Chapter IX: Introduction (1)

It is a privilege to be allowed to write a word of introduction to a textbook which so richly fulfils its function as does this volume on the manifold disorders classified under Neurosyphilis, a subject of which the importance for the welfare of society is found to loom the larger the more deeply its mysteries are probed.

The case histories with which its pages are so amply stocked are carefully analyzed in accordance with a broadly chosen plan, and the generalizations that precede and follow them are obviously based on a wide and varied personal experience such as alone could render a familiarity with the literature of the subjects treated adequate to its best usefulness. Both writers were indeed well adapted for this task. Dr. Southard, as everyone is aware, has long been a highly conscientious, ardent and productive worker in the department of pathological anatomy, and of late years a careful student of clinical diagnosis and methods, both at the Danvers State Hospital and still more, at the Psychopathic Hospital which he worked so hard to found; while Dr. Solomon’s researches, in the special field of neurosyphilis, have been of the highest order.

Undoubted as are the merits of the case-system of instruction that has been so much in vogue in recent years, and excellent as is the modern supplementation of this method by the use of published records, the danger is still real that the student will have presented to him a picture of nature in disease that is too diagrammatic, too concise, with the result that while the task of memory is lightened through simplified formulation, the training of the doubting and inquiring instincts is often given too little stimulus and scope. In this book this danger is deliberately met through the casting of emphasis rather on the pluralistic aspects of the processes at stake than (primarily) on their unitary aspects.

The student who utilizes this volume cannot but emerge from his study a more thoughtful person than he was at the period of his entry. He will have seen that clinical rules of thumb cannot be followed to advantage, and that, on the contrary, surprises are to be expected and prepared for. Let the recognition of this fact, if it seems to increase the difficulties in the way of diagnosis, not lead to pessimism in that respect, or to hopelessness in therapeutics. On the contrary the writers’ bias is towards the worth-whileness of clinical efforts and an increased respect for accuracy and thoroughness in the utilization of modern methods of research. The chance is indeed held open that even the gaunt spectre of “General Paresis” may prove to be less terrible than it seems, and for this hope good grounds are given.

It is in this way made clear, on the strength of anatomical evidence of much interest, that even if in the treatment of a given patient, the time arrives when a fatal or unfavorable result seems manifestly foreshadowed, it may be still worth while to renew the treatment with fresh zeal, for the sake of combatting some symptom or exacerbation, for which a locally fresh process furnishes the cause.

Another noteworthy principle here emphasized and illustrated is that the relationship between “functional” (hysterical, neurasthenic, migrainoid) symptoms and the signs (or symptoms) of organic processes is clinically important and worthy of much further study. This is a matter which, in a general sense, has interested me for many years. Above and over the “organic” hovers always the “functional,” as representing the first indication of the marvelous tendency to repair, or substitution, for which the resources of nature are so vast. Yet this functional tendency also has its laws, of which, in their turn, the organic processes display the action in quasi diagrammatic form. Hysteria, neurasthenia, migraine, etc., do not arise _de novo_ in each case, but conform to typical, though not rigid, formulas, susceptible of description. I have recently had the opportunity to study in detail an analogous series of transitions between the movements (and emotions) indicative of apparently purposeless myoclonic movements (on an epileptoid basis) and the movements of surprise, engrossment, purposeful effort, the excitement and joy by which the former were excited and into which they shaded over.

Taken altogether, this book represents work and thought in which, for amount and kind, the neurologists of Boston may take just pride.

JAMES J. PUTNAM.

ST. HUBERT’S, KEENE VALLEY, NEW YORK.
_August, 1917._

Me miserable! which way shall I fly
Infinite wrath and infinite despair?
Which way I fly is Hell; myself am Hell;
And, in the lowest deep, a lower deep
Still threatening to devour me opens wide,
To which the Hell I suffer seems a Heaven.

Paradise Lost, Book IV, lines 73–78.

I. THE NATURE AND FORMS OF SYPHILIS OF THE NERVOUS SYSTEM
(NEUROSYPHILIS)

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.

