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.
[Illustration:
1. Exudate in pia mater—mononucleosis.
]
[Illustration:
2. Superficial (subpial) cellular reaction of neuroglia tissue
(expanded cell bodies).
]
[Illustration:
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.
[Illustration:
4. Arteritis of pia mater.
]
[Illustration:
5. Focal vascular lesions.
Case 57. Seven months from infection. “Disseminated syphilitic
encephalitis,” Barrett. (Photographs by Barrett.)
]
[Illustration:
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.
=Differential diagnosis between NEUROSYPHILIS, DIABETIC
PSEUDOPARESIS and BRAIN TUMOR.=
=Case 64.= A large and imposing person, Calvin Hall, 55, had been
employed as a doorkeeper and guard, in which position he was on duty for
12 to 14 hours daily. Eventually, however, he had begun to have a good
deal of pain in the legs and a few months before observation, one day,
his legs gave way and he fell to the floor. There was, however, no loss
of consciousness, and he was carried to a general hospital. The result
of an examination there was that his family was informed that he had
some nervous trouble.
Hall now began to be melancholy and wept a good deal. His appetite and
sleep remained intact. He felt too weak to walk. At the end of about a
year, he began to improve and again became able to do a little light
work. About a month before coming to the Psychopathic Hospital, about
two years after the onset of symptoms, Hall suddenly began to talk
excessively, in a rambling and rather senseless way. A fortnight later,
he began to suffer from insomnia and restlessness.
Some medical facts were available: It seems that at 25 years this
patient had become infected with syphilis though there had never been
any secondary signs. He was married four years later but there had not
been any children. Moreover, for four years past, the patient had been
treated for glycosuria.
Upon admission, the patient’s sensorium was clear, but his orientation
was only partial. He could give a fair account of his life, but it
appeared that his memory was somewhat impaired. There were auditory
hallucinations (voices of relatives). He often mistook the identity of
persons about him. He talked in a grandiose fashion of his great
strength and especially of a God-given power to read minds. His flow of
thought was rapid, rambling, circumstantial, and with traces of
irrelevance. He was rather continuously busy and at times restive. There
was a good deal of emotional agitation and apprehensiveness, and again
the patient would become suspicious and tearful.
=Physically=, there was a discharging sinus connected with the right
humerus, close to the elbow. The pupils, though equal and regular, were
sluggish in reaction to light. The knee-jerks and ankle-jerks were
absent. There was no Romberg sign but there was some swaying in the
Romberg position. There was a moderate ataxia in walking. Glycosuria to
a moderate degree was determined. There were no casts or albumin in the
urine. The W. R. of the blood and of the spinal fluid was negative. The
albumin of the fluid, however, was considerably increased. X-ray
examination of the skull yielded a suggestion of absorption of the
posterior clinoid processes of the sella turcica. The X-ray examination
of the arm in the region of the sinus showed a chronic osteomyelitis,
possibly syphilitic (or diabetic?).
The diagnostic problems in the case of Calvin Hall are extremely
intricate. There are clinical suggestions of general paresis, not
confirmed by the laboratory findings.
1. Are we dealing with a case of diabetic pseudoparesis? Is the pain
in the legs of like origin, and has a neuritic process led to the
absence of the knee-jerks? The Allen treatment appears to have had
no beneficial result in this case.
2. Is there a tumor of the sella region, which could account for the
mental symptoms and the glycosuria? The spinal fluid albumin might
be regarded as consistent with a variety of psychoses, including
that of brain tumor. We have to remember the definite history of
infection, the sterile marriage and the possibly syphilitic
osteomyelitis.
=DIABETES AND NEUROSYPHILIS, relations?=
=Case 65.= Donald Barrie, a man of 61, diabetic for several years, had
begun to worry about the diabetes, feeling that he was about to die, and
had gone so far as to make several threats of suicide. Hence he was
brought to the Psychopathic Hospital for observation.
