to the area of distribution of the affected nerve. It is amenable to treatment. In advanced stages the reactions of degeneration are marked. In the form of general peripheral neuritis, the existence of which, as a distinct disease, is being at present claimed, the characteristic symptoms are as yet not sufficiently determined or the pathology demonstrated by post-mortem research to admit of much discussion.
In metallic and infectious disorders a history of definite causation is present. Metallic disorders may present special distinctive signs, such as lead-line, wrist-drop, etc.
In hysteria a precedent characteristic history is usual. The onset is often sudden and an emotional element is present. The symptoms are bilateral and protean. Trophic changes are absent. No reactions of degeneration are present.
(2) Local Diagnosis.—It may be said of spinal tumors in a modified sense, as it can be said of brain tumors, that they are not good pathological experiments for illustrating the functions of the exact areas which they occupy. The spinal canal has such narrow limits, the tumor itself soon attains such a relatively large size and causes such wide vascular engorgement, and the different tracts and systems of the cord are so closely packed together, that the tumor does not often invade only one functional area and escape another. Hence the regional diagnosis presents special and greater difficulties than the diagnosis of the level of the cord at which the tumor presents itself. A tumor which destroys the trophic centres for the arm in the anterior cornua might exert sufficient backward pressure to paralyze the motor tracts running to the leg; or a cervical tumor, as in Case 5, might produce symptoms which are almost wholly observed in the legs. It will be seen, however, by reference to the table, that in Case 3, reported by Wilks, we have a tumor whose exact anatomical seat could have been predicted, and which seems to have reproduced almost the upper-arm paralysis of Remak. When we compare these two cases, in which the pathological conditions are so similar, it will be observed that the paralyzed arm is much wasted, which indicates a lesion of its trophic centre, whereas the affected legs in the other case are irritated by pressure and by isolation, but are not wasted, because their trophic centres are far below the point of lesion. The invasion of the trophic centres, and the accompanying wasting of particular groups of muscles, especially when this occurs early in the case, with the consequent reactions of degeneration in these muscles, would furnish very valuable indications both as to the region and the level of the cord involved (Case 29). Unfortunately, the exact observations are wanting in most of the cases as reported.
M. Allen Starr, in a recent paper,7 has devoted much labor to the elucidation of the functions of different segments and regions of the cord. He demonstrates the existence of groups of cells in the gray matter, especially in the anterior horns, each of which he believes constitutes a physiological unit. He affirms that these cell-groups preside over certain associated movements or combinations of certain muscles, and, quoting from Spitzka, says that “the nearer a muscle is to the ventral aspect of an animal the nearer will its nucleus be to the median line of the cord; and the nearer the muscle is to the dorsal aspect of the animal the nearer will its nucleus be to the lateral cornua of the cord. Flexor nuclei are therefore in internal, extensor nuclei in external and posterior, cell-groups.” The only light that such a theory throws upon the subject of diagnosis is by affording a possible explanation of the fact that spastic flexion is much more common than spastic extension, and may be due to the fact that the cell-groups for flexion lie deeper and are more protected than those for extension; and the additional fact above referred to, that a paralysis of associated muscles or groups of muscles, with degeneration, as in the types of Remak, would indicate with great clearness the destruction of the cell-group which presides over them. Starr, in his article, also tabulates the various reflexes and their seats in the cord. As this subject is of much importance in any exact study of spinal-cord diseases, we will state here some of the facts as given in that article: The neck-pupil reflex (dilatation of the pupil on irritation of the neck) has its seat from the fourth to the seventh cervical segment; the elbow-tendon reflex in the fifth and sixth cervical; the wrist tendons from the sixth to the eighth cervical; the palmar in the seventh and eighth cervical; the epigastric and abdominal skin reflexes in the fourth to the eleventh dorsal segments; the cremasteric reflex in the first to the third lumbar; the patellar tendon in the second to the fourth lumbar, and bladder and sexual centres in same; the rectal centre in the fourth lumbar to the third sacral; the foot-clonus and Achilles-tendon reflex in the first sacral. A destructive lesion, such as a tumor, at any one of these points would cause abolition of that particular reflex, and this would probably occur early in the case. Our table of cases does not present any such observation, whereas exaggerated reflexes, such as occur from a compressing lesion above the seat of the excited centre, are recorded in abundance. Many of these deductions are of course only possible early in the history of the case, as at a late stage the secondary degenerations have caused too widespread havoc to admit of any exact localization. The distinction must also be sought for between a destructive lesion and the symptoms of irritation which it may project to distant parts. Fürstner's cases of syringo-myelia,8 in which were marked vaso-motor changes, such as pallor, flushings, copious sweat, and trophic disorders in the integument and its appendages, seem to show that a lesion in the gray matter just posterior and external to the central canal is necessary for such phenomena. Similar vaso-motor changes may be observed in some of the tabulated cases, as in No. 4, in which there were islands of heat and cold in the leg, with a hydromyelia in the cord. Sensory symptoms are very common in cases of spinal tumor, but they furnish indications rather of the exact level of the lesion than of its region.