Apparent new formation of small blood vessel. Photographed by Dr. A.
M. Barrett.
]

Rod cells (Stäbchenzellen) in paretic neurosyphilis. Photographed by
Dr. A. M. Barrett.
]

Granular ependymitis—microscopic appearance of a marked example of
“sanding” of ventricle.
]

Characteristic and constant in paretic neurosyphilis is the =Plasmocytosis and Lymphocytosis, Perivascular= in distribution about small cortical vessels. There is also a characteristic (though characteristically less prominent) =Plasmocytosis and Lymphocytosis, Meningeal= in distribution. The pleocytosis of the spinal fluid, almost constant though variable in amount in life, is an indicator of the meningeal picture and less directly of the parenchymatous picture.

=Granular Ependymitis= (“sanding” of ventricle floors) is characteristic and may be regarded as part of the parenchymatous picture. This ependymitis is an indicator how chemical changes could be readily produced at least in the ventricular fluids, since the limiting membranes of the nerve tissue are here subject to multiple breaks. The “sanding” is a neuroglia reaction to these multiple small breaks (Weigert’s explanation).

Parenchymatous losses have led to =Atrophy and Sclerosis=, of very varying extent in different parts of the encephalon. The atrophy is characteristic in paretic neurosyphilis, but by no means constant. Numerous cases have come to autopsy without clearly defined gross atrophy. Sclerosis is also characteristic and even more frequent than atrophy, doubtless because sclerosis represents an earlier phase of a process eventuating in gross atrophy.

A =Tabetiform Picture= characterizes the spinal cord, but in this case the tabetic clinical picture did _not_ precede the paretic clinical picture. We are consequently to regard the tabetic spinal process as incidental and on all fours with the =Cerebellar and Pontine Atrophy=.

=VASCULAR NEUROSYPHILIS (“syphilitic cerebral thrombosis”).
Autopsy.=

=Case 4.= James Pierce was an almshouse transfer to the Danvers Hospital in his fiftieth year. He died three years later. The accompanying brain pictures demonstrate so extensive a lesion of the left hemisphere that it is of great interest to determine if possible the genesis and course of his disease. It appears that syphilis had been acquired somewhere about the age of 38 or 40, so that the total duration of the process was between 13 and 15 years. In Pierce’s forty-third or forty-fourth year, he had a shock while walking in the streets of his native city, whereupon he was subsequently transferred to the Danvers Hospital, whose data have been summed up as follows (we are obliged to Dr. Charles T. Ryder for these data):

=Neurological examination:= Neuromuscular condition: Barely able to
walk or stand without assistance; hemiplegia of right side; swings
foot out and drags toe out and around in attempting to walk. Right
hand held by side, flexed at right angle; fingers contracted and
thumb thrown across palm. Can lift arm from side; practically no
movements of forearms or fingers; atrophy of deltoid, arm, forearm,
and hand. Muscular movements of left upper extremities fairly well
performed; good strength.

=Cranial nerves:= Refuses to respond to any tests to determine
hearing or vision, but evidently hears what is said to him, and in
his movements gives no evidence of deafness. Right corner of mouth
droops; tongue protrudes straight.

=Reflexes:= Pupils dilated; margins irregular; left pupil larger;
they vary in size but it is impossible to determine whether the
variation is due to light or accommodation reflex. Reflexes of right
side extremely exaggerated throughout; there is little ankle clonus;
Babinski is not obtained, patient holding his toes in flexed
position in resisting attempts to elicit reflexes.

=Sensations:= Reaction to pain stimuli on either side. Evidently
some anesthesia on right side, but pressure is apparently very
painful. There is considerable spasticity of limbs on right side on
passive motion. Too demented to make accurate tests.

The above examination was made on May 6, 1904. On May 20th the
record states:

There is almost complete sensory aphasia with word-deafness; some
paraphasic circumlocution. Many of his words are very well
enunciated but have no meaning. Is apparently unable to recognize
objects or their uses.

Brother stated that he was always supposed not to be over bright.
Physician’s certificate states that he is epileptic, averaging two
attacks per week. On the 15th of May he had a general convulsion;
was unconscious for half an hour, and dull and drowsy for two hours
afterwards. On the 19th, he had a similar attack in the afternoon,
the convulsion lasting a minute, and he was stuporous for an hour.

