Chapter 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.

[Footnote 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:

----+-----------------------+--------------------+------------------
No. | Reporter and Journal. | Symptoms. |Results.—Remarks.
----+-----------------------+--------------------+------------------
1 | Luis Streisand | Epilepsy, delirium | Rapid cure with
| _Die Lues als Ursache | of exaltation, | mercury.
| der Dementia_, Inaug. | alteration of |
| Diss., Berlin, 1878. | speech, headache, |
| | failure of memory. |
----+-----------------------+--------------------+------------------
2 | _Ibid._ | Delusions, | Cure with
| | delirium, general | mercury.
| | mania, great |
| | muscular weakness. |
----+-----------------------+--------------------+------------------
3 | Müller of Leutkirch | Symptoms resembling| Cure by iodide of
| _Journ. of Mental | general paralysis, | potassium.
| Dis._, 1873-74, 561. | and diagnosis of |
| | such made until a |
| | sternal node was |
| | discovered. |
----+-----------------------+--------------------+------------------
4 | Esmarch and W. Jersen | Sleeplessness, | Cure by mercury.
| _Allgem. Zeitschrift | great excitement, |
| f. Psychiatrie_. | restlessness, |
| | great activity, |
| | incoherence, and |
| | violence. |
----+-----------------------+--------------------+------------------
5 | Leidesdorf | Complete mania; | Complete cure by
| _Medizin. | played with his | iodide of
| Jahrbucher_, xx., | excrement, and | potassium.
| 1864, 1. | entirely |
| | irrational. |
----+-----------------------+--------------------+------------------
6 | Beauregard | Symptoms resembling| Cure by iodide of
| _Gaz. hébdom. de Sci. | those of general | potassium.
| méd. de Bordeaux_, | paralysis. |
| 1880, p. 64. | |
----+-----------------------+--------------------+------------------
7 | M. Rendu | Loss of memory, | Mercurial
| _Ibid._ | headache, | treatment, cure.
| | irregularity of |
| | pupils, ambitious |
| | delirium, periods |
| | of excitement, |
| | others of |
| | depression, |
| | embarrassment of |
| | speech, access of |
| | furious delirium, |
| | ending in stupor. | {1020}
----+-----------------------+--------------------+------------------
8 | M. Rendu | Hypochondria, | Mercurial
| _Gaz. hébdom. de Sci. | irregularity of | treatment, cure.
| méd. de Bordeaux_, | pupils, headache, |
| 1880, p. 64. | failure of memory, |
| | melancholy, stupor.|
----+-----------------------+--------------------+------------------
9 | Albrecht Erlenmeyer | Melancholia with | Iodide of
| _Die Luëtischen | hypochondriasis, | potassium, cure.
| Psychosen_, Neuwied, | sleeplessness, |
| 1877. | fear of men, and |
| | belief they were |
| | all leagued |
| | against him. |
----+-----------------------+--------------------+------------------
10 | _Ibid._ | Religious | Iodide of
| | melancholia, with | potassium, cure.
| | two attempts at |
| | suicide, ending in |
| | mania. |
----+-----------------------+--------------------+------------------
11 | _Ibid._ | At times very | Iodide of
| | violent, yelling, | potassium, cure.
| | shrieking, |
| | destroying |
| | everything she |
| | could get hands |
| | on, at times |
| | erotomania; no |
| | distinct history |
| | of infection, but |
| | her habits known |
| | to be bad, and had |
| | bone ozæna and |
| | other physical |
| | syphilitic signs. |
----+-----------------------+--------------------+------------------
12 | _Ibid._ | Epileptic attack | Cured by
| | followed by a long | mercurial
| | soporose condition,| inunction.
| | ending in mental |
| | confusion, he not |
| | knowing his nearest|
| | friends, etc.; |
| | almost dementia. |
----+-----------------------+--------------------+------------------
13 | _Ibid._ | Great fear of | Cured by
| | gensd'armes, etc., | mercurial