Barrie was rather well developed and nourished, although he looked far
older than he was. There was a marked arcus senilis; the skin was dry
and rough; the radial and other accessible vessels were markedly
sclerosed; abdomen obese; right testicle very low with thickened and
hard epididymis.
=Neurologically= there was little abnormal to discover. The pupils were
irregular; both reacted fairly well to light. There was a slight tremor
of the extended hands, and still less of the tongue. The voice was
slightly thick and the patient stumbled somewhat on test phrases. Urine:
specific gravity, 1029; sugar; no acetone; no diacetic acid. Sugar 2 to
11 grams for 24 hours on ordinary diet. It proved impossible to get the
patient sugar-free, either by cutting down the carbohydrates or by using
the Allen method.
=Mentally=, the depression with reiteration of wrong-doing and
self-accusation because of the contraction of syphilis, were the
striking features. There was, to be sure, a slight imperfection of
memory for remote events; memory for recent events and knowledge of
current events was very poor. Barrie claimed that his condition was
entirely hopeless, that his memory was exceedingly bad, and that he was
no longer capable of supporting his family.
1. What shall be said as to diagnosis in a man of 61 with
glycosuria, depression, amnesia, sluggish pupil, slight tremor,
slight speech defect, and a history of syphilis? The W. R. of the
serum proved positive, and also the W. R. of the spinal fluid. The
gold sol reaction of the fluid was of the syphilitic type. There
were 112 cells per cmm., there was an excess of albumin, and a
large amount of globulin. Accordingly, the diagnosis of PARETIC
NEUROSYPHILIS (“general paresis”), especially in view of the
laboratory findings, seems necessary.
2. What is the cause of the glycosuria? First: possibly it has no
relation with the syphilis; secondly: it may possibly be due to a
syphilitic involvement of the pancreas; thirdly: it is barely
possible that it is due to syphilitic disease of the fourth
ventricle or of the base of the brain, involving the pituitary
region. Perhaps our case is too complex for analysis. At all
events, the case brings up the possibility of a syphilitic
glycosuria.
3. Can the diabetes in the case of Barrie be explained as
syphilitic? Warthin of Ann Arbor has recently described somewhat
remarkable spirochete findings in his autopsy material. The order
of organic infection according to frequency is: aorta, heart,
testis, adrenal, pancreas, nervous system, liver, and spleen.
Warthin has called attention to the relation of pancreatitis and
spirochetosis to diabetes in a recent review[13] of findings in 41
autopsied cases from the University Hospital in Michigan. Warthin
found active luetic lesions in the pancreas in 6 cases.
=Hemianopsia in a case of neurosyphilis.=
=Case 66.= Lawrence Washington, a colored cabman, 58 years of age, began
to forget addresses given him by his fares. Moreover, he could no longer
see as well as before, especially on looking toward the right side. He
himself states that the visual trouble dated back as long ago as his
39th year, at which time he had a terrific pain in both temples, leading
back from the eyes. Washington thought that his vision had been getting
slowly but steadily worse ever since.
We got the impression that the amnesia claimed by Washington was more or
less subjective and he was found to be well informed. This association
of amnesia and impairment of vision naturally suggests syphilis. The
patient himself stated that he had had a chancre at the age of 18.
We found the W. R. of the serum to be appropriately positive. The W. R.
of the spinal fluid was also positive though weakly so. There was an
excess of albumin; globulin appeared in large amount; the gold sol
reaction was of the syphilitic type; there were 186 cells in the spinal
fluid.
Is this case one of paresis or of some other form of cerebrospinal
syphilis? Let us consider the data of the =physical examination=. On the
whole, the patient was well preserved. There was a slight radial
arteriosclerosis, but on the whole the cardiovascular system was almost
negative. The blood pressure was 100 systolic, 65 diastolic.
=Neurologically= the visual field of the left eye was somewhat limited,
and there was a temporal hemianopsia of the right eye. The
ophthalmoscopic examination showed a disseminated choroiditis on both
sides. The right pupil failed to react to light. The left pupil reacted
slowly. Both pupils reacted properly to accommodation.
