Chapter LXV: Introduction: Notwithstanding the numerous measurings and weighings of (1)

human brains made in the course of time by different investigators, no absolute standard measure or weight has as yet been established by which we can accurately determine a pathological increase or decrease in the size and weight of this organ. The want of such a standard is principally due to the difference generally existing in the dimensions and weights of even a certain number of brains taken from individuals belonging to the same race or nation. The same difficulties are met with in the attempt at establishing a rule by which to measure the mental capacity of a certain brain, for the question has as yet not been solved whether this capacity depends upon the quantity or quality of the brain-substance. In speaking of hypertrophy of the brain, therefore, we must keep in mind that a large brain must not be considered hypertrophied unless there exists a disproportion between its size and that of the cavity from which it was removed; in other words, when its growth or hypertrophy meets a resistance at the inner walls of the cranial cavity. According to Virchow, a further distinction must be made between the increase (hyperplasia) of the nervous elements themselves and that of the supporting connective tissue, the neuroglia. To the latter condition particularly corresponds the peculiar doughy consistence of the white substance of hypertrophied brains.

{997} ETIOLOGY.—Hypertrophy of the brain is sometimes congenital, and then associated with dwarfishness and a defective development of the cranium. Generally, however, it is an extra-uterine affection, originating mostly during infancy and childhood, though it is also met with at the age of puberty, and even in adult life. The male sex is more predisposed to the affection than the female. When hypertrophy of the brain occurs during infancy, it is almost always associated with an excessive development of the lymphatic glands, with a defective involution of the thymus gland, and with rachitis, but generally without cachexia. No special exciting cause can be assigned to the affection during youth or adult age, though it has been stated that during these periods of life the disease may supervene upon tubercle or carcinoma of the brain. Repeated active or passive congestion (Rokitanski, Andral), as well as the introduction of lead into the system, has also been supposed to incite the disease.

PATHOLOGICAL ANATOMY.—Directly after the removal of the vault of the cranium, and on cutting through the dura mater, the brain, as if liberated from pressure, is observed to swell out to such a degree as to render the replacement of the removed skull-cap impossible. The enveloping membranes are found closely adapted to one another and to the brain; they are very thin, bloodless, and dry, and their vessels are empty and pressed flat. The hemispheres of the cerebrum are large, and their convolutions, mutually pressing against one another, are flattened at their surfaces, so that the intervening sulci are hardly recognizable. After the removal of the brain from the skull the abnormal dimensions of the cerebrum present a remarkable contrast to the normal size of the cerebellum, pons, and medulla oblongata, which also appear flat and broad from pressure. When a horizontal section is made through the hemispheres of the cerebrum and a little above the corpus callosum, the centrum ovale appears unusually large. The cavities of the ventricles are very narrow, their walls touching one another. There is no serum in the pia mater or in the ventricles. The substance of the brain is pale, bloodless, and dry. The white substance, upon which the hypertrophy particularly depends, is of a doughy consistence, comparable to the boiled white of an egg or cheese, whilst the gray substance is so pale as to be hardly distinguishable from the former.

The condition of the skull is, according to Rokitanski,[1] as follows: In cases in which the hypertrophy of the brain has advanced to a high degree, and in which the sutures of the skull are united, the bones of the cranium are found thinner and their inner table roughened by absorption. This is especially the case in the bones which form the vault of the cranium. The holes or other deficiencies found at the base of the cranium in the plates of the frontal, ethmoid, and sphenoid bones are not entirely due to the absorbing process, but rather to the thinness of these bones. In infants the cavity of the cranium enlarges in proportion to the hypertrophy of its contents; the head then resembles in form so much that of hydrocephalus that it may lead to errors in diagnosis. In some cases in which the disease rapidly develops to a high degree there is observed on the infant's skull a loosening and separation of the sutures of the cranial vault, with red coloration and suffusion of their cartilages.

[Footnote 1: _Lehrbuch der Pathologischen Anatomie_, 3d ed., 1855, vol. ii. p. 431.]

{998} SYMPTOMS.—There is a gradually increasing muscular weakness, manifesting itself especially in the lower extremities, and giving rise to an unsteady, stumbling gait and frequent falling, caused perhaps by the excessive weight of the head, and also an inability of securely grasping objects. Besides these disturbances of motion, epileptic spasms appear, at first light in degree and at long intervals, but becoming later on in the course of the disease more frequent and severe. Continuous or intermittent attacks of headache almost always accompany the disease, and, furthermore, vertigo, tinnitus aurium, photophobia, and dimness of sight with dilatation of the pupil; general sensation also becomes blunted, but without ever amounting to anæsthesia. In a number of cases there is no disturbance of the psychical functions, though in others symptoms of mental excitement amounting even to delirium have been observed. Most frequently the intelligence sinks from the beginning of the disease, to end in complete idiocy. The pulse also has been observed to fall considerably during the last stage.

The COURSE of the disease is, according to Andral and Hasse,[2] almost always chronic, and if an acute development of the affection has been spoken of, it may be supposed that the latter has commenced a considerable time previous to the manifestation of the symptoms during the last stage. Andral divides the disease into two stages, of which the first is chronic and frequently latent, whilst the other is more acute and leads to a rapid fatal termination. In most cases death is caused by such intercurrent affections as give rise to irritation and hyperæmia of the brain, and thus increase the already abnormal pressure upon this organ.

[Footnote 2: “Krankheiten des Nervensystems,” 2d ed., 1869, in _Handbuch der Speciellen Pathologie und Therapie_, edited by R. Virchow, vol. iv. 1st div., p. 578.]

DIAGNOSIS, PROGNOSIS, AND TREATMENT.—It has already been mentioned that errors in diagnosis may very easily be committed on account of the great resemblance in the form of the head in cases of hypertrophy of the brain and of hydrocephalus, though it has been stated that in the former affection convulsions, in the form of epileptic spasms, predominate, to be followed during the last stage by symptoms of depression; whilst in hydrocephalus the symptoms of depression manifest themselves from the beginning of the disease, and, moreover, the rachitic deformities are more prominently shown in the form of a chicken-breast. A positive diagnosis can only be made by the autopsy.