| | mania, with | inunctions with
| | hallucinations, | iodide
| | loud crying, | internally;
| | yelling, etc., | subsequently
| | then convulsion, | return of
| | followed by great | convulsions,
| | difficulty of | followed by
| | speech. | hemiplegia and
| | | death.
----+-----------------------+--------------------+------------------
14 | _Ibid._ | Great unnatural | Iodide of
| | vivacity and | potassium, cure.
| | loquacity, wanted | Attended to
| | to buy everything, | business, and
| | bragged of enormous| seems as well as
| | gains at play, | before. Relapsed.
| | etc.; some trouble | (See Symptoms.)
| | of speech. |
| | |
| _Ibid._ | Fifteen months | Failure of
| Relapse of Case 14. | after discharge | various
| | from asylum | anti-specific
| | relapse; symptoms | treatment.
| | developing very |
| | rapidly, delirium |
| | of grandeur of the |
| | most aggravated |
| | type, with marked |
| | progressive |
| | dementia, failure |
| | of power of speech,|
| | and finally of |
| | locomotion. |
----+-----------------------+--------------------+------------------
15 | A. Erlenmeyer | Failure of mental | Iodide of
| _Die Luëtischen_, | powers, inequality | potassium in
| etc. | of pupils, | ascending doses
| | trembling of lip | failed. Recovery
| | when speaking, | under mercurial
| | uncertainty of | inunctions.
| | gait, almost |
| | entire loss of |
| | memory, once |
| | temporary ptosis |
| | and strabismus. |
----+-----------------------+--------------------+------------------
16 | _Ibid._ | Failure of mental | Iodide of
| | powers, pronounced | potassium,
| | delirium of | corrosive-
| | grandeur, | sublimate
| | hallucinations of | injections. Cure.
| | hearing, failure of|
| | memory, strabismus |
| | and ptosis coming |
| | on late. |
----+-----------------------+--------------------+------------------
17 | _Ibid._ | Failure of memory | Cure with use of
| | and mental powers, | iodide and
| | slight ideas of | mercurial
| | grandeur, | inunctions.
| | disturbance of |
| | sensibility and |
| | motility, aphasia |
| | coming on late. |
----+-----------------------+--------------------+------------------
18 | _Ibid._ | Melancholy, great | Iodide of
| | excitability, | potassium failed;
| | ideas of grandeur; | mercurial course
| | after a long time | improved; joint
| | sudden ptosis and | use cured
| | strabismus. | patient.
----+-----------------------+--------------------+------------------
19 | _Ibid._ | Various cerebral |
| | nerve palsies, |
| | great relief by |
| | use of mercurial |
| | inunctions, then |
| | development of |
| | great excitement, |
| | delirium of |
| | grandeur, failure |
| | of memory and |
| | mental powers, and |
| | finally death from |
| | apoplexy; no |
| | autopsy. |
----+-----------------------+--------------------+------------------
20 | J. B. Chapin | Melancholia with | Iodide of
| _Amer. Journ. | attempted suicide, | potassium, cure.
| Insanity_, vol. xv. | epilepsy, headache,|
| p. 249. | somnolent spells. |
----+-----------------------+--------------------+------------------
21 | _Ibid._ | Acute mania, noisy,| Iodide of
| | very destructive; | potassium, cure.
| | syphilitic disease |
| | of tibia. |
----+-----------------------+--------------------+------------------
22 | Snel | Maniacal | Cured by specific
| | excitement. | treatment.
----+-----------------------+--------------------+------------------
23 | Wm. Smith | Apathetic | Rapidly cured by
| _Brit. Med. Journ._, | melancholy, | conjoint use of
| July, 1868, p. 30. | indelicate, | iodide and
| | speaking only in | mercurials. The
| | monosyllables, and | symptoms first
| | much of the time | developed 3
| | not at all, sullen | months after
| | and menacing. | chancre.
----+-----------------------+--------------------+------------------