On November 8th he had a severe epileptic convulsion. His body was
curled up to the right. The convulsive seizure lasted for two
minutes and was followed by complete unconsciousness for an hour,
when the patient roused and appeared as usual in a few minutes. From
that time to December 15th he had five epileptic convulsions; he was
much more feeble, and unable to help himself as much as formerly.

Nov. 7, 1905: Patient has had occasional convulsions since last
note, but none during the last three months. He is confined to bed,
has become very much demented, and shows very marked speech defect,
so that he is almost unintelligible. He understands only the
simplest directions. Legs are considerably contracted and knees are
flexed. Arm and hand on the right are paralyzed and show some
atrophic changes; partially flexed. Left elbow jerk is very lively.
On May 23, 1906 he was reported as having Achilles on right side
only, and Babinski on right side. He died January 5, 1907.

=The autopsy findings= were as follows:

=Head:= Calvarium of moderate thickness; diploë present; dura
slightly adherent over bregmatic region. Longitudinal sinus contains
cruor clot. Dura is somewhat thickened and slightly more opaque than
normal. Pacchionian granulations, small but fairly numerous. Pia
contains throughout a considerable excess of clear serous fluid. The
convolutions in general are of good breadth and proportion. There is
an atrophic area roughly circular in outline and about 2 cm. in
diameter in the posterior part of the right third frontal
convolution corresponding to Broca’s area on the opposite
hemisphere. The space thus formed is filled with edema held by the
pia. On the left side is a similar subpial collection which covers
the site of the posterior portions of all of the third frontal
convolutions, parts of the lower end of the precentral convolution,
and the whole of the first temporal convolution, which have
disappeared entirely. The basal vessels show slight changes.

Cerebellum and basal ganglia are grossly normal.

The spinal membranes are negative. The regions of the pyramidal
tracts in the cord are firm, project slightly from surface of
section, and are china white.

=Summary:= Here is a picture made up almost purely of VASCULAR NEUROSYPHILIS, with SECONDARY SPINAL (PYRAMIDAL TRACT) CHANGES. Doubtless the genesis of this picture is allied to that of Case 1 (Alice Morton) and to that of the terminal vascular complications in a tabetic, Case 2 (Francis Garfield).

The absence of meningeal and parenchymatous (i.e., outside the region of necrosis produced by the vascular disease) lesions is characteristic of an important group of neurosyphilitic diseases. It is clear that the case, although one of _extensive_ lesions, is _not_ one of _diffuse_ lesions in the sense of Case 1 (Alice Morton).

The spinal fluid picture in life may nevertheless show (as other cases amply demonstrate) a certain amount of lymphocytosis and possibly plasmocytosis, together with a variety of other changes. Treatment might be expected to keep down these associated changes, although obviously the effects of the necrosis are final and definite. Franz in Washington has succeeded in “reeducating” some of these hemiplegics, employing lower mechanisms of the nervous system.

Vascular neurosyphilis—effects of syphilitic thrombosis of Sylvian
artery 10 years before death. (Case 4.)
]

=Case 4.= (See previous figure for brain lesion.) Three levels of the
spinal cord showing unilateral pyramidal tract sclerosis, 10 years
after cerebral thrombosis.
]

=JUVENILE PARETIC NEUROSYPHILIS (“juvenile paresis”). Autopsy.=

=Case 5.= John Lawrence was an under-sized negro, who came under hospital observation when he was 23 years of age. There was some evidence that the patient’s father was a neurosyphilitic although accurate data were out of the question. At all events, John had Hutchinsonian teeth, a forward bowing of the tibiae, and Argyll-Robertson pupils. These findings together with a history of backwardness at school seem to stamp the diagnosis. It seems that there had been a change for the worse from the age of 18, though the boy had been able to sell newspapers and black shoes up to within a year of his arrival at the hospital. During the last months of his life, he showed a general incoördination, with false movements suggesting those of a drunken person. There were numerous tremors, the glance was shifting, and there was a tendency to nystagmus. Some of these phenomena (taking into account that the Hutchinsonian teeth were not entirely typical and there was even at times some doubt as to whether the pupils were actually stiff) led to a question of the diagnosis multiple sclerosis.

There was, however, little doubt that the case was one of juvenile paresis. Among the symptoms found at various times in this case are the following: disorientation for time, place and persons, confusion, with coarsely irrelevant replies to questions, ill-defined and transitory delusions of persecution, auditory, tactile, and visual hallucinations, and defective memory.