The knee-jerks could be obtained only on reinforcement, and when
obtained, the right was apparently more active than the left. The left
Achilles was absent; the right present. There were no other abnormal
reflexes.
The motility of the facial muscles was somewhat impaired.
Finger-to-finger and finger-to-nose tests were rather poorly done. The
muscle sense was good; there was no swaying in Romberg position; and
there was no speech defect.
We are unable to decide whether the case is one of the =parenchymatous=
type (paretic) or of the =meningovascular= type of =neurosyphilis=. It
is certainly rather unusual to find hemianopsia in a paretic.
We have been unable to get definite results from the treatment of this
case, since the patient would not return for months after getting an
injection or two of salvarsan, on the ground that he was improved enough
and did not require further treatment.
1. What conclusion can be drawn from the 186 cells per cmm. in the
spinal fluid? Ordinarily this finding would indicate an active
process. Some writers have claimed that a cell count running above
100 per cmm. was an indicator of diffuse non-paretic
neurosyphilis. It does not appear that this claim has been
substantiated. It is remarkable that this case shows an interval
of 40 years between infection and the occurrence of definite
clinical symptoms. With respect to the cell count, both in
untreated and in treated cases, the following conclusions from a
recent article (Solomon and Koefod)[14] are in point:
1. The number of cells found in the fluid of untreated cases
offers no definite information of prognostic value.
2. One is not justified in drawing any conclusions as to whether
the case is cerebrospinal syphilis or general paresis, nor the
time the process has been active, nor the severity of it, from the
cell count.
3. The cell count may vary greatly from month to month, or when
the interval is but several days, while at other times it may
remain very nearly the same after an interval of months.
4. Cases showing natural remissions may show no reduction in the
cell count, or other spinal fluid findings.
5. Cases treated with salvarsan, either intraspinously or
intravenously, tend to show a more or less rapid fall in the cell
count. This count will, as a rule, remain low during treatment,
but is likely to rise when treatment has been discontinued, but
may rise during treatment after having first fallen.
6. Cases may show remissions during treatment and still have a
pleocytosis.
7. Treated cases having the cell count fall to normal may at the
same time become very much worse and develop more marked paralytic
symptoms.
8. In general paresis the cell count in no way parallels the other
spinal fluid findings.
9. In cases in which the other tests show an improvement, for
instance cerebrospinal syphilis, the cell count also readily and
early drops to normal. At times it may drop to normal before other
spinal fluid tests become negative; again it may be last to reach
normal.
10. The change in cell count seen in syphilitic disease untreated
is also found in non-syphilitic diseases, as brain tumor.
11. The cell count offers nothing of prognostic importance in
syphilis of the nervous system unless accompanied by improvement
of the other laboratory signs.
12. The cell count is not an index to the predominance of
irritative or degenerative changes.
=Case of CEREBRAL MALARIA and SYPHILIS: simulation of PARETIC
NEUROSYPHILIS (“general paresis”).=
=Case 67.= Joseph Temple, 45, who had been a sea-going steamboat
steward, was brought to the hospital in a semi-stupor. He was entirely
uncoöperative, often resistive, attempting to bite the physician’s
fingers, and for the most part lying curled up. He was incontinent and
tube-fed. This phase, it seems, had begun the night before entrance to
the hospital. Twenty-four hours later, an extraordinary change was
noted. Temple became alert and attended to his wants, began to eat well,
and began to behave as normally as probably he ever behaved.