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 {1021} 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. Mendel[64] 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.

[Footnote 64: _Progres. Paral. der Irren_, Berlin, 1880.]

[Footnote 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. Chauvet[66] for an epitome of the most important recorded opinions.

[Footnote 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]

[Footnote 67: _Paralysie générale des Alienés_, 1879.]

[Footnote 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]

[Footnote 69: _Loc. cit._]

[Footnote 70: _Loc. cit._]

[Footnote 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.

{1022} 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 {1023} 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 Huebner[72] 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.

[Footnote 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 {1024} 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]

[Footnote 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 {1025} 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.

[Footnote 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 {1026} 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.

[Footnote 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 {1027} 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]

[Footnote 76: _American Journ. of Neur. and Psychiat._, vol. ii.]

{1028}

TUMORS OF THE BRAIN AND ITS ENVELOPES.

BY CHARLES K. MILLS, A.M., M.D., AND JAMES HENDRIE LLOYD A.M., M.D.

DEFINITION.—Under the head of Tumors of the Brain and its Envelopes will be considered all forms of growths occurring within the skull, whether these involve the cranium itself, the membranes of the brain, the brain-substance, or several of these parts conjointly. A large majority of these growths spring from the brain-membranes.[1]

[Footnote 1: To this article will be appended a table of one hundred cases of brain tumor. Our researches included the investigation of many more cases, four to five hundred in all. Such a table, indeed, could be indefinitely extended. Our object, however, has been not so much to present a large number of cases, and these in great detail, but rather in the most condensed manner to give a definite number, carefully selected, from which to draw conclusions. The cases have not been chosen with the view of upholding any peculiar or preconceived views as to pathology, diagnosis, localization, etc., but because of the carefulness with which they have been recorded. They have been selected also, as will be seen, with the view of determining by clinico-pathological data the possibility of localizing such growths during life. Many of our general conclusions as to pathology, symptomatology, and diagnosis have been drawn from this table.]

With the exception of such merely substitutional terms as cerebral or intracranial growths, neoplasms, or adventitious products, we have no general synonyms for brain tumors.

The literature of the subject of brain tumors is second only to that of such subjects as syphilis and hysteria. Vol. ii. of the _Index Catalogue of the Library of the Surgeon-General's Office, U. S. A._, contains no less than 632 references to this subject: number of books, 43; articles, 589. The books and papers occur in different languages, as follows: British, 142; French, 174; German, 133; American, 91; Italian, 30; Latin, 15; Swedish, 14; miscellaneous, 33.

ETIOLOGY.—Under predisposing or constitutional causes are first to be classed such special inheritances as tuberculosis or carcinoma and tendencies to vascular degeneration. The occurrence of syphilis of course predisposes its victims to cerebral or membranous growths, as it does to other so-called tertiary forms of this disorder.

Hereditary predisposition only enters in so far as the individual inherits a general tendency to the development of such affections as cancer and tubercle. Hereditary tendency to a brain tumor per se does not exist, but the individual who is of the tuberculous or cancerous diathesis under special causes may develop an intracranial growth. As to the comparative frequency with which diathetic tumors originate, a reference to the tabular statement which will be given under {1029} Pathology will be sufficient. Gliomata are very common, but with them vie in frequency sarcomatous, tubercular, and gummatous growths. Any table, unless it includes a very large number of cases—at least a thousand or more—would be misleading as to the proportionate frequency of these different forms of intracranial constitutional diseases; but it is safe to say that syphilitic, tuberculous, and carcinomatous or sarcomatous tumors are of comparatively frequent occurrence.

Tumors of the brain occur oftener among men than women. This fact is dependent largely upon the difference between the habits and occupations of the two sexes. Men, in the first place, are much more addicted to alcoholic, venereal, and other abuses which give rise to special degenerations or constitutional infection; and secondly, they are more exposed to traumatisms. In 100 cases the tumors occurred among males in 58 cases, among females in 40 cases, and sex was not recorded in 2 cases.

Statistics show that intracranial growths are more likely to occur between puberty and middle age. Although gliomata may be found at any age, brain tumors in children are more likely to be of this character. This is what might be expected from the nature of these growths. Histologically, gliomata are most closely allied to the embryonal stage of the connective tissue, and, according to Cohnheim, tumors generally are the result of a surplusage of embryonal tissue—tissue which has remained over after the requirements of normal development have been met. Of 16 gliomata, 3 occurred in patients under ten years, 2 between the ages of ten and twenty, and 4 between twenty and thirty.