There are no remarks to be made on the prognosis and treatment of hypertrophy of the brain.

{999}

SYPHILITIC AFFECTIONS OF THE NERVE-CENTRES.

BY H. C. WOOD, M.D., LL.D.

Introduction.

Syphilitic affections of the nerve-centres are best studied by separating those of the spinal cord from those of the cerebrum, and in the present article this natural division of the subject is adopted. Further, cerebral syphilis in its most characteristic or gummatous form usually attacks the brain-membranes, or perhaps in some cases the perivascular sheaths of the vessels, and only secondarily affects the tissue of the brain itself. The question of the occurrence of specific disease of the brain-cortex is so important that it shall have a separate discussion. It is perfectly well proven that with or without other brain lesion the vessels of the brain may undergo an atheromatous degeneration as the direct result of a syphilitic dyscrasia; but such disease links itself on the one hand with the subject of syphilitic disease of the general vascular system, and on the other hand with cerebral apoplexies, softenings, and other degenerations. Moreover, the space here allotted to brain syphilis is very insufficient. I, therefore, shall not enter upon the further discussion of syphilitic degeneration of the brain-vessels. The etiology of brain and spinal syphilis is best discussed under one heading.

* * * * *

GENERAL ETIOLOGY.—We do not know why in any individual case syphilis selects one portion of the nervous centres rather than another for attack; indeed, it is only rarely that any exciting cause can be discovered.

It is not unnatural to expect that any agency which is capable of exciting an inflammation of a nerve-centre may, when present in a syphilitic person, provoke a specific disease of such centre. Thus, thermic fever is a very common cause of chronic meningitis, and in the _Journ. de Méd. et Chir._ (Paris, 1879, p. 191) a case is reported in which cerebral syphilis followed a sunstroke; I have myself seen one similar instance, and in Roberts's case of precocious cerebral syphilis (see p. 804) the first convulsion came whilst the man was fishing on a very hot day, and may have been precipitated by the exposure.

Blows and other traumatisms would be expected to figure largely as exciting causes of nervous syphilis, but they, in fact, are only rarely present. I have seen one or two cases of specific brain disease attributed to violence by the patient, and several cases of possibly specific spinal {1000} disease—one in which a poliomyelitis followed a fall on the ice; one in which, after a fall from a cart and marked spinal concussion, a local myelitis developed;[1] and one of a general myelitis following an injury by a horse. The only records of such cases are those of Broadbent[2] and those collected by Heubner.[3]

[Footnote 1: _Univers. Hosp. Dispen. Service-Book_, x., 1875, p. 58.]

[Footnote 2: _Lond. Lancet_, 1876, ii. p. 741.]

[Footnote 3: _Ziemssen's Encyclopædia_, xii. 301.]

Various authorities attach much influence to over-study and other forms of cerebral strain in exciting brain syphilis. Engelstedt is stated to have reported cases having such etiological relations, and Fournier[4] affirms that he has especially seen the disease in professional men and other persons habitually exercising their brains to excess. Neither in private nor public practice have I met with an instance where over-brainwork could be considered a distinct etiological factor, whilst I have seen some hundreds of cases from amongst the laboring classes, in whom the intellectual faculties are chiefly dormant.

[Footnote 4: _La Syphilis du Cerveau_.]

The drift of the evidence in medical literature is so pronounced, and so in accord with my own experience, that I believe it may be positively affirmed that in the vast majority of cases of nervous syphilis no exciting cause can be found.

Inherited syphilis seems to be less prone than the acquired diathesis to attack the nervous system, but is certainly capable of so acting. As early as 1779, Joseph Glenck[5] reported a case of a girl, six years old, cured by a mercurial course of an epilepsy of three years' standing and of other manifestations of hereditary syphilis. Graefe found gummatous tumors in the cerebrum of a child nearly two years old.[6] O. Huebner[7] details the occurrence of pachymeningitis hæmorrhagica in a syphilitic infant under a year old. Hans Chiari[8] reports a case in which very pronounced syphilitic degeneration of the brain-vessels was found in a child fourteen months old. Both Barlow[9] and T. S. Dowse[10] report cases of nerve syphilis in male infants of fifteen months. For other similar cases the reader is referred to an article by J. Parrott,[11] and to a paper by M. E. Troisier.[12]

[Footnote 5: _Doctrina de Morbis Venereis_, Vienna.]

[Footnote 6: _Arch. f. Ophthalm._, Bd. i. Erst Abth.]

[Footnote 7: _Virchow's Archiv_, Bd. lxxxiv. 269.]

[Footnote 8: _Wien. Med. Wochenschrift_, xxxi. 1881, 17.]

[Footnote 9: _Lond. Patholog. Soc. Trans._, 1877.]

[Footnote 10: _The Brain and its Diseases_, vol. i. p. 76.]

[Footnote 11: _Archiv. de Physiologie_, 1871-72, p. 319; also to his “Leçons sur le Syphilis hered.,” _Progrès méd._, 1877 and 1878.]

[Footnote 12: _Arch. de Tocologie_, x. 411.]

Recorded cases prove decisively that even after puberty specific nervous affections may primarily attack the unfortunate offspring. Thus, Nettleship reports[13] the development of a cerebral gumma in a girl of ten years, and J. A.. Ormerod[14] of a tumor of the median nerve (probably gummatous) in a woman of twenty-three, both the subjects of inherited syphilis. Thomas S. Dowse[15] details a case of cerebral gumma at the age of ten years, and Samuel Wilks[16] one of epilepsy, from inherited taint, in a boy of fourteen. J. Hughlings-Jackson reports[17] paraplegia with epilepsy in a boy of eight, hemiplegia in a girl of eighteen, and hemiplegia in a woman of twenty-two;[18] the nervous affection in each case being associated with or dependent upon inherited syphilis. E. Mendel reports[19] a case of a child {1001} who had inherited syphilis, and developed in her fifteenth year a maniacal attack with hallucinations. I have seen cerebral syphilis occur at twenty-one years of age as the first evident outbreak of the inherited disorder.

