Chapter XV: Introduction (7)

=Note.= THYROID: Weight 125 grams. Both lobes and isthmus enlarged.
One lobe more than the other; lobe on one side measuring 6 × 4 cm.

Anatomical Diagnoses

Enlargement of thyroid gland.

Exophthalmos with dilated pupils.

Fatty degeneration of thoracic muscles.

Slight aortic sclerosis.

Dilatation of right heart.

Hypertrophy of left ventricle.

Slight tricuspid endocarditis.

Bicuspid aortic valve.

Hypostatic pneumonia.

Acute and chronic splenitis.

Fatty liver (central necroses?).

Acute nephritis.

Chronic gastritis.

Small breasts.

Axillary hair absent.

Petechial eruption of chest.

Varicose veins.

Chronic external adhesive pachymeningitis of left side.

Moderate swelling of right hemisphere with venous injection.

Slight occipital gliosis of both sides.

Slight gliosis of orbital and hippocampal gyri of right side.

Sclerosis with atrophy of occipital and hippocampal white matter of
right side.

Gliotic lesion (1.5 × 2 × 2 cm. of right lenticular nucleus
involving anterior commissure).

1. Was the exophthalmic goitre in Carrie Pearson due to syphilis?
Unfortunately we have no clear proof that Carrie Pearson was
syphilitic. She was stated to have been syphilitic by the
physician who treated her before her commitment to Danvers
Hospital. There is, however, no proof of syphilis, inasmuch as the
patient died in the pre-Wassermann period.

2. Is the thalamic lesion probably syphilitic? No lymphocytosis or
plasmocytosis characterizes the lesion, which is the only lesion
of the sort in the Danvers collection. It would not do to call a
lesion syphilitic just because it is _sui generis_. In any event,
the clinical analysis of the case faced the claim of syphilis as
an actual factor in the patient’s life and as a possible factor in
the goitre.

=It is well known that the ARGYLL-ROBERTSON PUPIL is characteristic
of the so-called “PARA-SYPHILITIC DISEASES” (“general paresis” and
“tabes”); does this sign occur in other neurosyphilitic conditions?=

=Case 54.= Julius Kantor was a shoemaker of 35 years, who came to the hospital for treatment because his family physician had found a positive W. R. in Kantor’s blood serum. He had had a cough for a number of years, and during the last year a little blood had been found in the sputum; whereupon Kantor had been placed under active anti-tuberculosis treatment. The enterprising family physician had found the positive W. R. in the first days of his treatment for tuberculosis. There was, in fact, a history of a chancre nine years before, which had not been followed by any secondary or tertiary symptoms, and which had been but scantily treated.

There were no mental symptoms.

Kantor was =physically= fairly well developed and nourished. There were a few piping râles in the left upper chest, both in front and back, and also a slight dulness with increased vocal and tactile fremitus. No tubercle bacilli, however, could be found on repeated sputum examination.

=Neurologically=, the pupils were myotic and both showed the Argyll-Robertson reaction. There were no abnormal reflexes whatever, and there was neither ataxia nor speech defect. Not only the blood but also the spinal fluid W. R. proved to be positive; there was a marked increase in the albumin and globulin; there was a gold sol reaction of the syphilitic type, and there were but three cells per cmm.

1. In view of the headache in case Kantor, what other causes of
headache are to be considered? It is certain that irritations of
the dura mater can produce headache, and the physiological
observation of the sensitiveness of the membranes and the
non-sensitiveness of the brain substance is an ancient and
classical observation. Internal hemorrhagic pachymeningitis
produces severe headache. The relations of this disease to trauma,
to arteriosclerosis, and possibly to syphilis (alcohol perhaps
should also be considered) in certain instances have not been
entirely cleared up. Syphilitic headaches are, according to
Lewandowski, dependent also upon a dural affection or upon a
periosteal affection. The headaches of brain tumor are also
commonly related to dural conditions, either directly due to the
pressure of the tumor itself, or indirectly to the heightened
intracranial pressure consequent upon the tumor. It is clear that
the tension under which the dura mater lies is not always
localized in the region of a brain tumor or a syphilitic lesion.
Head has claimed that brain tumor produces headaches of two kinds,
according to whether the disease affects the dura mater or is
dependent upon an increase of pressure in the brain. It does not
appear that the pia mater has any relation to headaches, but
meningitis, in which the inflammation is confined to the pia
mater, is nevertheless associated with headache; the headache is
here supposed to be due to the increase in brain pressure, and
thus actually to an effect wrought upon the dura mater. Vasomotor
disorders and various types of cephalic hyperemia are thought to
produce a kind of headache, but Lewandowski calls this kind of
headache somewhat in question. Reflex headaches are stated to be
produced indirectly by a process of radiation from interior
lesions in the brain. There are certain headaches called nodal
headaches (_Schwielen-Kopfschmerz_). Hypermetropia, caries of the
teeth, adenoids, and diseases of the nose and axillary cavities,
to say nothing of thoracic and abdominal diseases, are also
counted among conditions that may produce headaches. In this
connection, Head has claimed differential zones of headache
corresponding to certain diseases.

The brain itself may produce headache through intoxications,
through conditions produced by a variety of diseases; may follow
neuroses. Alcohol may produce headaches in some persons even when
it is taken in very small doses. Certain uremic cases yield
headaches, as do also gouty and chlorotic conditions. According to
Lewandowski, the headaches of arteriosclerotics are due possibly
to vasomotor disturbances in the membranes, or one may think of
nutritive cerebral disorders. A peculiar form of headache is that
of fatigue after mental work, allied to which is the neurasthenic
headache; constitutional headaches have been assumed to occur, to
say nothing of hysterical headaches. There remains also the
important question of migraine, for which a vasomotor explanation
has been proposed.

2. Was Kantor suffering from tuberculosis of the lungs? The
hypothesis of lung syphilis ought certainly to be very seriously
considered. Upon repeated sputum examination, no tubercle bacilli
have yet been found.