One hundred cases gave the following results as to age:

Under 10 years . . . . 10 cases.
From 10 to 20 years . . 12 "
" 20 to 30 " . . 18 "
" 30 to 40 " . . 24 "
" 40 to 50 " . . 12 "
" 50 to 60 " . . 13 "
" 60 to 70 " . . 3 "
Over 70 years . . . . . 1 "
Not recorded . . . . . 7 "
----------
100 cases.

It is now generally admitted that injuries play a most important part as exciting causes of brain tumors. Frequently in our experience an apparently direct relation has existed between a head injury and the origin of the neoplasm. In 6 out of 12 cases reported by one of us,[2] a history of traumatism was present, although in 5 of these a history of syphilis was also present. The great frequency with which injuries of all kinds occur must of course not be overlooked in this connection. It is said by those who oppose the idea of the direct causal relations of injury that almost every one could trace such disease to falls or blows which few escape. In some of the cases of brain tumor, however, the history of injury bears a direct relation in time to the initial symptoms of the tumor. Certain tumors, as the fibromata, osteomata, angiomata, would appear to be of more frequent occurrence as the direct result of traumatism. The part played by injuries in the production of carcinomata and sarcomata, whether in the brain or elsewhere, has not {1030} infrequently been the subject of dispute. We have no doubt that, a constitutional predisposition existing, an injury frequently leads directly to the development of some form of malignant growth. In not a few of the syphilitic cases the history would appear to show that an injury to the skull had localized the constitutional poison.

[Footnote 2: _Archives of Medicine_, vol. viii. No. 1, August, 1882.]

Echinococci and cysticerci are found within the cranium, and sometimes give rise to tumors, but the statement of Obernier can hardly be regarded as true, that they play an important part in the production of cerebral tumors. Our table shows only two cases of this kind.

SYMPTOMATOLOGY.—The symptoms of intracranial tumors from the standpoint of the course or progress of the affection can be divided into an early or beginning, a middle or developed, and a late or terminal stage.

Headache, vertigo, and vomiting are early symptoms, varying in severity from slight to very serious manifestations, and slight and changing mental phenomena are present. Eye symptoms, such as slight diminution or blurring of vision, may or may not show themselves; the ophthalmoscope may reveal the earliest appearances of choked disc or neuro-retinitis.

In the second or middle stage, the period of the fully-developed disease, we have an intensification and greatly increased constancy of all the general symptoms, with some additional manifestations. Mental disorder increases; headache becomes more violent, and sometimes more localized; amblyopia tends to advance to complete blindness, with marked swelling of the papilla, or special disorders of vision, such as hemianopsia, occur; palsies, ataxia, convulsions, contractures, rigidity of limbs, altered reflexes, local spasms, anæsthesia, hyperæsthesia, paræsthesiæ, neuralgias, appear according to the extent and location of the growth. Certain special phenomena, according largely to the seat of the tumor, may also appear, such as disorders of hearing, taste, or smell, polyuria, diabetes, albuminuria, polyphagia, or dysphagia.

In the third or terminal stage paralysis, anæsthesia, convulsions, etc. become more profound; mentality sometimes decreases to nearly complete imbecility, although in many cases a fair amount of mental power is preserved almost to dissolution. Involvement of the bowels and bladder becomes a distressing symptom, involuntary evacuations adding to the torments of the patient. Bed-sores, acquired or trophic, appear; the patient's limbs are painfully contractured; great emaciation is usually present. With agonized head, often blind or deaf, with torturing pains in anæsthetic, paralyzed, and wasted limbs, covered with painful sores, filthy involuntarily, imbecile or demented, death comes as a thrice-happy relief.