[Footnote 13: _Trans. Lond. Path. Soc._, xxxii. 13.]

[Footnote 14: _Ibid._, p. 14.]

[Footnote 15: _Loc. cit._, p. 71.]

[Footnote 16: _Lectures on Dis. of Nerv. Syst._, Philada., 1878, p. 333.]

[Footnote 17: _Journ. Ment. and Nerv. Diseases_, 1875, p. 516.]

[Footnote 18: _Brit. Med. Journal_, May 18, 1872.]

[Footnote 19: _Archiv f. Psychiatrie_, Bd. i. 313.]

When a nervous affection develops first at a comparatively late period, and no very apparent evidences of the inherited taint are present, there is great danger of the case being misunderstood; indeed, in some instances an immediate diagnosis may be scarcely possible. It is probable that in most of the reported recoveries from alleged tubercular meningitis the disease has been syphilitic.

Some time since I saw, in an orphan of fourteen, a chronic basal meningitis, and in the absence of any history and of any evidences of syphilis gave the fatal prognosis of tubercular disease; but, to my astonishment, under the long-continued and free use of iodide of potassium complete recovery occurred. Another child, reported by a very good practitioner as cured of tubercular meningitis, and afterward for a long time under my own care, I believe suffered from hereditary syphilis. Cases of this character have also been reported by F. Dreyfous.[20]

[Footnote 20: _Revue mensuelle des Malad. des Enfants_, 1883, i. 497; see also _Gaz. hébdom. Sci. méd. de Montpellier_, 1883, v. 89.]

It is of course very important to diagnose between a tubercular meningitis and one due to hereditary syphilis. Without a history certainty is not possible, but a general indefiniteness of symptoms and slowness of progression should arouse suspicion, especially if the absence of the pulse-retardation indicated that the vault rather than the base of the cranium was involved.

The relation of inherited syphilis to various nervous affections not distinctly specific cannot yet be determined. Arrested development, and the consequent epilepsy, idiocy,[21] early brain sclerosis, are probably sometimes due to the inheritance; and the cases collected by E. Mendel[22] show that chronic hydrocephalus is frequently of specific origin.[23]

[Footnote 21: See _Brain_, vol. vii. 409.]

[Footnote 22: _Archiv f. Psychiatrie_, Bd. i. 309.]

[Footnote 23: See, also, _Virchow's Archiv_, Bd. xxxviii. p. 129.]

Another very important question connected with the etiology of these disorders is as to the time of their development. Nervous diseases following acquired syphilitic infection certainly belong to the advanced stages of the disorder. Huebner reports[24] a case in which thirty years elapsed between the contraction of the chancre and the nervous explosion. I have seen a similar period of thirty years. Fournier reports intervals of twenty-five years, and thinks from the third to the tenth year is the period of maximum frequency of nervous accidents.

[Footnote 24: _Ziemssen's Encyclopædia_, xii. 298, New York ed.]

The fact that nervous syphilis may occur many years after the cessation of all apparent evidences of the diathesis is of great practical importance, especially as the nervous system is more prone to be attacked when the secondaries have been very light than when the earlier manifestations have been severe. I have repeatedly seen nervous syphilis in persons whose secondaries have been so slight as to have been entirely overlooked or forgotten, and who honestly asserted that they never had had syphilis, although they acknowledged to gonorrhœa or to repeated exposure, and confessed that their asserted exemption was due to good fortune rather than to chastity.

{1002} The following citations prove that this experience is not peculiar. Dowse[25] says: “Often have I had patients totally ignorant of having at any time acquired or experienced the signs or symptoms of syphilis in its primary and secondary stages, yet the sequelæ have been made manifest in many ways, particularly in many of the obscure diseases of the nervous system.” Buzzard[26] reports a case of nervous syphilis where the patient was unconscious of the previous existence of a chancre or of any secondaries. Rinecker also calls attention[27] to the frequency of nervous syphilis in persons who afford no distinct history of secondary symptoms.

[Footnote 25: _The Brain and its Diseases_, London, 1879, vol. i. p. 7.]

[Footnote 26: _Syphilitic Nervous Affections_, London, 1874, p. 80.]

[Footnote 27: _Archiv f. Psychiatrie_, vii. p. 241.]

Although syphilis is prone to attack the nervous system many years after infection, it would be a fatal mistake to suppose that nervous disease may not rapidly follow the chancre. What is the minimum possible intermediate period we do not know, but it is certainly very brief, as is shown by the following cases of this so-called precocious nervous syphilis. Alfrik Ljunggrén of Stockholm reports[28] the case of H. R——, who had a rapidly-healed chancre in March, followed in May of the same year by a severe headache, mental confusion, and giddiness. Early in July H. R—— had an epileptic attack, but was finally cured by active antisyphilitic treatment. Although the history is not explicit, the nervous symptoms appear to have preceded the development of distinct secondaries other than rheumatic pains.

[Footnote 28: _Archiv f. Dermatol. u. Syphilis_, 1870, ii. p. 155.]

Davaine is said[29] to have seen paralysis of the portio dura “a month after the first symptoms of constitutional syphilis.” E. Leyden[30] found advanced specific degeneration of the cerebral arteries in a man who had contracted syphilis one year previously. R. W. Taylor details a case in which epilepsy occurred five months after the infection.[31] In the case of M. X——, reported by Ad. Schwarz,[32] headache came on the fortieth day after the appearance of the primary sore, and a hemiplegia upon the forty-sixth day. S. L——[33] had a paralytic stroke without prodromes six months after the chancre. A. P. L——[34] had an apoplectic attack seven months after the chancre; A. S——, one five months after her chancre. In a case which recently occurred in the practice of A. Sydney Roberts of this city the chancre appeared after a period of incubation of twenty-six days, and two months and eight days subsequent to this came the first fit; eight days after the first the second convulsion occurred, with a distinct aura, which preceded by some minutes the unconsciousness. An interesting observation in this connection is that of Ern. Gaucher[35] of a spinal syphilis occurring six months after the appearance of a chancre.