Early in life, the patient had had a habit of falling asleep in school hours, and had experienced a number of falls at various times. During an attack of measles he had had a number of spasms, each of which lasted ten minutes or more.

The =autopsy= showed death to be due to an early bronchial pneumonia. The thymus was persistent, measuring 3 × 2 × .5 cm. The marrow of the femur was red.

There was a moderate degree of =sclerosis of the aorta= confined to a few plaques in the arch (not a characteristic syphilitic scarring of the aorta). The spleen was small and had a thickened capsule.

The majority of the lesions, however, were in the =nervous system=, and the following description is taken from the routine hospital records to exemplify the findings in a fairly characteristic case of JUVENILE PARESIS.

=Head:= Scalp closely adherent to =calvarium=. Calvarium heavy
without diploë. =Dura= adherent to calvarium in bregmatic region.
Sinuses contain liquid blood. Arachnoidal villi in considerable
quantity. =Pia mater= contains considerable clear fluid and shows
diffuse haziness and focal thickenings. The diffuse haziness is
almost universal and is best marked over the superior surface of the
cerebellum. The focal thickenings are of general distribution over
the veins of the sulci on the superior surface of the brim and are
heaped up to form considerable linear mounds near the region of the
arachnoidal villi. The superior surface of the cerebellum is
traversed by similar linear mounds of fibrous tissue running at an
angle to the laminæ. There is no notable increase of fibrous tissue
at the base.

=Brain:= Weight 965 grams. The sulcation is roughly symmetrical
except in the occipital poles where there is unusually rich and
complex but shallow sulcation. The cortical substance is everywhere
firmer than normal, but the sulci fail to flare notably. In a few
places there is a focal increase of consistence of still greater
degree with apparent local hypertrophy (or gliosis with increase of
substance). These foci are in the right second temporal gyrus (3 cm.
in diameter) and in the left first temporal gyrus (of same size but
somewhat less firm) and are of a whitish, waxen appearance, being
visible several feet away by reason of their color and apparent
encroachment upon the adjacent sulci. The foci are sharply limited
by the sulci laterally, but pale out gradually before and behind.

The convolutions of the vertex show another type of lesion. The
tissue of the greater part of the vertex resembles that of the
flanks and base in being firmer than normal and of a grayish pink
color. Behind the fissure of Rolando on the right side and behind
the anterior limits of the ascending frontal region on the left side
the brain tissue of the vertex becomes suddenly still firmer and of
a yellowish gray color. This lesion disappears gradually into the
occipital microgyria behind and the gyri gradually lose their
yellowish tint. The lesion fades away gradually so that it fails to
involve the temporal convolutions.

The cerebral tissue cuts firmly and smoothly. The tissue of the
frontal region is a little edematous. The white matter is of a
normal appearance. The ependyma of all the ventricles is somewhat
sanded. The fourth ventricle is most affected.

The =cerebellum= is not edematous and is as firm as the normal
olivary bodies. The cerebellar hemispheres are symmetrical and of a
normal appearance, save that the laminæ are slightly narrower than
usual and very compactly set. The color, where not obscured by the
haziness of the pia mater, is of a grayish pink somewhat suggestive
of freshly tanned shoe leather. The substance cuts smoothly and
firmly. The dentate nuclei are unusually firm. The =pons= is small,
but of the usual color. Lower structures normal except the =cord=
which is small and shows curious deviations from the normal
markings. The posterior horns and gray commissure are at many levels
the only structures to preserve the normal gray appearance, so that
the H or butterfly appearance is replaced by a crescent. At these
levels, traces of gray matter often stand out in the loci of the
anterior horns.

The important =anatomical diagnoses= in the nervous system are as follows:

Atrophy of cerebrum, 965 grams (there is of course a question whether we are not dealing with a degree of cerebral hypoplasia).

Focal scleroses of cerebrum, suggesting the tuberous scleroses of Bourneville.

Occipital microgyria.

Cerebral and cerebellar gliosis.

Chronic ependymitis.

Gliosis of the gray matter of the spinal cord.

Chronic diffuse and focal leptomeningitis.