Cases have been reported in which intracranial tumors of considerable size were discovered on autopsy, and had evidently been present for years without their presence having been discovered or even suspected; but these are extremely rare. In general terms, however, it may be said that every case of brain tumor must give rise to symptoms. These symptoms may be few or many, slight or severe, brief or protracted, constant or paroxysmal, according to circumstances. Our experience and study of the literature of the subject lead us to the conclusion that the pathological character of the growth would have some influence as to the severity and prominence of symptoms. Gliomata—which are {1031} sometimes hard to distinguish from the brain-substance, particularly the soft gliomata, producing comparatively little pressure—when located in certain so-called latent areas of the brain might escape detection during life; so might also very small tumors of any kind situated deeply in the substance of the brain.

The position taken by Obernier, that individuality has much to do with the activity of symptoms of brain tumor, has some strength. He refers particularly to the psychical response to the irritation caused by brain tumors, which differs very much in different individuals. The fact, which has been pointed out in the article on Hysteria, that hysterical symptoms are often present in cases of brain tumor as well as in other forms of organic disease, is also sometimes the cause of mistake by the diagnostician. The cases of Hughes Bennett and Eskridge will be referred to in this connection under Diagnosis.

When the subject of local diagnosis is considered farther on, those regions which are most active and those which hold an intermediate position in the production of particular and distinctive symptoms will be more particularly discussed.

* * * * *

The possibility of the existence of multiple tumors should never be overlooked. These are of comparatively frequent occurrence, and sometimes give rise to a puzzling symptomatology. In our search through the literature of the subject for cases we met with many examples of multiple tumors. In preparing our table multiple tumors were usually omitted. They give sometimes symptoms and signs so conflicting as to make a local diagnosis impossible, and even to confuse the expert as to general diagnosis. In other cases, however, one growth of larger size or in an active region takes command of the situation, and leads clearly to its diagnosis in spite of other less significant tumors. This was shown, for example, in one of our cases (Case 14). From the symptoms not only was the presence of the larger tumor diagnosticated, but its exact location was indicated during life. Post-mortem examination, however, showed a smaller tumor at the inferior angle of the right lobe of the cerebellum, and also some basal meningitis with effusion which had not been suspected. The first tumor was one of the ascending frontal convolution, extending into the fissure of Rolando. Its presence and location had been diagnosticated by the right brachial monoplegia and paresis of the leg, which later increased to paralysis of the limb, with right facial paresis, ptosis, partial anæsthesia of right side of face, and slight clonic spasms of the right arm. At the present time, when the question of operating for the removal of brain tumors is so prominently before the profession, and is now generally regarded as justifiable for growths in this one location, the cortical motor area, the possibility of the coexistence of another tumor should be carefully weighed. In this case, owing to the clear diagnostic indications, the question of operation was considered at the time of consultation, but fortunately—in large part because the patient was almost in extremis—it was decided not to operate. The operation would have been futile, the autopsy showing that the probable immediate cause of death was the basilar effusion which accompanied the cerebellar growth.

In another case reported by one of us[3] three gummata were found in {1032} three different locations: one in the prefrontal region, another in the retro-central fissure, and a third in the supramarginal convolution. The general symptoms of brain tumor were present in this case, but no localization was possible.

[Footnote 3: _Arch. Med._, viii. No. 1, August, 1882.]

The following are other illustrations of multiple tumors and of tumors with other lesions out of a large number which we have collected:

M. 44.—Several severe falls on the head. Recent chancre with secondary symptoms. Six months before death headache which grew worse; most severe in right supraorbital region, and also obstinate vomiting, vertigo, hiccough, insomnia. Some loss of power on both sides. Slow in answering and indisposed to talk. Choked discs. Involuntary evacuations. Some improvement under treatment, and then relapse. Complete blindness. Polyuria. Specific gravity of urine as low as 1003 and 1005. Appetite at times ravenous. During last few weeks of illness head inclined to the right side, probably from spasm. Intense thirst throughout the illness.

