Читать книгу Neurosyphilis - Elmer Ernest Southard - Страница 6

Table of Contents

PARADIGM to show possible abundance and variety of symptoms and lesions in DIFFUSE NEUROSYPHILIS (“cerebrospinal syphilis”). Autopsy.

Case 1. Mrs. Alice Morton^[1] was in the hands of at least five well-known specialists in different branches of medicine and surgery during the nineteen years of her disease. It appears that she acquired syphilis upon marriage at the age of 23 to a man who later became tabetic and acknowledged syphilitic infection previous to marriage. Mrs. Morton remained without children and there were no miscarriages.

At the age of 27, she developed iritis, paresis of the left eye muscles, and ulceration of the throat, with destruction of the uvula. The syphilitic nature of her disease was at once recognized and the classical treatment was given, although, through numerous shifts in consultants, this treatment was never pushed to the limit. At 28 Mrs. M. began to suffer from severe headaches resembling migraine and accompanied by attacks of paræsthesia; at 35, came severe pains in the back and difficulty in walking.

At 36, the migraine attacks began to be accompanied by blurring of vision and dizziness. The difficulty in walking became extreme, affecting particularly the right foot. The legs became spastic, there were pains and hyperæsthesia of the chest, and severe cramps of the legs. Antisyphilitic treatment at this time yielded marked improvement.

During her thirty-sixth year, Mrs. M. sustained curious transient losses of vision and of hearing. She was also irritable, and at this time developed her first pronounced mental symptoms, namely, delusions concerning her relatives. There were also a few seizures of an epileptiform nature.

At 38 there was a spell of total deafness, followed by improvement. The eye muscles were also subject to a variable involvement with intervening spells of improvement. The knee-jerks were lost, but after a time returned in less pronounced form. Shortly, an absolute paralysis and extensive decubitus developed, and death occurred at 39.

The autopsy is briefly summarized below, but it is important in the understanding of Mrs. M.’s case (particularly some of the sensory symptoms and the transiency of certain symptoms) to consider the pre-infective history. Although there seems to be no doubt that the patient acquired syphilis at about 23 years of age from a syphilitic husband, who himself later became tabetic, yet it is of note that the patient was the only child of parents, both of whom also suffered from mental disease. Mrs. M.’s father died of what was called softening of the brain (one should avoid terming all old cases of so-called “softening of the brain” syphilitic, since the older diagnosticians did not always distinguish between non-syphilitic arteriosclerotic effects and syphilitic disease). Mrs. M.’s mother also died insane (confusion and emotional depression). It is clear, then, that we do not need to suppose that every symptom shown by Mrs. M. is directly due to destructive or irritative lesions immediately due to the spirocheta pallida. The case is, in fact, an excellent lesson as to the association of structural and functional effects in neuropathological cases.

Mrs. M. as a child had shown talent, but was somewhat nervous and eccentric. At one time, she had an attack of hysterical dysphasia; at another time, an attack of hysterical dyspnea; during another period, an apparent obsession (kicking the mopboard at regular intervals). Moreover, she had for years suffered from migraines of a severe and unusual type. Both the hysterical tendency and the migrainous tendency became mingled with the results of the neurosyphilis in later stages of the disease in such wise that it was hard to tell exactly where the structural phenomena left off and the functional phenomena began.

For example, at the age of 32, nine years after infection and four years after the earliest nerve symptoms traceable to syphilis, and at about the time of the onset of spinal cord symptoms, an attack was described as follows:

The patient had a very severe attack of migraine (?) yesterday, preceded and accompanied by paraphasia, so severe that for three hours she was unable to make herself understood, and indeed felt “as if her ideas were getting away from her.” This attack was ushered in by a numbness of the forefinger and thumb of the right hand, which lasted for about three hours, though the earlier attacks had lasted for only about ten minutes. During this period the hand felt as if it had been frozen and the loss of muscular power was so great that she was unable to hold objects in the hand. In some of the attacks this paræsthesia has affected the entire left half of the body, and occasionally the right half. Sometimes the seizures come on with great suddenness, so that once, when she was attacked while in the middle of the street, she had considerable difficulty in reaching the sidewalk. After the worst part of the attack is over a certain amount of paraphasia may persist for some days, together with awkwardness in the use of the right hand and numbness. She has had a great deal of nausea and vomiting, without reference to the taking of food.^[2]

Bearing in mind the mingling of structural with functional symptoms in this case, let us consider the autopsy findings.