3. Is Kantor a case of general paresis? In the absence of mental
symptoms, and in consideration of the mildness of the reactions,
it is certainly not easy to make the diagnosis of general paresis.
However, the diagnosis of tabes dorsalis is not justified either.
Accordingly, we may answer our question: whether the
Argyll-Robertson pupil occurs in other neurosyphilitic diseases,
by pointing out that in the case of Julius Kantor, as in the case
of Henri Lepère (105) and Frederick Stone (106), the
Argyll-Robertson pupil has been found in syphilitic conditions
that are neither typically paretic nor typically tabetic.

=Does the Argyll-Robertson pupil necessarily indicate
neurosyphilis?=

=Case 55.= Daniel Falvey, 44 years of age, was an almshouse transfer to the Danvers State Hospital in the year 1904, when the principle of state care was adopted in Massachusetts. As in most of the almshouse transfers of that day, little could be discovered as to antecedents. He had been a mill-worker from the time of his immigration in 1890, at 30 years of age. He had been somewhat alcoholic. There was a shock some 17 months before his death, which occurred about seven weeks from the date of transfer.

Not only was he unable to walk unsupported, but when supported there was a slight dragging of the left leg and the gait was noted to be somewhat propulsive. The tongue and hands were tremulous, and the left grasp was somewhat weaker than the right. Both knee-jerks were increased although neither more than the other. There was no sensory disorder.

Although but 44 years of age, Falvey presented the appearance of a much older man. His heart was somewhat enlarged and there was a degree of peripheral arteriosclerosis. On the whole, no special attention was attracted to this case clinically and he was regarded as an example of arteriosclerotic dementia, like many another among the transfers. However, we owe to Dr. H. M. Swift the important observation of the Argyll-Robertson pupils. The case was studied long before the Wassermann method was available, and is here reported merely to call attention to the fact that the stiff pupils may have other neural origin than neurosyphilis.

The autopsy material in the case was worked up by one of the authors.[11] The autopsy had been performed by Dr. A. M. Barrett, who found on section through the brain stem at the anterior border of the pons a mass springing from and continuous with the pineal gland, lying in the third ventricle and the aqueduct of Sylvius. Upon further study, this mass was found to begin posteriorly in the pineal body itself, from which the mass could hardly be told in the gross except by an injected border.

This mass proved upon microscopic examination to be a psammoma, which histologically resembled a glioma rather than a sarcoma. Throughout the mass there was a variable content of fibrillary intercellular substance having the histological reactions of neuroglia fibrillæ. The histological details (mitosis, large giant cells with multiple nuclei, etc.) do not here concern us. We deal with a neoplasm springing from the pineal gland growing on the posterior half of the third ventricle, the anterior orifice of the aqueduct of Sylvius, and the space between the velum interpositum as far back as the posterior corpora quadrigemina. There is no evidence in the body of old syphilis; although it is possible that the stiff pupils were neurosyphilitic, it seems probable that they were related to the pineal tumor. At all events, there are in the literature evidences that the pineal-quadrigeminal group of tumors and other lesions may bring about pupillary disturbances. On this account, we here include the case. The tumor hardly led to an error in diagnosis since neither neurosyphilis nor brain tumor was at all expected clinically.

1. Can alcoholism produce identical results? See Case Murphy, (60),
one of alcoholic pseudoparesis.

2. What is the nature of stiff pupils? A pupil is called stiff in
the sense of the Argyll-Robertson pupil if it fails to react to
illumination either of itself or of the other eye and at the same
time if it reacts properly in convergence and accommodation. Of
course the stiffness of a blind eye must not be regarded as an
Argyll-Robertson pupil. In a case of right-sided Argyll-Robertson
pupil, therefore, the left pupil reacts properly both to direct
illumination of itself and to illumination of the right eye, but
the right eye fails to react to illumination of either eye. Such
an Argyll-Robertson right pupil will remain of the same width both
in darkness and in light. Clinicians agree that the
Argyll-Robertson is diagnosticated rather too frequently than too
seldom, and this by reason of the fact that a sluggishness of
light reaction is interpreted as stiffness. The sign, as is well
known, has come to be regarded as almost pathognomonic of tabetic
or paretic neurosyphilis. Nonne, however, has found among 510
cases of alcoholism, nine instances of Argyll-Robertson pupil and
19 cases of sluggish light reactions. The pathological anatomy of
this sign is still doubtful although a number of schematic
accounts are available; among hypotheses, one may think of an
elective effect of the tabetic or paretic degeneration upon reflex
collaterals. The explanation would then resemble that for absent
knee-jerks and kindred reflex disorders. We should then
hypothesize a loss of the finer processes of the terminal
arborizations about the cells of the nucleus of sphincter nucleus
iridis. However, the situation of the sphincter iridis has not yet
been absolutely determined.

When a pupil is said to be entirely stiff it means that it reacts
neither to light nor accommodation. This condition not
infrequently follows the partial stiffness or Argyll-Robertson
reaction.

3. Is the Argyll-Robertson pupil more tabetic than paretic? This has
been claimed at times, but in point of fact, the Argyll-Robertson
pupil is very frequent in paresis, and so also are posterior
column changes. According to statistics of Bumke, 36% of tabetics
fail to show the Argyll-Robertson pupil, and 38% of paretics.
When, however, finer methods, such as those standardized by
Weiler, with photographic records, are employed, the number of
cases without at least a tendency to the Argyll-Robertson pupil
becomes much smaller.