(1) Large sarcoma in the white substance of the right parietal and frontal lobes, involving ascending parietal convolutions. (2) Softening and abscess of the right temporo-sphenoidal lobe and posterior base of the right anterior lobe. Softened mass size of a hickory-nut in the upper portion of the right ascending frontal convolution. (3) Small cystic tumors of the choroid plexuses.[4]

[Footnote 4: J. T. Eskridge, _Trans. of Philada. Path. Society_, for 1878-79, vol. ix. p. 119.]

M. 5.—Convulsions, beginning either in right foot or in right face; not unconscious during fits. Right-sided hemiplegia. Double optic neuritis, followed by atrophy. Gradual increase in size of head, with gaping sutures. Later, rigidity of right arm and leg. Shortly before death the following symptoms suddenly set in: headache, convulsions of right side with unconsciousness and retraction of head, rigidity of right arm, and tremor of right leg. Later, both arms flexed, with constant tremors of left arm, spreading over whole body. Right sterno-cleido-mastoid was stiff; almost opisthotonos.

(1) Enormous tubercular tumor of left cerebral hemisphere, extending from depth of Sylvian fissure and eroding skull. Corpus striatum soft and diffluent. Weight, over eleven ounces. (2) Tumor, double size of pea, in lower part of middle lobe of cerebellum.[5] This case was especially interesting for the onset of the cerebellar symptoms late in the case, caused probably by a late appearance of the cerebellar tumor.

[Footnote 5: Hughlings-Jackson, _Med. Times and Gaz._, London, 1872-73 (2), p. 34.]

M. 2.—Screaming fits. Vomiting. Paralysis of left side of face, including corrugator and orbicularis palpebrarum. Winking in both eyes suspended. Paralysis of external rectus muscle. Agitation and contractures of right arm and leg. Later, unsteady movement of head and right arm and jerking of right leg.

(1) Small glioma in left hemisphere of cerebellum, and (2) a similar tumor projecting from left lateral half of floor of fourth ventricle at common nucleus of the abducens and facial nerves.[6]

[Footnote 6: W. H. Broadbent, _Proc. Clin. Soc. Lond._, v. 66-68.]

F. 45.—Epileptiform attacks for fourteen years. Vertigo, occipital headache, disordered vision, intolerance of light, sleeplessness, excitability, constipation, with paræsthesia of left side. Retraction of head, {1033} vomiting. Very late, convulsive movements of right eye and right side of mouth; then general clonic spasms, coma, and death. No paralysis. For twenty-six years there had been an induration of one breast, which toward end of life had a somewhat scirrhous appearance.

(1) Glioma in right hemisphere of cerebellum 1¾ inches in diameter. (2) Glioma in centre of middle lobe of left hemisphere of the cerebrum ¾ inch in diameter. Surrounding nervous tissue softened and broken down.[7]

[Footnote 7: William L. Bradley, _Tr. Conn. Med. Soc._, 1880, p. 39.]

M. 65.—Sudden attack of spasm of left arm, with paresis and coldness and numbness. Headache and staggering. Wandering in mind. Not unconscious. Twitching in eye. Two days later violent convulsion in entire left half of body, with vomiting; not unconscious. After convulsion left hemiplegia, with left deviation of tongue. Head and eyes turned to right; vertigo. Partial anæsthesia of left face, body, and limbs. Paræsthesia; painful nervous shocks through affected limbs. Sounds in back of head. Later, mind cleared up and many symptoms ameliorated. Very late, convulsion and coma. Cheyne-Stokes respiration, involuntary evacuations. Duration, seven weeks.

(1) Tumor in ascending parietal convolution at junction of upper and middle thirds. (2) Large tumor occupied entire occipital lobe, but did not present on surface, reaching to convexity of descending cornu of lateral ventricle.[8]

[Footnote 8: W. H. Broadbent, _Tr. Clin. Soc. Lond._, v. 233-236.]