ANATOMICAL

FORMS OF NEUROSYPHILIS

AUTONOMIC (SYMPATHETIC) NEUROSYPHILIS?

PERIPHERAL NEUROSYPHILIS

CENTRAL NEUROSYPHILIS

MENINGEAL

VASCULAR

PARENCHYMATOUS

MENINGOVASCULAR

VASCULOPARENCHYMATOUS

DIFFUSE ( = MENINGOVASCULOPARENCHYMATOUS)

GUMMA

Chart 1

CLINICAL FORMS OF NEUROSYPHILIS

HEAD AND FEARNSIDES, 1914

SYPHILIS MENINGOVASCULARIS

CEREBRAL FORMS

HEMIPLEGIA

AFFECTION OF THE CRANIAL NERVES

MUSCULAR ATROPHY

LATERAL AND COMBINED DEGENERATIONS

EPILEPSY

SYPHILIS CENTRALIS

DEMENTIA PARALYTICA

TABES DORSALIS

MUSCULAR ATROPHY

OPTIC ATROPHY

GASTRIC CRISES

EPILEPTIC MANIFESTATIONS

Chart 2

Peripheral neurosyphilis: The lesions of the cranial nerves were characteristically asymmetrical. Whereas the left third nerve looked entirely normal, the right third nerve had its diameter reduced two-thirds. On the other hand, the fourth nerves were equal and apparently normal. The sensory portion of the left fifth nerve was normal; the right fifth nerve was normal. The right sixth nerve agreed with the right third nerve in being atrophic, and was in fact reduced to a mere thread without contained nerve fibres at a point 2 mm. from its superficial origin. Although the right third nerve was atrophic, it was the left seventh and eighth nerves which had become atrophic; the process had spared the right seventh and eighth nerves. The remainder of the cranial nerves were grossly normal, except that the optic nerves had an outer zone of a translucent nature. So far, no spirochetes have been demonstrated in any portion of the nervous system of this case, but such asymmetrical and focal cranial nerve lesions are perhaps due to local spirochetal infection, punctuating (as it were) the diffuse process.

How much of the transient blindness, deafness, and ocular paralysis can be explained on the anatomical findings in these nerves? Possibly a portion of the phenomena can be so explained. Thus, the mechanical conditions of pressure inside and outside these nerves, both in their peripheral course and in their passage through the membranes, can be readily understood to differ during the acute and subacute inflammation, during the process of repair in the pial tissues, and during the process of overgrowth of neuroglia tissue about the superficial origins of the nerves. Of course, the majority of lesions of these nerves were entirely extinct at the time of the autopsy, and their history could be surmised only from the appearances in the left eighth nerve. Here occurred a sharply marked focal area of gliosis with apparently total destruction of nerve fibres and related with a lymphocytosis of the investing membrane (one of the few areas of lymphocytosis found anywhere in this case).

If it were not for the pre-infective history, the hysterical dysphasia and dypsnea, the youthful obsessions, the migrainous tendency, and the psychopathic inheritance, we might be tempted to try to explain the transient blindness, the deafness, and ocular palsies on the basis of mechanical and toxic variations in the conditions of the peripheral cranial nerves. The existence of a trace of lymphocytosis in the left eighth nerve leads to the hypothesis that treatment might still be effective in this particular region (see below in discussion of spinal symptoms).

Spinal neurosyphilis: Not only the spinal cord but also the posterior and anterior nerve roots exhibited severe lesions. These lesions were both meningeal and parenchymatous. The meningeal process differed in its intensity in different parts of the spinal cord, being severest in the thoracic region. At one point in this region, the dura mater was so firmly attached to the pia mater that the line of demarcation between the two membranes was hard to make out. In fact, it seems clear that there could have been no free intercommunication between the spinal fluid above these adhesions of dura to pia mater and the spinal fluid below the adhesions. Accordingly, it seems that lumbar puncture, had it been practised in this case, would have failed to show features representative of the whole cerebrospinal fluid system. Moreover, since at no point in this region of adhesions or in the pia mater of the spinal cord below this point, were found any lymphocytes, it seems clear that the ordinary lumbar puncture would have failed to reveal a pleocytosis. Whether this fluid would have yielded a positive globulin and excess albumin test, it is now impossible to say; but it appears that the process in the lower part of the spinal cord was to all intents and purposes extinct.