In connection with the important question as to the classical
Argyll-Robertson pupil and pupillary sluggishness to light, it may
be inquired what are the ocular signs in neurosyphilis? Joffroy
has tabulated the signs in 300 general paretics as follows:

Sign. No. of Per cases. cent. Alterations of light reflex 235 78 Inequality 205 68 Abolition of light reflex (bilateral or unilateral) 156 52 Abolition of light reflex (bilateral) 133 44 Irregularity of pupil 117 39 Irregularity of both pupils 109 36 Diminution of light reflex 108 36 ditto (bilateral) 79 26 Alteration in accommodation reflex 79 26 Diminution of accommodation reflex 52 17 Mydriasis 41 13 Myosis 40 13 Diminution of light reflex (unilateral) 35 11 Abolition of accommodation reflex 35 11 Diminution of accommodation reflex (bilateral) 29 9 Abolition of accommodation reflex (bilateral) 26 8 Diminution of accommodation reflex (unilateral) 23 7 Fundus changes 21 7 Vascular changes 16 5 Abolition of accommodation reflex (unilateral) 12 4 Paresis of the third nerves 10 3 Ptosis 9 3 Irregularity of one pupil 8 3 Nystagmus 7 2 Visual acuity lost 7 2 Atrophy of disc 6 2 Total blindness 5 2 Paralysis of the fourth nerves 1 1

=Can neurosyphilis exist in the absence of positive findings in the
spinal fluid?=

=Case 56.= There was no great difficulty in setting up a diagnosis of general paresis in the case of James Burns, a mechanic of 31 years of age, who came voluntarily to the Psychopathic Hospital for treatment. The point in Burns’ case was that the spinal fluid proved entirely negative in all respects despite the fact that the serum W. R. was positive, and despite the following facts of history and mental examination.

The patient claimed syphilitic infection seven years before, namely, at 24 years of age, and also claimed that he had infected his wife, who was in fact at the time undergoing antisyphilitic treatment. He complained of insomnia, worry, depression, hypersensitivity to noises (such as those made by his own children), thoughts of suicide, and amnesia. The amnesia, however, might be regarded as subjective since our tests failed to show amnesia. Nor was there any diminution in arithmetical ability. Despite the patient’s claim that he had been “way off in his way of thinking,” there appeared to be no delusions. Beyond a certain flightiness in conversation, we could hardly get any evidence of psychosis unless of the neurasthenic order.

=Physically=, however, the left pupil failed to react to light though it was found to react to distance, and the right pupil exhibited a diminution of its reaction to light. There was no ataxia of gait, yet there was a complete Romberg reaction. There was a moderate tremor of the hands and of the tongue. Otherwise there were no reflex disorders upon systematic examination, nor was there any demonstrable disorder in the rest of the physical examination.

1. What is the diagnosis in the case of James Burns? On the whole we
agree with Nonne, that negative spinal fluid findings (of course,
in the absence of treatment) preclude the diagnosis of general
paresis. The symptoms might possibly be explained, however, by
means of a localized syphilitic involvement of the cerebrum, no
cells or products of inflammation having penetrated to the spinal
fluid. According to Head and Fearnsides, this condition may be
found especially in the anterior or middle fossa. Accordingly,
going upon these views of Nonne and of Head and Fearnsides, we
should be entitled to make, perhaps, a diagnosis of cerebral
syphilis.

2. What is the significance of the Argyll-Robertson pupil in James
Burns? Nonne states that if one follows cases with
Argyll-Robertson pupil over a sufficient period of years, they one
and all eventuate in active symptoms of cerebrospinal syphilis
(not necessarily of the cortical type), and this despite the fact
that the pupillary change may have been present a number of years
before any other symptom had developed.

=Neurosyphilis (“DISSEMINATED ENCEPHALITIS”) within seven months of
initial infection. Autopsy.=

=Case 57.= We borrow the main features of a remarkable case examined at the Danvers State Hospital clinically by Dr. H. W. Mitchell and reported elaborately by Dr. A. M. Barrett. This case, whom we shall call John Summers, acquired syphilis at about the end of the third week in May, 1902, and consulted a physician on June 12, at which time a characteristic initial lesion of syphilis was plain. Summers was excessively alcoholic at times and was not seen by a physician again until July 2, just after an alcoholic debauch. At this time there was ulceration of the primary lesion, and a papillary eruption had developed over the arms, chest, abdomen, and legs. Mercurial treatment and mixed treatment were given. Arthritis occurred but disappeared with increased dosage.

About six months after infection, the patient developed severe headaches, hardly controllable by treatment. Amnesia and a certain stupidity, with neglect of personal habits, and even of eating, developed, whereupon Summers was admitted to the Danvers Hospital, December 11, 1902. He weighed 124 pounds, was extremely feeble, with dull and expressionless face, coarse purposeless movements of arms; left pupil larger than right; right external strabismus and ocular ptosis; increased knee-jerks, crossed adductor reflex, coarse tremors of arms and hands; and extreme clouding of consciousness. It was doubtful whether the pupils were stiff to light or not.

The patient died on the ninth day, December 18, in a state of coma. After admission, his stupor had become more marked; there had been incontinence of urine and fæces, and the patient could be aroused only by loud tones. Difficulty in swallowing had developed; the right-sided ptosis had become more marked, and muscular twitchings had developed on the right side. When the left leg was pinched, there was twitching of the left leg and arm. There was slight spasticity of the right arm and leg. An examination upon the day of death definitely showed a lack of reaction of the pupils to light.

1. Exudate in pia mater—mononucleosis.
]

2. Superficial (subpial) cellular reaction of neuroglia tissue
(expanded cell bodies).
]

3. Cellular gliosis of deeper layers of cortex. Apparent increase in
capillary supply, possibly relative to loss of neural elements.
]

Case 57. Neurosyphilis (“disseminated syphilitic encephalitis” of A. M. Barrett), fatal seven months from initial infection. (Photographs by A. M. Barrett.)

Dr. Barrett was able to find in the literature a case of Bechterew which histologically resembled his own case, but though in the instance reported by Bechterew the first symptoms developed within the year following infection, death did not occur until two years later.

In view of a total duration of symptoms clearly not over seven months, it is interesting to inquire how far microscopic brain changes could have proceeded. Neither calvarium nor dura mater showed changes. There was a slight haziness of the pia mater over the convexity, but the pia mater over the base (especially below the cisterna and from thence spreading out over the pons and into the fissure of Sylvius) was not only hazy but definitely thickened and hyperæmic. The thickening was most marked about the root of the right third nerve (corresponding with the eye findings in life). There was also a macroscopic thickening of the left Sylvian artery. Section of the brain showed nothing abnormal except a small area among the pyramidal fibres of the right side of the pons, where there was a single hemorrhagic area about 7 mm. in diameter around which there were small punctiform hemorrhages. (Compare twitchings of left leg and arm upon stimulation of left leg, and note also the muscular twitchings and slight spasticity of right leg and arm noted just before death.) This case was examined and reported upon in 1905. We learn from Dr. Barrett that a re-study of the case with modern methods has failed to demonstrate a spirochetosis.