* * * * *

Headache is the most frequent and positive symptom of brain tumor. It is rarely absent; in most cases it has peculiar characteristics. Its usual type might be described as continuous pain, sometimes of persistent severity, but generally with exacerbations or paroxysms of great violence. No suffering can surpass that which some of the victims of intracranial neoplasms are compelled for months or years to endure. It is only equalled by the torture produced by malignant growths in the vertebral axis, the throat, or the bones of the pelvis. The pain is not, however, always of this character. In a comparatively few cases it is described by the patient as dull or moderate, or he simply complains of distressing sensations of weight, pressure, or constriction. Usually in these cases, however, the pain increases and becomes less and less endurable. In 100 cases the headache is described as agonizing, violent, severe, or torturing 20 times; as moderate or dull, 26 times. It is simply mentioned 20 times, but here presumably it was of the ordinary severe type. Thus in 66 cases, or 66 per cent., headache of some type was recorded. No mention was made of headache at all in 29 cases, in some of which, however, it was doubtless present. In only 5 cases was it stated not to have been present: 3 out of these 5 were said to be gliomata, and of the other 2, one was probably gliomatous, and the other a cyst in the brain-substance. Tumors of this kind, which exert comparatively little pressure and which are not connected with the membranes, are probably those which are least likely to cause pain. In several instances the patients complained spontaneously of the pain being greatest in the region of the head nearest the seat of growth. With reference to cerebellar tumors, it somewhat generally supposed that occipital pain is usually present. It {1034} sometimes is and sometimes is not. In 15 cases of tumor of the cerebellum and of the fourth ventricle the pain was described as occipital twice, as fronto-occipital three times, and as cervico-occipital once. Callender has noticed that cortical lesions are more frequently accompanied by localized pains than lesions of deeper parts.[9] Some of our cases would seem to bear out this view, although the data are not numerous or complete.

[Footnote 9: _St. Bartholomew's Hospital Reports_, 1869, and Ferrier's _Localization of Cerebral Disease_, p. 99.]

In a few cases percussion of the head elicited or intensified pain in the region corresponding to the location of the tumors. We believe that percussion of the skull may afford valuable aid in localizing encephalic tumors, particularly when they are meningeal or cortical. Ferrier[10] holds that percussion often elicits pain over the cranial region beneath which a lesion is localized, and Alex. Robertson of Glasgow[11] has also directed attention to the value of percussion of the skull in the localization of disease on the surface of the brain.

[Footnote 10: _Brain_, January, 1879.]

[Footnote 11: _Abstr. Internat. Med. Congress_, 1881, p. 85, and _Journal of Nervous and Mental Disease_, April, 1882.]

Vomiting is of frequent occurrence, although it does not usually come on as early or remain as persistently as headache. It is more likely to be paroxysmal, with longer or shorter intervals of relief. It frequently occurs at the time of the exacerbations of the headache, and between the paroxysms of vomiting, nausea, slight or great, is often present. It probably occurs in about 50 per cent. of all cases of brain tumor. It is recorded as having been present in 34 out of 100 cases. It was comparatively infrequent in antero-frontal growths, occurring only twice out of 10 cases.

A case reported by one of us (Case 70) was a remarkable example of persistent cerebral vomiting. For four weeks before her death the patient vomited almost incessantly. The tumor in this case involved the middle cerebellar peduncle and the adjacent region of the right cerebellar hemisphere, and had grazed and superficially softened the floor of the fourth ventricle.

We incline to agree with Ferrier[12] that the majority of cases of cerebral vomiting can be ascribed to irradiation of irritation by the nerves of the cerebral membranes or to the physical effects of acute pain. The nuclei of the trigeminus and of the vagus are in close anatomical relationship in the medulla oblongata. Irritative impressions conveyed from the cerebral membranes, and particularly from the dura mater, to the trigeminal nuclei in the medulla are transferred to the vagus and give rise to vomiting. In tumors of the cerebellum the vomiting may be brought about through the affection of the centres of equilibration and the concurrent vertiginous sensations (Ferrier), or it may be due to direct irritation by contiguity of the floor of the fourth ventricle. In general, the tumors situated farthest forward in the brain appeared to be the least likely to give rise to vomiting.

[Footnote 12: _Brain_, July, 1879.]