However, there was one region of more severe inflammatory involvement. The spinal cord in the cervical region showed a lymphocyte infiltration of its vessels amounting to a mild myelitis (meaning, thereby, an inflammatory process of the spinal cord remote from the pia mater). Moreover, in this region, there was, besides the perivascular infiltration of the substance, also an infiltration of the overlying membranes themselves, especially in and near the posterior root zones.

The lessons of this finding are several: The inflammatory process in this case does not appear to have been entirely extinct! Can we not suppose that treatment might still have benefited this local inflammation (perivascular infiltration of the cervical spinal cord substance and overlying lymphocytic meningitis)? Can we not also picture the gradual ascent of the inflammatory lesions from lower segments to higher segments and possibly conceive of the gradual elevation of the zone of hyperæsthesia manifested in this case as following the gradual displacement upward of the lymphocytic process? Are there spirochetes in this tissue? So far none have been discovered, possibly through inaccuracies of available technique. To the neuropathologist, however, the lesion looks like a local reaction to organisms.

In addition to the spinal meningitis, chronic and acute, as above described, there were extensive parenchymatous spinal lesions.

In the first place, the meningitis had affected practically all the posterior roots so that the explanation of the posterior column sclerosis of this case is clear. The meningitis had apparently been so marked, also, that all the fibres anywhere near the periphery of the spinal cord had been likewise destroyed. The posterior columns and the posterior root zones were markedly sclerotic; or as we say (having reference to the overgrowth of neuroglia tissue) gliotic. But there was as much sclerosis (gliosis) of the lateral columns (particularly in the posterior two-thirds) as there was in the posterior columns and root zones. In fact, the entire posterior half or two-thirds of the spinal cord markedly outstripped the anterior portions of the cord in the severity of the gliosis (sclerosis) shown.

But although we can explain the posterior column sclerosis, the sclerosis of the posterior root zones and the marginal sclerosis (Randsklerose) round the entire periphery of the cord, on the basis of long-standing effects of old meningitis, we cannot thus explain another finding, namely, the destruction of the fibres in the lateral columns. This, in fact, is explained through lesions (mentioned below) that affected the encephalon. The net result of all these lesions of the spinal cord was to leave only the gray matter and a small amount of surrounding fibres (belonging to short tracts uniting nearby segments) intact. Briefly stated, every long tract in the spinal cord appeared upon examination to be extensively degenerated. The genesis of this parenchymatous loss was, however, double, being in part due to a local meningeal process (sometimes known as “perimeningitis”) and in part due to a cutting off of the pyramidal tract fibres on both sides by lesions higher up in the nervous system.

Case I. Spinal Cord (Three Levels) Showing:

A. Marginal sclerosis—effect of old meningitis now extinct.

B. Posterior column sclerosis—effect of meningitis about posterior roots also now extinct.

C. Bilateral pyramidal tract sclerosis—effect of cerebral thrombotic lesions.

Note distortion of tissues in B and C, partly artificial (tissues in places diffluent).

ANATOMICAL FORMULAE
MENINGOVASCULOPARENCHYMATOUS INVOLVEMENT
M, V, P, or Combinations Applied to the Classification of Head and Fearnsides
I.	SYPHILIS MENINGOVASCULARIS
	CEREBRAL FORMS	M or V or MV[3]
	HEMIPLEGIA	V
	AFFECTION OF THE CRANIAL NERVES	M
	MUSCULAR ATROPHY	M
	LATERAL AND COMBINED DEGENERATIONS	M
	EPILEPSY	M or V
II.	SYPHILIS CENTRALIS
	DEMENTIA PARALYTICA	MVP or VP
	TABES DORSALIS	MP
	MUSCULAR ATROPHY	P
	OPTIC ATROPHY	P
	GASTRIC CRISES	(M? or) P?
EPILEPTIC MANIFESTATIONS	P?
Chart 3

Can we offer any explanation of the partial return of knee-jerks after their temporary total loss at a certain period of the disease? We may assume that the knee-jerks were functionally lost about a year before the death of the patient through the partial or even almost complete destruction of the entering posterior root fibres at that level of the spinal cord which is directly related with the knee-jerk. The later partial return of the knee-jerks apparently requires us to suppose the maintenance of some fibres and collaterals by which a functional connection can be effected between the fibres of the posterior roots and the anterior horn cells which innervate the quadriceps femoris. Let us now suppose that pari passu with the actual return of the knee-jerks, the destructive processes that are affecting both pyramidal tracts high up in the nervous system are now advancing. It is clear that, whatever inhibitory influence these pyramidal tracts have been exerting up to this time upon the knee-jerk reflex arc, that influence is now to be decidedly reduced in amount and possibly absolutely lost. Upon the loss of such inhibitory influences exerted from above, the few persisting connections of the posterior roots and anterior horn cells are now permitted to resume their functions.