The meninges show infiltration and destructive and proliferative changes of the blood vessels. Condensed extracts from Dr. Barrett’s full report follow:

There were local variations in the severity of the meningitis. The
sulci showed the most marked infiltration. The slighter degrees of
exudation were made up largely of lymphocytes with a few plasma
cells, occasionally large mononuclear cells, and rarely a
polymorphonuclear leukocyte. Where the exudation was more extensive,
the large mononuclear cells became more common and the
polymorphonuclear leukocytes increased in number. The large
mononuclear cells were often phagocytic, containing from one to six
leukocytes. The exudate was always most abundant about the blood
vessels. The plasma cells were always most numerous in the
adventitia of the veins, here greatly outnumbering the leukocytes.
The polymorphonuclear leukocytes were relatively infrequent except
where there were necrotic areas, which areas were usually continuous
with an infiltration of a vessel wall.

As to vascular changes, the media was not often involved, nor was
the adventitia so often affected as the intima. Such lesions as
appeared in the intima and adventitia were infiltrative rather than
proliferative. The elastica of the blood vessels proved to show but
slight changes.

A characteristic change was the endarteritis,—of a focal nature with
a few large mononuclear and lymphocytic cells pushing the intima
inward at the edge of a lesion. In the more marked portion of the
focal process, the thickness of the intima was greatly increased by
proliferation. Great numbers of large mononuclear cells could be
seen between the intima and the elastica. About these cells and
interlacing among the other elements of the proliferating tissue was
an excess of connective tissue fibres.

The meningeal veins were more often diseased than the arteries;
there was adventitial infiltration with lymphoid and plasma cells;
sometimes the vein walls had become necrotic and infiltrated with
polymorphonuclear leukocytes.

It will be remembered that the left Sylvian artery was grossly
thickened, and microscopic section of this vessel showed a partial
thrombosis.

The brain showed diffuse and focal changes. The _diffuse_ process
was one of nerve cell degeneration and proliferative changes in the
neuroglia and blood vessels, and no section of the many examined
proved to be free from such changes, although in the majority of
instances, these diffuse changes were slight. The cortical layers
showed more of these diffuse changes than did the white substance.
Barrett considered that the glial cell changes were more delicate
indicators of the cortical changes than the nerve cell changes. He
found rod cells, satellitosis, superficial gliosis, and a large
gamut of changes in the neuroglia. There were two rather
characteristic nerve cell changes: a shrinkage change going on to
almost complete destruction, and a type of cell swelling, also
apparently proceeding to complete destruction.

4. Arteritis of pia mater.
]

5. Focal vascular lesions.

Case 57. Seven months from infection. “Disseminated syphilitic
encephalitis,” Barrett. (Photographs by Barrett.)
]

Paretic neurosyphilis (“general paresis”)—cerebral atrophy, _without_
meningitis. Therapeutics cannot hope to restore lost tissue.

Duration. 3 years from beginning of well marked symptoms; 6 years from
beginning of obvious symptoms; 12 years from a so-called “nervous
prostration.”
]

Among _focal_ changes, there were four main types: Areas of
encephalitis, having the general appearance of granulation tissue,
areas of simple necrosis or softening, apparently directly related
to vascular changes near by, hemorrhages, and certain foci regarded
as gummatous.

Save for pial infiltration and a few vascular changes, there was
very little change in the medulla and spinal cord. There was a
hypertrophic gliosis of the margin of the medulla and cord
throughout, and a focal lesion of well marked gliosis at one point
in the bulb. There were no fibre degenerations in the medulla or
cord, nor were there any coarse fibre degenerations in the cortex
itself except in one locus, the left gyrus rectus. This case is of
peculiar value in showing to what extent lesions may proceed in a
period of six to eight months after primary infection.

Of course the case is in one sense entirely atypical. The lesions were not confined to the nervous system. Aside from the maculo-papular eruption and ecchymosis of the skin, there was a diffuse hemorrhage of the inner half of the conjunctiva of the left eyeball, a small hemorrhagic focus in the mitral valve, a caseous nodule, one cm. in diameter, in the apex of the left lung whose tuberculous or syphilitic nature is left in doubt; a broad milk-colored patch of thickening of the capsule of the liver. It is to be noted that there were no gross lesions of the aorta.

=On the classical assumption that PARETIC NEUROSYPHILIS (“general
paresis”) is a fatal disease, is there a disease PSEUDOPARETIC
NEUROSYPHILIS (“pseudoparesis”) which may recover or pursue a long
course like that of a case of diffuse neurosyphilis (“cerebrospinal
syphilis”)?=

=Case 58.= Peter Burkhardt had been an efficient highway inspector, but in his forty-fifth year he had begun to be unable to do his work satisfactorily. His wife had become somewhat afraid of him. He had had somnolent spells in his chair and had squandered money. The mental symptoms had lasted for some six months, but had become more marked during the month preceding admission. Burkhardt would at times fail to recognize his friends.

The general =physical condition= of Burkhardt was very good. The pupils were irregular and reacted sluggishly both to light and to accommodation. The knee-jerks and ankle-jerks were absent. There were no other neurological disorders upon systematic examination. There was a speech defect. =Mentally=, little could be determined except a certain sluggishness.

History and physical examination at once suggested general paresis. The serum W. R. was doubtful, but the spinal fluid reaction was positive, as was the gold sol reaction (which was “paretic”); the globulin and albumin were greatly increased; there were 48 cells per cmm. Antisyphilitic treatment, consisting of salvarsan twice a week and potassium iodid by mouth, was followed by a rapid mental improvement. After two months, Burkhardt was discharged apparently normal, with all the blood and spinal fluid tests negative. He has been taken back into the highway service.