[Footnote 29: Buzzard, _Syphilitic Nervous Affections_, London, 1874.]

[Footnote 30: _Zeitschrift f. klin. Med._, Bd. v. 165.]

[Footnote 31: _Journ. Nervous and Mental Dis._, 1876, p. 38.]

[Footnote 32: _De l'Hémiplegia syphilitique Prêcoce_, Inaug. Diss., Paris, 1880.]

[Footnote 33: _Ibid._]

[Footnote 34: _Ibid._]

[Footnote 35: _Revue de Méd._, 1882, ii. 678.]

This citation of cases might be much extended, but is sufficient to show that nervous syphilis occurs not very rarely within six months after infection, and may be present in two months.

{1003} Gummatous Brain Syphilis.

CLINICAL HISTORY.—Brain syphilis of the type now under consideration may declare itself with great suddenness. An apoplectic attack, a convulsive paroxysm, a violent mania, or a paralytic stroke may be the first detected evidence of the disease. In most of these cases the coming storm ought to have been foreseen, and to a greater or less degree averted. The onset of cerebral syphilis is, however, generally more gradual, the symptoms coming on slowly and successively. Proper treatment, instituted at an early stage, is usually successful, so that a careful study of these prodromes is most important. They are generally such as denote cerebral disturbance, and, although they should excite suspicion, are not diagnostic, except as occurring in connection with a specific history or under suspicious circumstances.

Headache, slight failure of memory, unwonted slowness of speech, general lassitude, and especially lack of willingness to mental exertion, sleeplessness or excessive somnolence, attacks of momentary giddiness, vertiginous feelings when straining at stool, yelling or in any way disturbing the cerebral circulation, alteration of disposition,—any of these, and, a fortiori, several of them, occurring in a syphilitic subject, should be the immediate signal of alarm, and lead to the examination of the optic discs, for in some cases the eye-ground will be found altered even during the prodromic stage. Of course if choked disc be found the diagnosis becomes practically fixed, but the absence of choked disc is no proof that the patient is free from cerebral syphilis. In regard to the individual prodromic symptoms, my own experience does not lend especial importance to any one of them, although, perhaps, headache is the most common. There is one symptom which may occur during the prodromic stage of cerebral syphilis, but is more frequent at a later stage—a symptom which is not absolutely characteristic of the disease, but which, when it occurs in a person who is not hysterical, should give rise to the strongest suspicion. I refer to the occurrence of repeated, partial, passing palsies. A momentary weakness of one arm, a slight drawing of the face disappearing in a few hours, a temporary dragging of the toe, a partial aphasia which appears and disappears, a squint which to-morrow leaves no trace, may be due to a non-specific brain tumor, to miliary cerebral aneurisms, or to some other non-specific affection; but in the great majority of cases where such phenomena occur repeatedly the patient is suffering from syphilis or hysteria.

The first type or variety of the fully-formed syphilitic meningeal disease to which attention is here directed is that of an acute meningitis. I am much inclined to doubt whether an acute syphilitic meningitis can ever develop as a primary lesion—whether it must not always be preceded by a chronic meningitis or by the formation of a gummatous tumor; but it is very certain that acute meningitis may develop when there have been no apparent symptoms, and may therefore seem to be abrupt in its onset. Some years ago I saw, in consultation, a man who in the midst of apparent health was attacked by violent meningeal convulsions, with distinct evidences of acute meningitis. He was apparently saved from death by very heroic venesection, but after his return to consciousness developed very rapidly a partial specific hemiplegia, showing that a latent gumma had {1004} probably preceded the acute attack. On the other hand, an acute attack is liable at any time to supervene upon a chronic syphilitic meningitis. At the University Hospital dispensary I once diagnosed chronic cerebral syphilis in a patient who the next day was seized with violent delirium, with convulsions and typical evidences of acute meningitis, and died four or five days afterward. At the autopsy an acute meningitis was found to have been engrafted on a chronic specific lesion of a similar character. In the case reported by Gamel,[36] in which intense headache, fever, and delirium came on abruptly in an old syphilitic subject and ended in general palsy and death, the symptoms were found to depend upon an acute meningitis secondary to a large gumma.

[Footnote 36: _Tumeurs gommeuses du Cerveau_, Inaug. Diss., Montpellier, 1875.]

In this connection may well be cited the observation of Molinier[37] in which violent delirium, convulsions, and coma occurred suddenly. A very curious case is reported by D. A. Zambaco[38] in which attacks simulating acute meningitis occurring in a man with a cerebral gummatous tumor appear to have been malarial. In such a case the diagnosis of a malarial paroxysm could only be made out by the presence of the cold stage, the transient nature of the attack, its going off with a sweat, its periodical recurrence, and the therapeutic effect on it of quinine.

[Footnote 37: _Revue méd. de Toulouse_, xiv. 1880, 341.]

[Footnote 38: _Des Affections nerveuses-syphilitiques_, Paris, 1862, p. 485.]

In the cases of chronic brain syphilis which have come under my observation, most usually after a greater or less continuance of prodromes such as have been mentioned, epileptic attacks have occurred with a hemiplegia, or a monoplegia, which is almost invariably incomplete and usually progressive; very frequently diplopia is manifested before the epilepsy, and on careful examination is found to be due to weakness of some of the ocular muscles. Not rarely oculo-motor palsy is an early and pronounced symptom, and a marked paralytic squint is very common. Along with the development of these symptoms there is almost always distinct failure of the general health and progressive intellectual deterioration, as shown by loss of memory, failure of the power to fix the attention, mental bewilderment, and perhaps aphasia. If the case convalesce under treatment, the amelioration is gradual, the patient travelling slowly up the road he has come down. If the case end fatally, it is usually by a gradual sinking into complete paralysis, or the patient is carried off by an acute inflammatory exacerbation, or, as in two of my cases, amelioration may be rapidly occurring and a very violent epileptic fit produce a sudden fatal asphyxia. Death from brain-softening around the tumor is not infrequent, but a fatal apoplectic hemorrhage is rare.