Encephalic neurosyphilis: The lesions mentioned above as causing destruction of the pyramidal tracts of the spinal cord were symmetrically destructive and atrophic lesions of the gray matter of both corpora striata with atrophy of the anterior segments of the internal capsules. There was a degenerative process of the corpus callosum especially affecting the forceps minor of the tapetum. The ventricles were largely dilated, indicating a considerable destruction and atrophy of the white matter in general.

After the above discussion of the possible effects of pyramidal tract lesion in this case, it is unnecessary further to discuss the paraplegia produced by the cystic lesions of the corpora striata. The theorist might inquire how these cystic lesions are produced: whether by vascular blocking or by toxic effects of the accumulations of spirochetes. Evidence is lacking which would completely sustain either hypothesis. Still, we do know that lesions almost identical in appearance may be produced by the necrosis consequent to the plugging of nutritive vessels in an organ like the brain supplied with end arteries. Therefore, it is probable that most pathologists would believe these lesions of the corpora striata to be produced by vascular plugging of the nature of thrombosis.

It is worth while to note that there was a suggestion of foci of encephalitis made out upon the gross examination. The cortex in general showed strikingly few lesions. However, the convolutions did show in places numerous ill-defined areas of hyperemia and slight swelling. These areas were of irregular distribution and only a few mm. or cm. in diameter. No gross vascular lesions were demonstrable in connection with these focal areas. Microscopically, however, venous plugs of polymorphonuclear leucocytes were found, and the local hyperemias were found to be largely due to venous congestion. However, very few polymorphonuclear leucocytes were found outside the blood vessels.

The white matter of numerous convolutions showed microscopically certain pale spots suggestive of an early atrophic process. Very possibly these represent a general tendency in the cerebrum to the same process of parenchymatous loss which had proceeded to such a marked degree in the spinal cord.

There was a single large so-called cyst of softening in the cerebellum (1.5 mm. across by 0.5–7.5 cm. in depth).

How far can we explain the symptoms of this case on the basis of these encephalic lesions? We can offer no correlation with the cerebellar lesion; and possibly this lack of correlation is to be expected on account of its failure to affect the vermis. As to the cystic lesions of the corpora striata, their effect in producing paraplegia at the close of life is obvious, and their possible relation to the partial return of knee-jerks has been discussed. Literally amazing was the comparative integrity of the cortical gray matter of this case when the spinal cord and the interior structures of the encephalon had been subjected to such severe and numerous lesions. The only mental symptoms noted in the case were sundry delusions directed against the patient’s relatives and a certain optimism which led the patient to cling as if with an obsession to the belief that in the end she would get well.

VARIOUS FORMS OF NEUROSYPHILIS COLLECTED FROM SEVERAL SOURCES
MENINGEAL NEUROSYPHILIS (M)
GUMMA OF DURA MATER	M
GUMMATOUS MENINGITIS (Pial)	M
SYPHILITIC MENINGITIS (Pial)	M
SYPHILITIC CRANIAL NERVE PALSIES (Primarily Pial)	M
SYPHILITIC BULBAR PALSY	M
SYPHILITIC ROOT NEURITIS	M
SYPHILITIC TRANSVERSE MYELITIS	M
SYPHILITIC NEURITIS (Some Cases by Extension)	M
SYPHILITIC EPILEPSY (Some Cases)	M
SYPHILITIC MUSCULAR ATROPHY (Some Cases)	M
VASCULAR NEUROSYPHILIS (V)
SYPHILITIC ARTERIOSCLEROSIS	V
SYPHILITIC CEREBRAL THROMBOSIS	V
SYPHILITIC APOPLEXY	V
ANEURYSM	V
SYPHILITIC EPILEPSY	V
PARENCHYMATOUS NEUROSYPHILIS (P)
GUMMA	P
CEREBROSPINAL SCLEROSIS	P
SYPHILITIC PARANOIA	P?
SYPHILITIC CHOREA	P
SYPHILITIC EPILEPSY	P
TABETIC PSYCHOSIS	P?
SYPHILITIC MUSCULAR ATROPHY	P
SYPHILITIC NEURITIS	P
Chart 4a