1. What is the proper definition of pseudoparesis? Fournier termed
pseudoparesis certain cases that looked like paresis but were not
syphilitic in origin. Of these cases the most characteristic group
is that of alcoholic pseudoparesis. It is clear that there will be
no difficulty in the definition of a disease pseudoparesis whose
entity is presented in the adjective that precedes the term
(_e.g._, alcoholic pseudoparesis). According to this usage, a case
of pseudoparesis would be one in which the symptoms and possibly
some of the signs somewhat resemble the symptoms of paresis itself
but for which another etiology could be fairly established.

2. Are there any cases of syphilitic pseudoparesis? We are of the
opinion that the term should be dropped. It is true that there are
cases which clinically look like general paresis and exhibit the
appropriate laboratory signs of general paresis but seem to differ
from paresis in their course even when they receive no treatment
whatever. In the present phase of doubt as to the classification
of paretic and non-paretic forms of neurosyphilis, it seems to us
of doubtful utility to characterize a case as pseudo simply
because it differs in its course, particularly as the literature
has always duly recognized that a number of cases of general
paresis have had long courses and sometimes very long remissions.

There is also another group of cases that have been termed cases
of pseudoparesis, namely: certain cases of neurosyphilis which
clinically look like general paresis and seem to be following its
classical course but are interrupted by treatment. Here again it
seems to us doubtful whether the designation pseudo should be
attached to this group of cases, particularly while the whole
therapeutic question in the paretic group of neurosyphilis cases
remains _sub judice_. Accordingly we are tempted to include in the
group of paretic neurosyphilis cases that either get well of
themselves, or get well under treatment, or pursue a very long
course, or are subject to very long remissions. But we make this
decision in terminology without prejudice to the therapeutic
question and it is open to any critic to throw these cases into an
atypical non-paretic group of neurosyphilis cases.

3. How shall we explain the absence of ataxia of case Burkhardt when
knee-jerks are absent and when, therefore, we are entitled to
conclude a certain degree of spinal disease? As stated in
connection with case Sullivan (16), the absence of knee-jerks is
not a warrant for terming a case—paresis of the tabetic form. The
fact is that the lesion in paresis tends to be intraspinal, just
as the higher brain lesions tend to occur within the brain
substance. The meninges are relatively spared both within the
cranium and within the spinal canal. The characteristic
degeneration of posterior nerve roots which we find in tabes
dorsalis is not necessarily found in general paresis even when
there are somewhat extensive spinal lesions. Accordingly the
absence of sensory returns by way of the posterior nerve roots
which characterizes tabes dorsalis is not necessarily a phenomenon
of general paresis. The mechanism by which the knee-jerks are lost
depends upon histological detail. They may be lost when under
tabetic conditions the posterior roots are severely diseased and
when under paretic conditions only intraspinal collaterals or a
small portion of fibres are affected. The whole question hinges
upon where and to what degree the various reflex arcs are cut in
the disease. The tabetic phenomena are, as so commonly stated,
intradural; that is, the sensory or gangliospinal neurones at
certain levels are affected all the way in from the points at
which they pierce the dura mater. The affection of these and other
neurones in general paresis is an intraspinal and parenchymatous
affection.

=Neurosyphilis; auditory hallucinations; ideas of persecution;
attacks of excitement. SYPHILITIC PARANOIA (Kraepelin)?=

=Case 59.= Bridget Curley was a case that was discharged from the Psychopathic Hospital, recovered, after 26 days in hospital. The symptoms so resembled those of alcoholic hallucinosis that the diagnosis was made despite the fact that the patient consistently denied the use of intoxicants. There was, in fact, no proof that she drank alcohol. The case was, however, not clearly one of alcoholic hallucinosis or of any other well-defined form of mental disease. A provisional diagnosis of manic-depressive psychosis, manic phase, had, in fact, also been made.

The illness had begun with depression and inactivity, Bridget’s friends accounted for these conditions on the ground that a lover had departed for Ireland. A few days after the depression began, Bridget became dizzy and refused to give a boarder his breakfast, stating that she had lost her memory and had begun to hear bells ringing and people talking. She then became greatly excited and was brought to hospital, where the prolonged baths quieted her.

It seems that Bridget had had stomach trouble and headaches at the top of her head or sometimes in her temples. =Physical examination= showed the left pupil to be larger than the right, a slight tremor of the lips, a slight systolic murmur at the apex, slightly irregular pulse, and moderate edema of ankles. The blood serum was negative to the W. R., but lumbar puncture was executed and the fluid showed a positive W. R.

The patient was tested by the Binet and other methods, and although 35 years of age, seemed to be by the mental tests hardly over 11 years old. She was inclined to be feverish, somewhat restive, and pugnacious; rather slow of speech, sometimes refusing to answer and grimacing. Her pugnacity was, however, easily controllable, and the excitement was largely at night. This excitement subsided rapidly in the course of a few days.

1. What is the diagnosis in this case? The following diagnoses and
suggestions for diagnosis were made at the staff meetings:

Unclassified mania.
Manic-depressive psychosis, manic phase.
Toxic delirium.
Dementia praecox.
Bacterial infection of the brain.
Unclassified delirium.
Acute delirium.
Infectious psychosis.
Acute confusional psychosis.
Psychopathic personality by use of alcohol.
Mental deficiency with atypical mental state.
Syphilitic paranoia.

2. Is this a case of syphilitic paranoia? The so-called syphilitic
paranoia of Kraepelin is a rare and uncertain type of syphilitic
mental disease. Delusions and hallucinations are prominent. As a
rule, the onset is stated to be slow and insidious, or at any rate
there are a variety of indefinite prodromata. Jealousy is a
prominent feature, sometimes attended with marked sexual
excitement. Auditory hallucinations and ideas of persecution are
particularly in evidence. The most striking feature in Kraepelin’s
group was a sudden occurrence and equally sudden disappearance of
violent excitement, with or without external cause. Thus, an
excitement would be produced by a few words spoken, and
immediately after, the phase of excitement would pass and the
patient would become entirely friendly and accessible once more,
as if nothing had happened. About half of Kraepelin’s cases showed
a positive serum W. R. He does not report lumbar puncture
findings, and grounds the existence of disease upon certain
autopsied cases. The speech and writing disorder of paresis as
well as the characteristic disorientation for time and muscular
weakness of general paresis were absent in the group. It appears
that most cases of the group have hitherto been placed in dementia
praecox.