The clinical varieties of cerebral meningeal syphilis are so polymorphic and kaleidoscopic that it is almost impossible to reduce them to order for descriptive purposes. Fournier separates them into the cephalic, congestive, epileptic, aphasic, mental, and paralytic, but scarcely facilitates description by so doing. Heubner makes the following types:

“1. Psychical disturbances, with epilepsy, incomplete paralysis (seldom of the cranial nerves), and a final comatose condition, usually of short duration.

“2. Genuine apoplectic attacks with succeeding hemiplegia, in connection with peculiar somnolent conditions, occurring in often-repeated {1005} episodes; frequently phenomena of unilateral irritation, and generally at the same time paralyses of the cerebral nerves.

“3. Course of the cerebral disease similar to paralytica dementia.”

In regard to these types, the latter seems to me clear and well defined, but contains those cases which I shall discuss under the head of Cortical Disease.

Meningeal syphilis as seen in this country does not conform rigidly with the other asserted types, although there is this much of agreement, that when the epilepsy is pronounced the basal cranial nerves are not usually paralyzed, the reason of this being that epilepsy is especially produced when the gummatous change is in the ventricles or on the upper cortex. In basal affections the epileptoid spells, if they occur at all, are usually of the form of petit mal; but this rule is general, not absolute. The apoplectic somnolent form of cerebral syphilis, for some reason, is rare in this city, and it seems necessary to add to those of Heubner's a fourth type to which a large proportion of our cases conform. This type I would characterize as follows:

4. Psychical disturbance without complete epileptic convulsions, associated with palsy of the basal nerves and often with partial hemiplegia.

The most satisfactory way of approaching this subject is, however, to study the important symptoms in severalty, rather than to attempt to group them into recognizable varieties of the disease; and this method I shall here adopt.

Headache is the most constant and usually the earliest symptom of meningeal syphilis; but it may be absent, especially when the lesion is located in the reflexions of the meninges which dip into the ventricles, or when the basal gumma is small and not surrounded with much inflammation. The length of time it may continue without the development of other distinct symptoms is remarkable. In one case[39] at the University Dispensary the patient affirmed that he had had it for four years before other causes of complaint appeared. It sometimes disappears when other manifestations develop. It varies almost indefinitely in its type, but is, except in very rare cases, at least so far paroxysmal as to be subject to pronounced exacerbations. In most instances it is entirely paroxysmal; and a curious circumstance is, that very often these paroxysms may occur only at long intervals: such distant paroxysms are usually very severe, and are often accompanied by dizziness, sick stomach, partial unconsciousness, or even by more marked congestive symptoms. The pain may seem to fill the whole cranium, may be located in a cerebral region, or fixed in a very limited spot. Heubner asserts that when this headache can be localized it is generally made distinctly worse by pressure at certain points, but my own experience is hardly in accord with this. Any such soreness plainly cannot directly depend upon the cerebral lesion, but must be a reflex phenomenon or due to a neuritis. According to my own experience, localized soreness indicates an affection of the bone or of its periosteum. In many cases, especially when the headache is persistent, there are distinct nocturnal exacerbations.

[Footnote 39: _Book Y._, p. 88, 1879.]

It will be seen that there is nothing absolutely characteristic in the headache of cerebral syphilis; but excessive persistency, apparent causelessness, and a tendency to nocturnal exacerbation should in any {1006} cephalalgia excite suspicion of a specific origin—a suspicion which is always to be increased by the occurrence of slight spells of giddiness or by delirious mental wandering accompanying the paroxysms of pain. When an acute inflammatory attack supervenes upon a specific meningeal disease it is usually ushered in by a headache of intolerable severity.

When the headache in any case is habitually very constant and severe, the disease is probably in the dura mater or periosteum; and this probability is much increased if the pain be local and augmented by firm, hard pressure upon the skull over the seat of the pain.

Disorders of Sleep.—There are two antagonistic disorders of sleep, either of which may occur in cerebral syphilis, but which have only been present in a small proportion of the cases that I have seen. Insomnia is more apt to be troublesome in the prodromic than in the later stages, and is only of significance when combined with other more characteristic symptoms. A peculiar somnolence is of much more determinate import. It is not pathognomonic of cerebral syphilis, yet of all the single phenomena of this disease it is the most characteristic. Its absence is of no import in the theory of an individual case.

As I have seen it, it occurs in two forms: In the one variety the patient sits all day long or lies in bed in a state of semi-stupor, indifferent to everything, but capable of being aroused, answering questions slowly, imperfectly, and without complaint, but in an instant dropping off again into his quietude. In the other variety the sufferer may still be able to work, but often falls asleep while at his tasks, and especially toward evening has an irresistible desire to slumber, which leads him to pass, it may be, half of his time in sleep. This state of partial sleep may precede that of the more continuous stupor, or may pass off when an attack of hemiplegia seems to divert the symptoms. The mental phenomena in the more severe cases of somnolency are peculiar. The patient can be aroused—indeed in many instances he exists in a state of torpor rather than of sleep; when stirred up he thinks with extreme slowness, and may appear to have a form of aphasia; yet at intervals he may be endowed with a peculiar automatic activity, especially at night. Getting out of bed; wandering aimlessly and seemingly without knowledge of where he is, and unable to find his own bed; passing his excretions in a corner of the room or in other similar place, not because he is unable to control his bladder and bowels, but because he believes that he is in a proper place for such act,—he seems a restless nocturnal automaton rather than a man. In some cases the somnolent patient lies in a perpetual stupor.