MENINGOVASCULAR NEUROSYPHILIS (MV)
CEREBRAL SYPHILIS	MV
CEREBROSPINAL SYPHILIS	MV
SYPHILITIC EPILEPSY	MV
MENINGOPARENCHYMATOUS NEUROSYPHILIS (MP)
CEREBRAL SYPHILIS	MP
CEREBROSPINAL SYPHILIS	MP
TABES DORSALIS	MP
ERB’S SYPHILITIC SPASTIC SPINAL PALSY	MP
VASCULOPARENCHYMATOUS NEUROSYPHILIS (VP)
CEREBRAL SYPHILIS	VP
CEREBROSPINAL SYPHILIS	VP
PARETIC NEUROSYPHILIS (GENERAL PARESIS)	VP
LISSAUER’S GENERAL PARESIS	VP
MENINGOVASCULOPARENCHYMATOUS NEUROSYPHILIS (MVP)
CEREBRAL SYPHILIS	MVP
CEREBROSPINAL SYPHILIS	MVP
PARETIC NEUROSYPHILIS	MVP
TABOPARESIS	MVP
DOUBTFUL (TOXIC?, IRRITATIVE?) NEUROSYPHILIS (?)
“PARESIS SINE PARESI”
SYPHILITIC NEURASTHENIA
TABETIC PSYCHOSIS
SYPHILITIC PARANOIA
SYPHILITIC POLYURIA, POLYDIPSIA
SYPHILITIC NEURALGIA
Chart 4b

Summary: We have here dealt at length with a long-standing Diffuse Neurosyphilis affecting to some extent the entire meninges and producing a destruction of posterior column fibres and numerous other fibres of the spinal cord (tabetiform portion of the neurosyphilis picture). We have also found central lesions of the corpora striata affecting the destruction of both pyramidal tracts (paraplegic portion of the neurosyphilis picture). We have found evidences of acute inflammation (lymphocytosis) in the cervical region of the spinal cord and in the left eighth nerve (progressive inflammatory neurosyphilis picture). In short, we have presented a case of diffuse (meningovasculoparenchymatous) neurosyphilis characterized by an ascending character in a course of at least 16 years; we have indicated a number of possible clinical correlations, not only with the major portion of the clinical course (symptoms of myelitis and pyramidal tract destruction), but we have also mentioned, merely for their suggestive value, a number of finer correlations between histological findings and certain clinical features (notably transient losses of vision and hearing, and a partial return of the lost knee-jerks). Bearing in mind the clinical and anatomical findings of this case, we shall be able to discuss the cases that follow in a briefer and more condensed fashion.

TABETIC NEUROSYPHILIS (“tabes dorsalis,” “locomotor ataxia”) complicated by vascular neurosyphilis (hemiplegia). Autopsy.

Case 2. Francis Garfield had been a successful lumberman and had enjoyed good health until his forty-fifth year. Suddenly one day, while walking on the street, Garfield lost the use of his legs and for a time was quite unable to walk. However, he recovered locomotion and after a time there was nothing wrong with his leg movements except a slight ataxia.

At the age of 52 Garfield had to give up work. It appears that he had been becoming cranky, sometimes, for example, shouting, whistling and slamming doors, apparently to annoy the family. His intellectual capacity seemed to be maintained, although his memory was slightly impaired.

At 67 years there was an ill-defined seizure, followed a few days later by another seizure with aphasia (wrong words used and lack of understanding of things said).

For years Garfield had been totally deaf in the right ear (following explosion of a gun?). Now, however, the left ear also showed a sensory impairment. Slight slurring of speech had been noticed first in the sixty-sixth year.

Physically there was a slightly enlarged heart with accentuated second aortic sound and irregular rhythm. Neurologically, inability to stand or walk; marked ataxia in his leg movements; upper extremities quite well controlled; the pupils were small and unequal, the left being larger than the right; although the reactions were difficult to test, the pupils seemed to react slightly to direct light stimuli; the knee-jerks were absent; tests for sensibility so far as could be determined did not show any abnormalities; there was much complaint of sharp pains in the legs.