=The clinical symptoms of CHRONIC ALCOHOLISM are sometimes largely
identical with those of PARETIC NEUROSYPHILIS (“general paresis”):
differentiation by means of the laboratory findings.=

To demonstrate this proposition, the cases of Francis Murphy (60) and David Collins (61) are in point, being sharp foils to one another.

=Case 60.= A laboring man about 44 years of age was brought to the Psychopathic Hospital one summer day, in a stupor. This patient, Francis Murphy, had been at his regular work as axeman in the Park Service, when he suddenly fell in a heavy convulsion. He was carried to a general hospital, still in convulsions, and ether was administered to quiet the movements. The convulsions shortly ceased, but the patient’s consciousness failed to clear; hence his transfer to the Psychopathic Hospital.

Here he remained much disturbed and was placed in a room with a mattress on the floor. On this mattress he would crouch on all fours for a considerable time, looking fixedly downward as if at an object on the floor, unresponsive to questions but compliant with efforts to place him on his back. He gave the impression of daze and either disorientation or confusion.

Within twenty-four hours the patient became more tranquil and consciousness became clearer, but the patient was at a loss to bring to memory either recent or remote events. However, he replied to questions, giving some different story each time he was approached. Curiously enough, the patient seemed very contented and good-natured and would even laugh foolishly at times, saying that he felt fine and all ready to go out to work.

The general impression conveyed by Francis Murphy at once suggested the possibility of neurosyphilis. Convulsions, perhaps initial in middle age, with a post-convulsive stupor, followed by a partial clearing up, with persistent amnesia and a suggestion of fabrications with euphoria, bore out the suggestion.

The =physical examination= strengthened the impression of neurosyphilis. Well developed and nourished, florid, with a manual tremor and sweating of the palms, the patient was in general without physical symptoms. =Neurologically=, however, whereas the left pupil was larger than the right and reacted properly to light, the right pupil was a bit contracted, somewhat irregular, and either reacted not at all to light or very slightly so (reacting perfectly to accommodation). The knee-jerks could be obtained only with reinforcement, and several other reflexes could not be elicited (triceps, radial, ulnar, periosteal, Achilles, umbilical). Moreover, the heel-to-knee test was poorly performed; some of the common tests phrases were very poorly repeated; there was marked tremor in writing; and the paragraphia seemed to be not merely peripheral, for syllables were left out in words and ordinary words spelled incorrectly (psychographic disturbance).

We do not care here to insist that the right pupil was really an example of the Argyll-Robertson phenomenon since the slightest tinge of doubt is important if a positive diagnosis is practically equivalent to asserting syphilis. Practically, however, the right pupil was regarded as an Argyll-Robertson pupil under hospital conditions (flash-light reaction). Argyll-Robertson pupil, areflexia, speech disorder, writing disorder, memory disorder, conduct disorder, and euphoria, all with a history of convulsions, certainly warranted the tentative diagnosis of neurosyphilis.

As usual, resort was made to the W. R. in the serum and in the spinal fluid. One of the first results to come through from the laboratory was the absence of globulin, normal albumin, negative gold sol reaction, and a cell count of two cells per cmm. in the spinal fluid. Later the _W. R.’s_ were returned _negative_ for blood and spinal fluid.

In the meantime, an illuminating change had occurred in the patient, for two days later,—three days after the first convulsion in the park,—the patient had apparently quite recovered; his consciousness became nearly clear; he could remember every event up to the time of the convulsion, and his memory came back in appropriate degree for both remote and recent events.

The patient, it appeared, had for some time been drinking more and more heavily. In recent days, he had been taking five or six whiskeys and a half dozen beers daily on the average, and often much more. About ten years before, the patient narrated, there had been a convulsion at a ballgame, and this convulsion the patient himself called a “rum fit.”

Here, then, is a case of ALCOHOLIC PSEUDOPARESIS. Without the W. serum test and without the spinal fluid examination, it is probable that the diagnosis of general paresis might have clung to the patient for some time on account of the apparent Argyll-Robertson pupil, which had to be accepted as such on the flash-light data. In point of fact, in this case the pupil later reacted more normally to light, and the speech and writing disorders measurably cleared up.

1. Can alcohol produce the Argyll-Robertson pupil? The majority of
neurologists would today answer, Yes.

2. If in the case of Francis Murphy, the W. R. in the blood had
happened to be positive on account of a non-neural syphilitic
infection (spinal fluid negative), would the diagnosis _general
paresis_ be warranted? Probably the diagnosis _general paresis_
would have been made. If the patient had been lost to observation,
he might well have been regarded as an atypical paretic with
prodromal convulsions.

3. Would positive globulin and excess albumin in the spinal fluid
alone or in association with a positive serum W. R. warrant the
diagnosis _general paresis_ or _neurosyphilis_? The chances are
that most neurologists would advocate proceeding to treatment in
any case of positive serum reaction, whether or not there was
globulin or excess albumin; but the positive globulin and excess
albumin would probably not warrant the diagnosis _general paresis_
or _neurosyphilis_ in the absence of excess cells and the
characteristic gold sol reaction and W. R. in the fluid.

4. Is the case of Francis Murphy one of alcoholic epilepsy (as
suggested by Murphy’s own phrase, “rum fits”)? It must be
remembered that epileptics become alcoholic and that epileptic
convulsions increase or become more severe with alcoholism. On the
other hand, the literature indicates that alcoholism can produce
convulsions, as can many other factors. The literature also
indicates that there is a condition of epilepsy in which the
convulsive tendency sets in as a result of alcoholism in a patient
not previously disposed to epilepsy; it appears also that
sometimes, though very rarely, the epilepsy continues after
withdrawal of alcohol, and even after giving up the habit. Francis
Murphy appears to have had but two spells of convulsions, both of
them following heavy bouts with alcohol. There is so far, then, no
warrant for calling Francis Murphy’s case one of alcoholic
epilepsy.