Vertigo was noted in 31 out of 100 cases. Under this general designation dizziness, giddiness, reeling, insecurity in the erect posture, etc. have been included. The vertigo can be explained in several ways {1035} besides by the view which refers it to an affection of centres of equilibration in the cerebellum. Like headache and vomiting, it seems sometimes to be the outcome of pure irritation of the cerebral membranes. Alterations in the intracranial circulation produced by the growth of the tumor is another probable cause. Experiments have demonstrated the existence of a communication between the arachnoid cavity and the labyrinth, and consequently the increased intracranial tension present may result in pressure on the labyrinthine fibres of the auditory nerve, and thus occasion vertigo.

The importance of ophthalmoscopic examinations in suspected cases of brain tumor can scarcely be overestimated. They will often serve to clinch the diagnosis in favor of tumor. Of the 100 tabulated cases, choked discs were recorded in 8; optic neuritis or neuro-retinitis in 18. These results serve to show the importance of the examination of the eyes. It will be seen that with Allbutt, Norris, and others we make a distinction between choked disc, papillitis, congestion papilla, or ischæmia of the discs, and descending or interstitial neuritis. The differences between these two varieties of inflammation of the interocular ends of the optic nerve are well presented by Norris in a previous volume of this work,[13] which will make it unnecessary for us to describe the conditions present in these cases. An examination of the tabulated cases will show that true choked discs were present in tumors situated in all portions of the encephalon. They were not infrequently present in tumors of the convexity. Descending neuritis would seem most likely to occur when the tumors are so situated that inflammation set up by them can readily extend by anatomical continuity along the membranes to the optic nerves. The lymph-space theory best accounts for the occurrence of choked discs in cases of tumor situated remotely from the base. In choked disc the appearance is one of intense swelling and engorgement of the optic papilla. In descending neuritis, as described by Graefe and Norris, there is rather a dull-red suffusion of the disc. Some of the cases reported as neuritis may have been choked discs, and the reverse, as physicians often do not make any distinction.

[Footnote 13: Vol. IV. p. 771 _et seq._]

Several practical matters should be borne in mind in connection with this subject of the condition of the eye-ground. In the first place, sight is not always impaired in cases of even somewhat advanced choking of the disc, so that when other symptoms and indications lead to the suspicion of a brain tumor, unexpected confirmation may be obtained by ophthalmoscopic examination. Some remarkable cases of this kind have been reported, and doubtless have been observed by all ophthalmologists and neurologists of large practice. In some cases of growths of large dimensions also careful ophthalmoscopic examination has shown neither choked disc nor neuro-retinitis to be present. In two of Seguin's cases, for instance (Cases 28 and 29), these appearances were absent. In one he reports no neuro-retinitis, but only some fulness of the veins. In the other, a large sarcoma of the centrum ovale, ophthalmoscopic changes were absent. The absence of disturbance of vision, therefore, should not lead the physician to overlook making a thorough ophthalmoscopic examination; nor should the absence of ophthalmoscopic appearances lead him to make up his mind that serious intracranial disease was not present. {1036} The presence of double choked discs is in the highest degree significant of a brain tumor.

Observations on the temperature of the head have been made in a few cases of tumor of the brain. Full accounts of such observations in two cases (1 and 3 of Table) have been published by one of us.[14] It is not within the scope of the present paper to review the general subject of cranial or cerebral thermometry. We will simply, in passing, recall the labors of E. Seguin,[15] Broca,[16] Gray,[17] Lombard,[18] Maragliano and Seppilli,[19] and Amidon.[20]

[Footnote 14: _Philadelphia Medical Times_, Jan. 18, 1879, and _New York Medical Record_, Aug. 9, 1879.]

[Footnote 15: _Medical Thermometry and Human Temperature_, by E. Seguin, M.D., New York, 1876.]

[Footnote 16: _Progrès médical_, 1877.]

[Footnote 17: _New York Medical Journal_, Aug., 1878.]

[Footnote 18: _Experimental Researches on the Temperature of the Head_, London, 1881.]

[Footnote 19: Quoted in _Archives of Medicine_, 1879.]

[Footnote 20: _Archives of Medicine_, April, 1880.]