There is no doubt that we are here dealing with a case of Tabes Dorsalis plus certain complications due to Vascular Lesions. The case went on to death from rupture of aortic aneurysm (also doubtless a syphilitic complication). The death occurred at 71, four years after admission to Danvers Hospital.

MAIN FORMS OF NEUROSYPHILIS

(CLASSIFICATION OF THIS BOOK)

DIFFUSE NEUROSYPHILIS

(non-vascular forms of “cerebral,” “spinal” and “cerebrospinal syphilis”)

VASCULAR NEUROSYPHILIS

(“cerebral arteriosclerosis,” “cerebral thrombosis”)

PARETIC NEUROSYPHILIS

(“general paresis”)

TABETIC NEUROSYPHILIS

(“tabes dorsalis”)

GUMMATOUS NEUROSYPHILIS

(“gumma of membranes, of brain”)

JUVENILE NEUROSYPHILIS

(paretic, tabetic, diffuse)

Chart 5

POSSIBLE INVOLVEMENT
BRAIN AND CORD SYPHILIS
[M]embranes, [V]essels, [P]arenchyma
[MVP]	EARLY, LATENT?, SYMBIOSIS?, ATTENUATION?....
MVP	CEREBRAL, CEREBROSPINAL SYPHILIS, PARESIS	MVP
[M]VP	PARESIS; SYPHILITIC ARTERIOSCLEROSIS	VP
M[V]P	?SYPHILOTOXIN FROM MENINGITIS	MP
MV[P]	SYPHILITIC MENINGITIS; CEREBRAL OR CEREBROSPINAL SYPHILIS	MV
[MV]P	SYPHILOTOXIC ATROPHY OR SCLEROSIS	P
M[VP]	SYPHILITIC MENINGITIS	M
[M]V[P]	SYPHILITIC ARTERIOSCLEROSIS	V
M, V or P in brackets [] means not involved.
Chart 6

NEUROSYPHILIS

SIX TESTS

BLOOD WASSERMANN

SPINAL FLUID WASSERMANN

SPINAL FLUID CYTOLOGY

SPINAL FLUID GLOBULIN

SPINAL FLUID ALBUMIN

SPINAL FLUID GOLD SOL

Chart 7

This case has been especially worked up and published by Dr. A. M. Barrett on account of the fact that the vascular lesions of the brain had produced a condition of pure word-deafness. Reference is made to the Journal of Nervous and Mental Disease, Vol. 37, 1910, for a complete description of the brain findings and an analysis of the word-deafness, a summary of which is as follows:

“Reaction to Words and Sounds.—Total deafness to words spoken, but gives attention to sounds; no ability to recognize meaning of sounds heard; no ability to repeat words heard. Spontaneous Speech.—Retained ability to speak spontaneously, with rare paraphasic utterances; occasional inability to speak readily the word desired, but later always giving the correct reaction; calculation fair; spelling good except for occasional paraphasia; spelling good for words pronounced. Reaction to Things Seen.—Objects correctly recognized and named except for an occasional paraphasic reply; mistakes in pronunciation not recognized; correct color recognition. Reaction to Things Felt.—Good for familiar objects; an occasional paraphasic reply. Reaction to Words Seen.—Reads printing and writing understandingly; unimpaired reading except for an occasional paraphasic reply; meaning of familiar signs recognized; slight difficulty in readily understanding meaning of arithmetical signs. Writing.—Spontaneous writing and drawing ability retained; ataxia (tabetic) in writing movements; no ability to write from dictation. Internal language.—No evidence of impairment.”

The brain post mortem showed severe atheromatous degeneration of the arteries at the base of the brain. Both middle cerebral arteries showed scattered atheromatous patches. The pia mater was transparent and delicate, except in the regions of both Sylvian fissures. There were residuals of old softening in both temporal lobes. In the fresh brain the regions of the right and left first temporal convolutions were sunken inward, and the pia intimately adherent to the softened areas. The limits and more exact localizing of these softenings were worked out from serial sections.

Barrett found in his serial sections that, although the transverse temporal convolutions of the left hemispheres were intact, these convolutions were undermined throughout their entire extent by degenerations in the fibres of the center of the first temporal convolution. Barrett, accordingly, regarded his case as essentially a case of subcortical tissue destruction. He agrees with various authors that the pure word-deafness of his case is the result of an isolation of the receiving station in the transverse convolutions of the left hemisphere. The tissue destruction produced by the vascular lesion had cut off the transverse convolutions from the internal geniculate body.

