Part 3

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.

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.

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).