An important fact in connection with the somnolence is that it may develop suddenly without marked premonition. Thus in a case reported by J. A. Ormerod[40] a man who had been in good health, save only for headache, awoke one morning in a semi-delirious condition, and for three days slept steadily, only arousing for meals; after this there was impairment of memory and mental faculties, but no more marked symptoms.

[Footnote 40: _Brain_, vol. v. 260.]

Apathy and indifference are the characteristics of the somnolent state, yet the patient will sometimes show excessive irritability when aroused, and will at other periods complain bitterly of pain in his head, or will groan as though suffering severely in the midst of his stupor—at a time, {1007} too, when he is not able to recognize the seat of the pain. I have seen a man with a vacant, apathetic face, almost complete aphasia, persistent heaviness and stupor, arouse himself when the stir in the ward told him that the attending physician was present, and come forward in a dazed, highly pathetic manner, by signs and broken utterances begging for something to relieve his head. Heubner speaks of cases in which the irritability was such that the patient fought vigorously when aroused; this I have not seen.

This somnolent condition may last many weeks. T. Buzzard[41] details the case of a man who after a specific hemiplegia lay silent and somnolent for a month, and yet finally recovered so completely as to win a rowing-match on the Thames. I have seen a fair degree of recovery after a somnolence of four months' duration.

[Footnote 41: _Clinical Lectures on Dis. Nerv. Syst._, London, 1882.]

In its excessive development syphilitic stupor puts on the symptoms of advanced brain-softening, to which it is indeed often due. Of the two cases with fatal result of which I have notes, one at the autopsy was found to have symmetrical purulent breaking down of the anterior cerebral lobes; the other, softening of the right frontal and temporal lobes, due to the pressure of a gummatous tumor, and ending in a fatal apoplexy.

This close connection with cerebral softening explains the clinical fact that apoplectic hemorrhage is very apt to end the life in these cases of somnolent syphilis. But a prolonged deep stupor in persons suffering from cerebral syphilis does not prove the existence of extensive brain-softening, and is not incompatible with subsequent complete recovery. As an element of prognosis it is of serious but not of fatal import.

Paralysis.—When it is remembered that a syphilitic exudation may appear at almost any position in the brain, that spots of encephalic softening are a not rare result of the infection, that syphilitic disease is a common cause of cerebral hemorrhage, it is plain that a specific palsy may be of any conceivable variety, and affect either the sensory, motor, or intellectual sphere. The mode of onset is as various as the character of the palsy. The attack may be instantaneous, sudden, or gradual. The gradual development of the syphilitic gumma would lead us, a priori, to expect an equally gradual development of the palsy; but experience shows that in a large proportion of the cases the paralysis appears suddenly, with or without the occurrence of an apoplectic or epileptic fit. Under these circumstances it will be usually noted that the resulting palsy is incomplete; in rare instances it may be at its worst when the patient awakes from the apoplectic seizure, but usually it progressively increases for a few hours, and then becomes stationary. These sudden partial palsies probably result from an intense congestion around the seat of disease or from stoppage of the circulation in the same locality; whatever their mechanism may be, it is important to distinguish them from palsies which are due to hemorrhage. I believe this can usually be done by noting the degree of paralysis.

A suddenly-developed, complete hemiplegia or other paralysis may be considered as in all probability either hemorrhagic or produced by a thrombus so large that the results will be disorganization of the brain-substance, and a future no more hopeful than that of a clot. On the {1008} other hand, an incomplete palsy may be rationally believed to be due to pressure or other removable cause; and this belief is much strengthened by a gradual development. The bearing of these facts upon prognosis it is scarcely necessary to point out.

Although the gummata may develop at almost any point, they especially affect the base of the brain, and are prone to involve the nerves which issue from it. Morbid exudations, not tubercular or syphilitic, are rare in this region. Hence a rapidly but not abruptly appearing strabismus, ptosis, dilated pupil, or any paralytic eye symptom in the adult is usually of syphilitic nature. Syphilitic facial palsy is not so frequent, whilst paralysis of the nerve from rheumatic and other inflammation within its bony canal is very common. Paralysis of the facial nerve may therefore be specific, but existing alone is of no diagnostic value. Since syphilitic palsies about the head are in most instances due to pressure upon the nerve-trunks, the electrical reactions of degeneration are present in the affected muscles.

There is one peculiarity about specific palsies which has already been alluded to as frequently present—namely, a temporary, transient, fugitive, varying character and seat. Thus an arm may be weak to-day, strong to-morrow, and the next day feeble again, or the recovered arm may retain its power and a leg fail in its stead. These transient palsies are much more apt to involve large than small brain territories. The explanation of their largeness, fugitiveness, and incompleteness is that they are not directly due to clots or other structural changes, but to congestions of the brain-tissues in the neighborhood of gummatous exudations. Squint due to direct pressure on a nerve will remain when the accompanying monoplegia due to congestion disappears.

Motor palsies are more frequent than sensory affections in syphilis, but hemianæsthesia, localized anæsthetic tracts, indeed any form of sensory paralysis, may occur. Numbness, formications, all varieties of paræsthesia, are frequently felt in the face, body, or extremities. Violent peripheral neuralgic pains are rare, and generally when present denote neuritis. Huguenin, however, reports[42] a severe trigeminal anæsthesia dolorosa, which was found, after death from intercurrent disease, to have depended upon a small gumma pressing upon the Gasserian ganglion. A somewhat similar case has also been reported by Allen McLane Hamilton.[43]

[Footnote 42: _Schwiez. Corr. Blät._, 1875.]

[Footnote 43: _Alienist and Neurologist_, iv. 58.]