We are here, however, not considering the origin and relations of pure word-deafness but present the case as one of tabes dorsalis of 20 years standing, terminated by two characteristic syphilitic complications, first, an extensive destruction of brain tissue through cerebral thrombosis and secondly, fatal aortic aneurysm.

Summary: We have here dealt briefly with a long-standing case of Neurosyphilis of the Tabetic type: A characteristic but not necessary complication of the case is the Late Cerebral Vascular Involvement. The posterior column sclerosis is virtually the only spinal change. Spinal meningeal changes are absent (although it is to be assumed that chronic inflammatory changes in the posterior roots were at one time present in some quantity and although the spinal fluid characteristically shows lymphocytosis in tabetic neurosyphilis).

Whether the spirochetes produce special toxic components able to cause tabes or whether special kinds of spirochete are the tabes-making kinds is hard to say. Special qualities of individual tissue may be involved.

The cerebral lesions of a cystic nature are of vascular origin, like the differently localized encephalic lesions of Case 1 (Alice Morton). Vascular syphilis is not a special property of the vessels of the nervous system. In fact this very case died of aortic aneurysm.

PARETIC NEUROSYPHILIS (“general paresis,” “dementia paralytica,” “softening of the brain”). Autopsy.

Case 3. James Dixon, 44, was first seen at the Danvers Hospital, reciting verses in a dramatic and noisy way. He remained good-natured and jolly; nor was there any change in his euphoria until he had become physically weaker and more generally demented. In fact, Dixon appeared to become more and more expansive as he became physically weaker. He was in the habit of describing himself as “O. K., No. 1, Superfine.”

Physically the patient was gray and bald on vertex, had a dusky complexion, was very thin (6 ft. in height, weight 155 lbs.); the mucous membranes were pallid; the teeth rather poorly preserved; the heart was somewhat enlarged; the pulse irregular in rhythm, of poor volume and tension.

Neurologically, the patient showed a characteristic Romberg sign and ataxia in walking a straight line. The tremulous tongue was protruded to the left, and there was a coarse tremor of the extended fingers. The knee-jerks were absent, and the Achilles jerks could not be obtained; the plantar reactions were slight; the arm reflexes were present. The pupils were stiff to light. There was a marked vocal tremor. The sensations could not be tested on account of the patient’s mental state.

It appears that Dixon had left school at about 16, at about 22 had gone into the provision business, and later had become a hotel clerk. He had married at 28; there had been two miscarriages, at three months and six weeks respectively; one child was stillborn; four children were living.

The patient was not very alcoholic. The patient’s wife thought the symptoms had been coming on since his forty-first year when irritability set in, but he was not discharged from work until about a year since. He was taken back again after his wife’s pleas, and remained at work about three months; but for ten months before admission to the hospital, Dixon had done practically nothing, had shown a marked memory failure and speech defect, at the same time claiming to be a person capable of doing and accomplishing everything. He had become careless of his personal appearance, collected a drawer-full of stumps of cigars, carried lumps of coal in his pocket, laughed causelessly, and spat on the carpet.

We here deal with a case of unknown duration from the initial infection, but with symptoms lasting about three years and three months. Aside from the cause of death (empyema of left pleural cavity associated with acute hemorrhagic splenitis, acute ileitis, and bronchial lymphnoditis), the body showed a number of other lesions outside the nervous system. There was the usual sclerosis of the aorta, though perhaps less marked than usual. There was a curious acute arteritis with fusiform dilatation of the arteria profunda femoris, with an edema of the thigh muscles and blebs of the overlying skin. There were also multiple chronic caseating lesions of the liver, without evidence of fibrosis. The explanation of these liver lesions is not yet clear. There was a cloudy swelling of the kidney.

The calvarium was dense and the dura mater thick and adherent. There was a chronic leptomeningitis, which, however, was rather unusual in being most marked in the posterior cisterna and along the sulci of the cerebellar hemispheres. There was a general cerebral sclerosis, with a question of atrophy of the superior temporal gyri (suggesting the so-called Lissauer’s paresis). There was a marked cerebellar sclerosis with a consequent sclerosis (grossly palpable) of the commissural fibres of the pons. There was a generalized slight spinal sclerosis. As a fair sample of the variety of head findings in paretic neurosyphilis, the details of the head examination are presented.