7 “Localization of the Functions of Spinal Cord,” Am. Journ. Neur. and Psych., 2-3, p. 443.
8 Quoted by Starr.
FIG. 45.
Diagram of Spinal Column, Cord, and Nerve-exits (after Gowers).
FIG. 46.
Sarcoma compressing Cervical Cord, Case 17 of Table (E. Long Fox).
Before considering briefly the indications which point to the various levels of the cord as a possible seat of spinal tumor, it will be necessary also to make plain a few anatomical facts. It must be borne in mind, first, that the nerve-origins in the cord are never at the same level as their exits from the spinal canal, or, in other words, that the spinal segments do not correspond with the bodies of the same numerical vertebræ, and that there is, in fact, one more cervical segment than there are cervical vertebræ. The tendency is for the nerve-trunks to run downward before passing out of the canal, so that in every instance, without exception, from the medulla oblongata to the filum terminale the segments of the cord are above the corresponding vertebral body. This discrepancy increases as we descend the cord; whereas it is approximately correct to say of the cervical and dorsal regions that every segment is opposite the vertebral body which is numerically just above it, this difference becomes much greater in the lumbar and sacral regions. The cord itself terminates in the lumbar enlargement which ends opposite the interval between the first and second lumbar vertebræ. All the remainder of the canal is occupied by the descending trunks of the lumbar, sacral, and coccygeal nerves as they pass to their respective foramina, constituting the cauda equina. It must be recalled, however, that the vertebral bodies, lying very deep, cannot serve as guides, but that we are dependent upon the spinous processes as landmarks in diagnosis. These again differ in their levels from their respective vertebral bodies, as they are deflected at somewhat different angles at different regions of the spine. Gowers has illustrated these facts by a very graphic wood-cut9 (Fig. 45), from which the general rule may be drawn that each vertebral spine is about opposite the spinal segment which is numerically two places below it; thus the eighth dorsal spine is opposite the tenth dorsal segment, etc. The indications afforded by this exact anatomical knowledge have reference largely to the existence of pain on pressure and to any deformity of the bony structures. The cases as reported do not indicate that this method of research has been utilized, and it may possibly be of only theoretical importance; but it has been considered worthy of reference as an indication in diagnosis.
9 Diagnosis of Diseases of Spinal Cord, p. 6.
Sarcoma of Lower Cervical Cord, Case 13 of Table (Adamkiewicz).
It will be seen by reference to the table that usually certain general features in the symptomatology indicate the seat of the lesion. Thus in tumors of the cervical region pain and stiffness of the neck occur, while the first appearances of paresis and sensory disturbances are usually observed in the arms and about the chest. The centres for the forearm and hand lie in the lower portion of the cervical enlargement; that for the upper arm, including the supinator longus, in the upper portion. Mental symptoms are more marked, and in Case 4 several of the cranial nerves were implicated. In the lower cervical and upper dorsal region there are symptoms of dyspnœa, fixation of the chest (Nos. 20, 22, 24), and cough. The girdle symptom is an important indication at any level, as it is due to irritation of the nerves at the lowest level of healthy cord just above the transverse lesion. It has already been discussed under Symptomatology. In many of the dorsal cases (Nos. 32, 37, 38, 39, and 45) the symptoms are almost entirely confined to the legs and lower trunk, the arms escaping entirely. The condition of the bladder is usually given in the table as one of paralysis; this does not indicate whether automatic evacuation existed at the beginning of the case; which condition would indicate that the centre for micturition was below the lesion, and intact. It is probable that later in these cases the bladder is actually paralyzed by destruction of its centre in the cord, and this even when the tumor has been situated some distance above.