The special senses are liable to suffer from the invasion of their territories by cerebral syphilis, and the resulting palsies follow courses and have clinical histories parallel to those of the motor sphere. The onset may be sudden or gradual, the result temporary or permanent. Charles Mauriac[44] reports a case in which the patient was frequently seized with sudden attacks of severe frontal pain and complete blindness lasting from a quarter to half an hour; at other times the same patient had spells of aphasia lasting only for one or two minutes. I have seen two cases of nearly complete deafness developing in a few hours in cerebral syphilis, and disappearing abruptly after some days. Like other syphilitic palsies, therefore, paralyses of special senses may come on suddenly or gradually, and may occur paroxysmally.

[Footnote 44: _Loc. cit._, p. 31.]

Among the palsies of cerebral syphilis must be ranked aphasia. An {1009} examination of recorded cases shows that syphilitic aphasia is subject to vagaries and laws similar to those connected with other specific cerebral palsies. It is usually a symptom of advanced disease, but may certainly develop as one of the first evidences of cerebral syphilis. Coming on after an apoplectic or epileptic fit, it may be complete or incomplete: owing to the smallness of the centre involved and the ease with which its function is held in abeyance, a total loss of word-thought is not so decisive as to the existence of cerebral hemorrhage as is a total motor palsy. Like hemiplegia or monoplegia, specific aphasia is sometimes transitory and paroxysmal. Buzzard[45] records several such cases. Mauriac[46] details a very curious case in which a patient, after long suffering from headache, was seized by sudden loss of power in the right hand and fingers, lasting about ten minutes only, but recurring many times a day. After this had continued some time the paroxysms became more completely paralytic, and were accompanyed by loss of the power of finding words, the height of the crises in the palsy and aphasia being simultaneously reached. For a whole month these attacks occurred five or six times a day, without other symptoms except headache, and then the patient became persistently paralytic and aphasic, but finally recovered. To describe the different forms of specific aphasia and their mechanism of production would be to enter upon a discussion of aphasia itself—a discussion out of place here. Suffice it to say that every conceivable form of the disorder may be induced by syphilis.

[Footnote 45: _Loc. cit._, p. 81.]

[Footnote 46: _Aphasie et Hemiplégia droite Syphilit._, Paris, 1877.]

Owing to the centres of speech being situated in the cortical portion of the brain, aphasia in cerebral syphilis is very frequently associated with epilepsy. Of course right-sided palsy and aphasia are united in syphilitic as in other disorders. If, however, the statistics given by Tanowsky[47] be reliable, syphilitic aphasia is associated with left-sided hemiplegia in a most extraordinarily large proportion. Thus in 53 cases collected by Tanowsky, 18 times was there right-sided hemiplegia, and 14 times left-sided hemiplegia, the other cases being not at all hemiplegic. Judging from the autopsy on a case reported in Mauriac's brochure, this concurrence of left-sided paralysis and aphasia depends partly upon the great frequency of multiple brain lesions in syphilis, and partly upon the habitual involvement of large territories of the gray matter secondarily to diseased membrane. An important practical deduction is that the conjoint existence of left hemiplegia and aphasia is almost diagnostic of cerebral syphilis.

[Footnote 47: _L'Aphasie syphilitique_.]

Probably amongst the palsies may be considered the disturbances of the renal functions, which are only rarely met with in cerebral syphilis, and which are probably usually dependent upon the specific exudation pressing upon the vaso-motor centres in the medulla. Fournier speaks of having notes of six cases in which polyuria with its accompaniment, polydipsia, was present, and details a case in which the specific growth was found in the floor of the fourth ventricle. Cases have been reported of true saccharine diabetes due to cerebral syphilis,[48] and I can add to these an observation of my own. The symptoms, which occurred in a {1010} man of middle age, with a distinct specific history, were headache, nearly complete hemiplegia, and mental failure, associated with the passage of comparatively small quantities of a urine so highly saccharine as to be really a syrup. Under the influence of the iodide of potassium the sugar in a few weeks disappeared from the urine.

[Footnote 48: Consult Servantié, _Des Rapports du Diabète et de la Syphilis_, Paris, Thèse, 1876; also, case reported by L. Putzel, _New York Med. Record_, xxv. 450.]

Epilepsy.—Epileptic attacks are a very common symptom of meningeal syphilis, and are of great diagnostic value. The occurrence in an adult of an epileptic attack or of an apoplectic fit, or of a hemiplegia after a history of intense and protracted headache, should always excite grave suspicion.

Before I had read Fournier's work on _Nervous Syphilis_ I taught that an epilepsy appearing after thirty years of age was very rarely, if ever, essential epilepsy, and unless alcoholism, uræmic poison, or other adequate cause could be found was in nine cases out of ten specific; and I therefore quote with satisfaction Fournier's words: “L'épilepsie vraie, ne fait jamais son premier dêbut à l'âge adulte, à l'âge mûr. Si un homme adulte, au dessus de 30, 35, à 40 ans, vient, à être pris pour la première fois d'une crise épileptique, et cela dans la cours d'une bonne santé apparente, il y a, je vous le répète, hui ou neuf chances sur dix pour que cette épilepsie soit d'origine syphilitique.”

Syphilitic epilepsy may occur either in the form of petit mal or of haut mal, and in either case may take on the exact characters and sequence of phenomena which belong to the so-called idiopathic or essential epilepsy. The momentary loss of consciousness of petit mal will usually, however, be found to be associated with attacks in which, although voluntary power is suspended, memory recalls what has happened during the paroxysm—attacks, therefore, which simulate those of hysteria, and which may lead to an error of diagnosis.

Even in the fully-developed type of the convulsions the aura is only rarely present. Its absence is not, however, of diagnostic value, because it is frequently not present in essential epilepsy, and it may be pronounced in the specific disease. It is said that when in an individual case the aura has once appeared the same type or form of approach of the convulsion is thereafter rigidly adhered to. The aura is sometimes bizarre: a severe pain in the foot, a localized cramp, a peculiar sensation, indescribable and unreal in its feeling, may be the first warning of the attack. An aura may affect a special sense. Thus, I have at present a patient whose attacks begin with blindness.