5. Does the use of alcohol by a subject destroy the value of the
W.R.? It has been held by some that alcoholism interferes with the
accuracy of the W. R. This has not been our experience and for the
present we are of opinion that the results have the same value in
alcoholics as in non-alcoholics. The next case (Collins, 61) is
one in which a positive W. R. occurred in an alcoholic. When
dealing with paretic neurosyphilis it is especially true that the
W. R. is disturbed very rarely, if at all, by toxins or drugs,
except antisyphilitic drugs.

=Alcoholism may cloud the diagnosis of NEUROSYPHILIS.
Differentiation by laboratory tests.=

=Case 61.= David Collins was a steamfitter of about 43 years of age, picked up at 6.45 a.m. in the midst of convulsions and talking incoherently, in a state apparently of fairly clear consciousness. On arrival at the hospital, the patient was able to tell how he had always been a hard drinker, and how during the past week of unemployment he had taken large quantities of poor whiskey,—perhaps an average of a pint a day. Collins also told how he had had delirium tremens several times, but he said the present spell was quite unlike delirium tremens. There was no disorientation or impairment of memory, and the patient did not in any wise suggest a mental case a few hours after admission.

It appears, according to Collins, that he had obtained some work the night before, and had quit work about 6.30, whereupon he stepped into a barroom, took one drink of whiskey, left the barroom, walked down the street, and suddenly lost track of the world, coming to consciousness in a carriage with two policemen, but remaining, as he said, “dopy,” inattentive, and confused. After a meal, however, the patient began to feel better and soon felt quite all right.

The =physical examination= was quite negative except that =neurologically= there was lingual and manual tremor, a speech defect, apparent only with test phrases, unsteadiness of handwriting, left knee-jerk greater than right, a left-sided Babinski reflex, and a difficulty in executing rapid successive movements (dysdiadochokinesis). This degree of neurological disorder in our experience warrants lumbar puncture as well as a serum test. The lumbar puncture shortly disclosed a positive globulin and excess albumin, and the returns from the W. R.’s were positive for both spinal fluid and blood serum. The data of the gold sol reaction were not available on account of technical difficulties. However, it appears that the diagnosis of neurosyphilis could hardly be avoided in this case.

David Collins differs from Francis Murphy, then, in showing a positive blood and spinal fluid reaction for syphilis as well as a positive globulin and excess albumin. As above remarked, it is probable that the positive globulin and excess albumin would not warrant more than a suspicion of neurosyphilis taken by themselves.

Unfortunately, we were unable to persuade the patient to submit to treatment, and from the patient’s point of view possibly his decision, not to submit to treatment, was a good one since he has had no symptoms of any sort for a period of 18 months since his episode. However, as abundantly elsewhere demonstrated, we feel that the patient is wrong, and that the physicians are right in urging treatment.

1. Is not the convulsive episode an alcoholic phenomenon in David
Collins entirely separate from the patient’s general and
neurosyphilis? Possibly; however, an outbreak of neurological
symptoms with spontaneous recovery is not only consistent with the
diagnosis of syphilis, but somewhat characteristic of
neurosyphilis. We suspect that another attack will occur in David
Collins.[12] We shall from time to time make use of the social
service to suggest his going under treatment, and shall employ his
record of contact with a public institution to drive in our
suggestion. Still it is clear that there are numerous cases in the
community that are not accessible to social service initiated from
a public institution. Accordingly, educational propaganda is
necessary for salvage of the middle- and upper-class victims of
syphilis. It is a little unfortunate that the ethics of the
private practitioner make such salvage of middle- and upper-class
persons not very likely. Might it not be that an extension of
state medicine to this field would incidentally increase the
amount of successful private practice?

2. What may be the cause of such a convulsive episode as that of
David Collins? It would appear that the convulsions of general
paresis and of neurosyphilis in general often occur without gross
structural lesions of the brain. It may be suggested that vascular
irritation or parenchymal irritation by spirochetes, acting in
appropriate parts of the central nervous system, can produce such
convulsions.

3. What is the significance of the unilateral phenomenon in David
Collins (left knee-jerk greater than right; left-sided Babinski)?
The current explanation of hyperreflexia is that somehow
inhibitory impulses from upper portions of the nervous system have
ceased to influence the local arcs that mechanize reactions like
the knee-jerk and the normal plantar reflex. The phenomena are
commonly found in cases with pyramidal tract disorder, and in the
case of David Collins one may suspect, therefore, that there was a
central disorder affecting the right pyramidal tract above its
decussation. One might suspect that the convulsions were initiated
by a lesion (whether gross or microscopic in range) in the right
side of the cerebrum; but whether in the white matter or in the
gray matter must be left doubtful. The clearing up of all symptoms
suggests either that the lesion was microscopic in range or that
the phenomena were transient and functional.

4. Can the dysdiadochokinesis be used to indicate cerebellar lesion
in David Collins? Possibly; but it does not appear that the
difficulty in executing successive movements was unilateral. It
seems impossible to bring into close topographical relation the
basis for the Babinski and left-sided hyperreflexia, and the basis
for the dysdiadochokinesis. Alcohol is sometimes asserted to exert
an especial effect upon the cerebellum.

5. Must we suppose structural lesions, either (a) of the nature of
cell losses demonstrable microscopically, or (b) of the nature of
secondary degenerations demonstrable by Weigert myelin sheath
methods, in the case of David Collins? It appears that we do not
need to assert the existence of such lesions.

6. Could the hyperreflexia and the Babinski reaction be due to local
spinal cord disease? Possibly; but the existence of other
neurological symptoms (lingual and manual tremor, speech defect to
test phrases, ataxic handwriting, and dysdiadochokinesis) makes it
probable that there were lesions, or at any rate disordered
functions, within the cranium; and there appears to be no basis
for asserting local spinal cord disease.