He was now able to give a coherent history. It was now January. In the
previous September, he had left for Mexico; he was returning when he
suddenly fell to the deck, unconscious. After this fall, he had not been
well, having had chills and fever. At the Marine Hospital, he had been
diagnosed as suffering from malaria, and was given quinine. He had been
delirious a short time in the hospital, not being able to recognize his
wife, who called. He shortly improved so that his wife was able to take
him home. Nevertheless, headache, gastric distress, and intermittent
vomiting continued. A spell of confusion took place, two days before
admission. The patient tossed about, moaned, and failed to recognize
anyone. Malaria of the æstivo-autumnal type was demonstrated in the
hospital. The temperature always remained at normal. He was somewhat
emaciated and pale. The pupils were small, somewhat unequal, and reacted
though poorly to light and distance. The tendon reflexes were lively.
The W. R. of the serum was positive, and information from the patient’s
physician runs to the effect that there was a syphilitic infection some
seven or eight years ago, followed by secondary symptoms, but the
patient had refused to take any protracted treatment. The spinal fluid
examination was practically negative.
=Mentally=, the patient was euphoric, expansive, boastful, and showed a
marked emotional instability and considerable memory defect.
1. Can the diagnosis of general paresis be made in Joseph Temple?
Certainly the acute confusion and the syncope are consistent
enough with the diagnosis, yet the severe malaria makes it seem
likely that the phenomena were due to a cerebral attack of
malaria, and such occurrences are found in the æstivo-autumnal
form of malaria. Yet malaria would hardly explain the euphoria,
memory defect, and the pupillary findings, to say nothing of the
irritability and the active tendon reflexes. Even if we regard the
active tendon reflexes and the irritability as malarial, the other
phenomena remain outstanding as exceedingly suspicious of paresis.
On the other hand, if we try to support forcibly the diagnosis of
general paresis, we are hardly able to explain the negative
findings in the spinal fluid.
In point of fact, a study of the patient’s past life revealed a
story that the mental traits of euphoria, irritability, and memory
defect had been characteristic of the patient for many years. In
fact, there is some question whether the patient is not really to
be regarded as a moron of high grade.
Upon this basis, if we regard the confusional phenomena as
malarial and the persistent mental phenomena as characteristic of
a moron and somewhat exaggerated by the disease, we have merely to
explain the suggestive pupils. As to these, it must be remembered
that though they reacted poorly to light, still they reacted
somewhat, so it is not a question of explaining an
Argyll-Robertson pupil, but only an impaired pupillary reaction.
Of course, some workers are of the opinion that pupillary changes,
perhaps even the Argyll-Robertson pupils, may occur in syphilitic
cases that are not neurosyphilitic, or at all events are not
victims of central neurosyphilis. Finally, we must remember that
there are cases of neurosyphilis of a vascular type which yield
negative spinal fluids. The case leaves many questions unanswered.
=Can paretic and non-paretic neurosyphilis be differentiated by
means of the gold sol reaction? The gold sol reaction in this case
was an extremely mild one and would not at all have warranted the
diagnosis GENERAL PARESIS, yet the discovery of a heavy meningeal
exudate including an unusually heavy deposit of plasma cells even in
the spinal pia mater will perhaps warrant us in making a final
retrospective diagnosis of paretic neurosyphilis. Autopsy.=
=Case 68.= We would like to give the full effect of our surprise at the
outcome of the case of Margaret O’Brien, a school-teacher, 26 years of
age. To be sure, Miss O’Brien developed symptoms at 22 or 23 which we
can now explain consistently with the outcome of the case; for at that
time, she began to complain of severe pain in the head, especially in
the forehead and temples, and also became nervous, unable to remain
quiet, and given to insomnia. She was markedly depressed at the time and
would refuse to talk at times. However, only the headache in this
prodromal period could be regarded as particularly suggestive of
syphilis, and headache in an over-worked school-teacher is not uncommon.
In fact, the picture presented by the patient was one of catatonic
dementia praecox. The patient was admitted to the hospital after a
sudden onset of excitement. At first she was very restless, continually
looking about and getting up and walking away from the examiner, giving
the impression of understanding all questions but preserving an air of
indifference. A few days later, the patient was gotten to answer more
coöperatively. She remarked that the hospital was heaven although in
Boston; that it was summer time (correct) and that her memory was
greatly impaired. The physician was a messenger of God (delusion later
corrected). The patient had not done God’s will; her breath was leaving
her; God’s voice was heard from time to time, and Miss O’Brien had heard
it for a long time. God tells her to do His will. However, as Miss
O’Brien remarked, “I must think all this nonsense, turning against God.”