FIG. 51.
Fibroma of Lower Dorsal Cord, Case 32 of Table (W. Cayley).
FIG. 52.
Tumor of Cauda Equina, Case 45 of Table (W. W. Fisher).
With reference to tumors of the cauda equina, Erb10 says that they have in every respect a great resemblance to those which are situated higher and affect the cord proper. “They are hard to distinguish from the latter, but may be in many cases perhaps, if it is borne in mind that tumors of the cauda produce exclusively nerve-root symptoms, and that the signs of compression of the cord, of secondary myelitis, etc. are absent. The higher the tumor, the nearer it approaches the lumbar portion of the cord, the harder will it be to draw the distinction. In respect to tumors seated lower the following points may be attended to: the seat of the pains (which in such cases often attain enormous violence) is strictly localized in certain nerve-districts; all nerves leaving the spinal canal above the tumor are free; thus in myxo-sarcoma telangiectodes of the cauda I observed the pain strictly limited to the district of the sciatica, while the crural and the dorsal nerves were perfectly free; constant violent pain in the sacrum. If palsy occurs the reflex actions necessarily cease at once. Spasms are seldom observed, more frequently contractures. Atrophy of the muscles occurs rather frequently. The palsy and anæsthesia by their localization often give us the opportunity of fixing the upper limit of the lesion. Increase of the reflex acts and marked tendinous reflexions do not occur. Paraplegia, palsy of the bladder, bed-sores, etc. may develop exactly as in tumors occupying a higher seat, but the symptoms of paralysis do not seem to belong necessarily to the disease, as is shown in my case (just mentioned), which terminated fatally before paralysis or anæsthesia occurred.”
10 Op. cit.
Psammoma of Dorsal Cord, 38 of Table (after Charcot).
PROGNOSIS.—The prognosis of spinal tumors is generally very unfavorable. Syphilitic cases are of course the most hopeful, but even in these cases it is only when they are recognized early that much can be expected. A gumma that has grown to any dimensions will have so compressed the cord that even when the tumor is melted away by specific treatment its effects will remain.
DURATION AND TERMINATION.—Most cases of spinal tumor last from about six months to three years. Occasionally death may result, as from a rapidly-developing sarcoma, in less than six months, and somewhat more frequently in slowly-developing tumors, or in those which are held more or less in abeyance by treatment the sufferings of the patient are prolonged to four or five years or more. Hemorrhages into or around the growths sometimes take place, and are the cause of death, or more frequently of a sudden aggravation and multiplication of severe symptoms. Death sometimes takes place from the complete exhaustion which results from the disease and its accompanying secondary disorders, such as bed-sores, pyelitis, etc. Occasionally death results from intercurrent diseases, such as pneumonia, infectious fevers, etc., whose violence the weakened patient cannot well withstand. Sometimes the symptoms of a rapidly-ascending paralysis appear, probably due to an ascending myelitis or meningo-myelitis.
COMPLICATIONS AND SEQUELÆ.—Spinal tumors are sometimes complicated with other similar growths in the brain or the evidences of the same constitutional infection in other parts of the body. In one case of cysticercus of the cord sclerosis of the posterior columns was also present.
TREATMENT.—The treatment of spinal tumors can be compressed into very small compass. In cases with syphilitic history, or when such history is suspected, although not admitted, antisyphilitic remedies should be applied with great vigor. It should be borne in mind, however, that even in syphilitic cases after destruction of the cord by compression or softening specific remedies will be of no avail. In tubercular cases and in those in which the system is much run down tonics and nutritives are indicated. Bramwell11 advises an operation in any case in which the symptoms are urgent, in which the diagnosis clearly indicates the presence of a tumor, when there is no evidence of malignant disease, when the exact position of the growth can be determined, and when a vigorous antisyphilitic treatment has failed to produce beneficial results. As some meningitis, meningo-myelitis, or myelitis is usually present in cases of spinal tumor, treatment for the complication will assist in relieving the torments of the patient. Anodynes, particularly opium and its preparations, should be used freely in the later stages of the affection. Bromides and chloral are of little value except in association with opiates. Operation offers even less hope than in brain tumor, but in very rare cases should be taken into consideration.
11 Diseases of the Spinal Cord, Edinburgh, 1884.
TABLE OF FIFTY CASES OF SPINAL TUMOR.