=Differential diagnosis between NEUROSYPHILIS and ACUTE ALCOHOLIC
PSYCHOSIS.=

=Case 62.= Joseph Buck was a chef of 60 years who came in, seeking advice because his memory was getting poor; he was unable to remember names and what he was about to do. He was tremulous and had much pain in his limbs. He had been drinking heavily for weeks,—probably ten weeks; in fact, he described himself as having had “the shakes” and as having lately seen animals and people that were unreal. He had had the shakes before and the condition had lasted for two to three days after alcohol was discontinued.

=Physically=, Buck was tall, well developed, although poorly nourished, with a skin suggesting alcoholism. There was a slight acne over the back and chest; there was a slight enlargement of the heart, with blood pressure, systolic, 180, diastolic, 120. There was a corneal opacity of the left eye, which the patient said was the result of syphilis following a chancre, which he had acquired at the age of 27. There was also a ptosis of the upper lid of the left eye. The right pupil was irregular and reacted to light sluggishly, and with a very small excursion. The patient was slightly deaf in both ears. The deep reflexes were all lively and equal. The tremor was most marked in finely coördinated movements. There was a slight swaying in the Romberg position but the sign could not be said to be present. The gait was unsteady. There was a marked tenderness over the nerve trunks.

So far as =mental examination= went, it seemed that the patient’s claim of amnesia was subjective. There was certainly no more amnesia than a slight difficulty in recalling details. The diagnosis of alcoholism with convalescence from delirium tremens would certainly seem to have been sufficient for the phenomena, and the suggestion of alcoholic neuritis only confirmed the picture. To be sure, one might expect a diminution or absence of deep reflexes; still, these reflexes may be overactive in an irritative stage of the disease.

Naturally, however, the history of syphilis and the pupillary phenomena and ptosis, made the consideration of neurosyphilis necessary. Both serum and fluid W. R.’s proved positive; there was an excessive amount of albumin and globulin, the gold sol reaction was typically “paretic,” and there were 377 cells per cmm.

The patient improved upon a rest treatment and was given injections of mercury for his syphilis. After a few months he felt well enough to return to work, and continued at work throughout a season, receiving mercurial treatment throughout this time. A spinal fluid examination fifteen months later showed a weaker gold sol reaction, reduction in the amount of globulin and albumin, and but 26 cells to the cmm. The W. R.’s had remained positive.

1. What are the forms of syphilitic neuritis? According to Nonne,
syphilitic neuritis and polyneuritis have at last acquired
standing in neuropathology. The older claims depended upon
findings on palpation and recovery after antisyphilitic treatment.
Since the introduction of salvarsan, cases of ophthalmoplegia,
facial, acoustic, and optic nerve disease, as well as neuritis of
the extremities, have been reported in large numbers. These
phenomena are to be regarded as neurorecidives in the modern sense
of that term. The neurorecidive is not a salvarsan effect, but is
an effect of the syphilitic process itself, settling in the
peripheral nerves. Paresthesias are especially prominent in
peripheral mono- or polyneuritis, and this point is of some value
in differentiating the syphilitic peripheral neuritis from root
neuritis. Root neuritis is more often characterized by neuralgic
attacks. Objective hyperæsthesia of neuromuscular origin is also
found in these cases, demonstrated by pressure on the nerves. The
motor phenomena consist in a flaccid paresis or paralysis,
especially affecting the radial, ulnar, and peroneal nerves. Nonne
states that it is rare for syphilis to affect a single nerve
region, and he regards cases in which a single region alone is
affected as usually due to a local gummatous process.

2. What is the significance of 377 cells per cmm.? See discussion of
Washington (Case 66).

=Differential diagnosis between NEUROSYPHILIS and CHRONIC
ALCOHOLISM.=

=Case 63.= Albert Fielding, 46, was an insurance broker, who was brought to the hospital for excessive alcoholism. Indeed, he showed all the signs, both of chronic and acute intoxication, except that there was no nerve trunk tenderness. Fielding was very loquacious though his speech was rather thick. He showed tremor of hands and an alcoholic skin. Physical and neurological examination proved entirely negative.

Fielding claimed that he had had a nervous breakdown at about 36 years of age, after disappointment in love. He had the drinking habit and began to drink more and more. He had now become nervous and tremulous and had to drink in order to brace himself. After a few days, the patient began to be much better, having recovered from acute alcoholism. =Mental examination= now showed good memory with orientation intact. There was a certain tendency to reminiscence and to somewhat childish actions. He had attempted to stop drinking but had been unable to quit. As a matter of fact, his mother and father had been excessive drinkers and he had inherited the tendency, etc.

The =diagnosis= seemed to be plain. The routine W. R. upon the blood serum was negative. However, the patient had remarked during the history taking, that he had had a chancre and secondary symptoms of syphilis. Accordingly, lumbar puncture was resorted to. The fluid showed a slightly positive W. R.; the gold sol reaction was of the syphilitic type; there was a considerable increase in albumin and globulin, and there were 20 cells per cmm. The diagnosis of neurosyphilis seemed clear.

=Course=: The patient received six months’ treatment in a sanatorium but the symptoms remained almost as before, and the patient showed the same childishness and inability to take care of himself. Since the symptoms continued six months after the withdrawal of alcohol, it might well be suspected that the condition was more than a merely alcoholic one. However, in a number of purely alcoholic cases, such long-standing effects are found: even as long as six months or longer after the withdrawal of the alcohol, and one might conclude therefore that Fielding was actually a victim of alcoholic dementia. The spinal fluid after these six months (during which period antisyphilitic treatment was given) showed no change, and the prognosis was offered that the case would probably develop into one of paresis.

A year later, after six months sanatorial care and six months life in the community, the patient returned to the Psychopathic Hospital in an alcoholic condition. The lumbar puncture showed all signs negative except the W. R. which was slightly positive. The W. R. of the blood was negative.

In connection with this case, see the case of _paresis sine paresi_ (25).

1. What is the relation of the syphilitic and alcoholic process in
Robert Fielding? One does not like to break the so-called rule of
parsimony in diagnosis, but it would seem that the effects in
Fielding are the combined effects of syphilis and alcoholism.