A. Normal postcentral cortex. (Compare B.)

B. Nerve cell losses. Perivascular deposits of mononuclear cells, amongst which are numerous plasma cells. Note decrease in number of nerve cells. Note irregular disposition of nerve cells. From paretic neurosyphilis.

Crown bald, with a slight fuzzy growth of short hairs. Scalp slightly adherent to calvarium; latter of usual thickness but denser than normal. Dura adherent to calvarium in region of vertex; dura not remarkable. Sinuses normal. Arachnoid villi moderately developed. Pia mater a trifle thickened and rather evenly throughout the cerebral portion. Linear sulcal markings are remarkable for their absence. The wall of the cerebellomedullary cisterna is thick and opaque. The most prominent pial thickenings are over the cerebellum. These are linear or may show feathery out-growths and are seated over the sulci, particularly in the neighborhood of the fissure and about the great cerebellar notch. They correspond fairly well with the focal variation in consistence of underlying tissues noted below.

Brain weight, 1265 grams. Consistence somewhat increased throughout and somewhat evenly increased. The prefrontal region shows the maximal increase of consistence but the remainder of the frontal region and corresponding occipital region are much firmer than normal. The two superior temporal gyri appear to be firmer than adjacent gyri and are possibly slightly diminished in superficial diameter. The hippocampal gyri are fairly firm. The substance on section is a trifle more moist than normal. The gray and white matter cut quite evenly. Diminution in depth of gray matter, if existent, could not be demonstrated. The ventricles show a moderate sanding throughout, best marked in the fourth ventricle. The basal ganglia are not remarkable except for the development of numerous dilated perivascular spaces about the lenticulostriate vessels. The pons is atrophic, but more so on the right side. The pons, like the prefrontal cortex, shows on section a distinct increase of consistence immediately beneath the pia mater. The white bands of the pons on section are distinctly firmer than the intervening substance. The olives are of equal consistence. Weight of cerebellum, pons, and medulla, 155 grams. The cerebellum shows an obvious atrophic and gliotic process of a symmetrical character. The superior surface, including both vermis and hemispheres, shows a consistence above normal and general reduction of the depth measured from the white matter. The reduction in depth gives rise to a visible depression as compared with tissue posterior to the postclival sulci. The lobus cacuminis, though slightly raised from the surrounding lobes, is equally firm, if not firmer. The superior and inferior surfaces show practically an equal increase of consistence. The dentate nuclei are not especially increased in consistence. The flocculi are reduced in size about one-third.

There was slight universal increase in consistence of spinal cord, best marked in lumbar region.

Microscopic findings are here presented merely in sufficient detail to establish the diagnosis. The left superior frontal gyrus shows extensive and somewhat irregular cellular and fibrillar gliosis of the plexiform layer, together with an increase of thickened vessels having lymphocytes and plasma cells in their sheaths.

The perivascular infiltrations are most extensive in the lower layers of the cortex. The lamination is in places thoroughly obscured, except that representatives of the layer of large external pyramids are almost always demonstrable.

The layer of medium-sized pyramids has undergone more numerical loss of elements than have the other layers.

Gliosis of white matter.

Specimens from the cerebellum show a destructive process of great severity, but a little irregular in extent, affecting chiefly the Purkinje cell belt. The Purkinje cells are often absent throughout one side of a given lamina, and there has ensued a dense accumulation of neuroglia cells along a former Purkinje cell belt, together with a considerable gliosis of the molecular layer. Considerable gliosis of the white matter, both diffuse and perivascular in distribution.

Perivascular plasma cell infiltrations as in cerebrum, but largely meningeal or in the white matter.

Sections from the corpora striata demonstrate a mild and early granular ependymitis, considerable subependymal gliosis of cellular type, considerable perivascular gliosis in the white portions of the tissue, and a moderate infiltration of perivascular sheaths with pigmented cells, lymphocytes, and plasma cells. There is little evidence of alteration in the nerve cells. Some are unevenly pigmented.

Summary: We here present a case with numerous and widespread neurosyphilitic lesions. However, the gross cerebral vascular complications of Case 1 (Alice Morton) and of Case 2 (Francis Garfield) are notably absent in James Dixon. Rather atypical (there seems to be always something atypical in cases of neurosyphilis!) are the liver lesions and arteritis of the leg, atypical, that is to say, for Paretic Neurosyphilis. Highly typical of paretic neurosyphilis and almost constant therein is the aortic sclerosis.