In many, perhaps most, cases of specific convulsions, instead of a paroxysm of essential epilepsy being closely simulated, the movements are in the onset, or more rarely throughout the paroxysm, unilateral; indeed, they may be confined to one extremity. This restriction of movement has been held to be almost characteristic of syphilitic epilepsy, but it is not so. Whatever diagnostic significance such restriction of the convulsion has is simply to indicate that the fit is due to a cortical organic lesion of some kind. Tumors, scleroses, and other organic lesions of the brain-cortex are as prone to cause unilateral or monoplegic epilepsy when they are not specific as when they are due to syphilis.

Sometimes an epilepsy dependent upon a specific lesion implicating the brain-cortex may be replaced by a spasm which is more or less local and is not attended with any loss of consciousness. Thus, in a case now {1011} convalescent in the University Hospital, a man aged about thirty-five offered a history of repeated epileptic convulsions, but at the time of his entrance into the hospital, instead of epileptic attacks, there was a painless tic. The spasms, which were clonic and occurred very many times a day, sometimes every five minutes, were very violent, and mostly confined to the left facial nerve distribution. The trigeminus was never affected, but in the severer paroxysms the left hypoglossal and spinal accessory nerves were profoundly implicated in all of their branches. Once, fatal asphyxia from recurrent laryngeal spasm of the glottis was apparently averted only by the free inhalation of the nitrite of amyl. The sole other symptom was headache, but the specific history was clear and the effect of antisyphilitic remedies rapid and pronounced.

It is very plain that such attacks as those just detailed are closely allied to epilepsy; indeed, there are cases of cerebral syphilis in which widespread general spasms occur similar to those of a Jacksonian epilepsy, except that consciousness is not lost, because the nervous discharge does not overwhelm the centres which are connected with consciousness.[49] On the other hand, these epileptoid spasmodic cases link themselves to those in which the local brain affection manifests itself in contractions or persistent irregular clonic spasms. Contractures may exist and may simulate those of descending degeneration,[50] but in my own experience are very rare.[51]

[Footnote 49: Case, _Canada Med. and Surg. Journ._, xi. 487.]

[Footnote 50: Case, _Centralbl. Nerv. Heilk._, 1883, p. 1.]

[Footnote 51: A case of syphilitic athetosis may be found in _Lancet_, 1883, ii. 989.]

The clonic spasms of cerebral syphilis may assume a distinctly choreic type, or may in their severity simulate those of hysteria, throwing the body about violently.[52] It is, to my mind, misleading, and therefore improper, to call such cases syphilitic chorea, as there is no reason for believing that they have a direct relation with ordinary chorea. They are the expression of an organic irritation of the brain-cortex, and are sometimes followed by paralysis of the affected member; in other words, the disease, progressing inward from the brain-membrane, first irritates, and then so invades a cortical centre as to destroy its functional power.[53]

[Footnote 52: See Allison, _Amer. Med. Journ._, 1877, 74.]

[Footnote 53: Case, _Chicago Med. Journ. and Exam._, xlvi. 21.]

Psychical Symptoms.—As already stated, apathy, somnolence, loss of memory, and general mental failure are the most frequent and characteristic mental symptoms of meningeal syphilis; but, as will be shown in the next chapter, syphilis is able to produce almost any form of insanity, and therefore mania, melancholia, erotic mania, delirium of grandeur, etc. etc. may develop along with the ordinary manifestation of cerebral syphilis, or may come on during an attack which has hitherto produced only the usual symptoms. Without attempting any exhaustive citation of cases, the following may be alluded to.

A. Erlenmeyer reports[54] a case in which an attack of violent headache and vomiting was followed by paralysis of the right arm and paresis of the left leg, with some mental depression; a little later the patient suddenly became very cheerful, and shortly afterward manifested very distinctly delirium of grandeur with failure of memory. Batty Tuke reports[55] a case in which, with aphasia, muscular wasting, strabismus, {1012} and various palsies, there were delusions and hallucinations. In the same journal[56] S. D. Williams reports a case in which there were paroxysmal violent attacks of frontal headache. The woman was very dirty in her habits, only ate when fed, and existed in a state of hypochondriacal melancholy. Leiderdorf details a case with headache, partial hemiplegia, great psychical disturbance, irritability, change of character, marked delirium of grandeur, epileptic attacks, and finally dementia, eventually cured by iodide of potassium.[57] Several cases illustrating different forms of insanity are reported by N. Manssurow.[58]

[Footnote 54: _Die luëtischen Psychosen_.]

[Footnote 55: _Journ. Ment. Sci._, Jan., 1874, p. 560.]

[Footnote 56: April, 1869.]

[Footnote 57: _Medicin Jahrbucher_, xx. 1864, p. 114.]

[Footnote 58: _Die Tertiäre Syphilis_, Wien, 1877.]

That the attacks of syphilitic insanity, like the palsies of syphilis, may at times be temporary and fugitive, is shown by a curious case reported by H. Hayes Newington,[59] in which, along with headache, failure of memory, and ptosis in a syphilitic person, there was a brief paroxysm of noisy insanity.

[Footnote 59: _Journ. Ment. Sci._, London, xix. 555.]

DIAGNOSIS.—In a diagnosis of cerebral syphilis a correct history of the antecedents of the patients is of vital importance. Since very few of the first manifestations of the disorder are absolutely characteristic, whilst almost any conceivable cerebral symptoms may arise from syphilitic disease, treatment should be at once instituted on the appearance of any disturbance of the cerebral functions in an infected person.

Very frequently the history of the case is defective, and not rarely actually misleading. Patients often appear to have no suspicion of the nature of their complaint, and will deny the possibility of syphilis, although they confess to habitual unchastity. My own inquiries have been so often misleading in their results that I attach but little weight to the statements of the patient, and in private practice avoid asking questions which might recall unpleasant memories, depending upon the symptoms themselves for the diagnosis.