The patient frequently attitudinized and would remain in an apparently
catatonic condition for many minutes. For the most part, she was
resistive and mute and non-coöperative as to examination. From time to
time, she made impulsive suicidal attempts. So far as a somewhat
inadequate =physical examination= was concerned, nothing abnormal could
be made out; in particular, the pupils reacted normally to light and
were otherwise normal. The routine W. R. of the blood serum, however,
returned positive, and in accordance with the policy of the Psychopathic
Hospital, the patient was subjected to a lumbar puncture. The lumbar
puncture yielded a positive W. R., 109 cells per cmm., a positive
globulin and a considerable excess of albumin, and an exceedingly mild
gold reaction—syphilitic type.
Ten days after admission, the patient had a convulsion. She never
regained consciousness, continued to have convulsions for a few hours,
and died, apparently from paralysis of respiration. The heart continued
to beat for a short period after respiration ceased. The =autopsy= was
consistent with the diagnosis which had been rendered after the
surprising results of the W. R. in the blood and the laboratory findings
in the spinal fluid had been learned. There was a generalized
encephalitis with congestion of all the smaller cerebral vessels and
petechial areas in the meninges and upon the cortical surfaces. We
regard the case as one of syphilitic encephalitis.
The brain weighed 1265 grams, indicating a loss of 79 grams by Tigges’
formula (8 times the body length in centimetres). The pia mater was, in
the gross, quite normal within the cranium; nor were any cells found in
a smear from this pia mater; but the pia mater over the spinal cord was
visibly edematous, and a smear from the spinal pia mater showed great
numbers of lymphocytes and especially of plasma cells—a finding which
was confirmed in stained section, by which a remarkable display of
plasma cells was found plastered somewhat generally over the entire pia
mater of certain segments. The brain substance was softer than normal,
but displayed no differences of consistence. The stripping of the pia
mater of the temporal lobes on both sides yielded the so-called
“decortication” (that is, the adhesion of small bits of brain substance
to the pia mater). The optic nerves were somewhat thinner than normal.
No other gross lesions of the brain were found.
The dura mater, although dense and injected, was not otherwise abnormal.
There was an early visible sclerosis of the middle meningeal arteries,
more marked on the left side.
The cause of death, so far as the autopsy revealed it, was bronchial
pneumonia. There was a diffuse nephritis.
1. Are the hallucinations in the case of O’Brien characteristic?
Hallucinations are regarded as playing a minor rôle in general
paresis. In fact, earlier workers sometimes denied that
hallucinations occurred at all, and this denial has been made once
more of late by Plaut,[15] but Kraepelin quotes Obersteiner as
observing hallucinations in 10%, and regards that figure as
approximately corresponding with his own experience. Junius and
Arndt are cited as finding 17% of their cases hallucinated.
Auditory hallucinations are somewhat more frequent than those of
vision (alcoholic psychosis must be considered). The visual
hallucinations of paresis are thought by Kraepelin to be related
with atrophy of the optic nerves, and he states that they occur by
preference in patients having such atrophy. Hallucinations though
not common are more frequent in non-paretic neurosyphilis than in
paretic neurosyphilis.
2. What was the cause of death in Margaret O’Brien? The autopsy, as
above stated, indicated pneumonia. In point of fact, this patient
developed convulsions and ceased respiration, the heart continuing
to beat for some time after respiration had ceased. It may be that
the death should be counted as one of neurosyphilitic seizure.
=Tonsillar abscess associated with neurosyphilis (Lues Maligna?).=
=Case 69.= Frank Mason, 49 years, a rectifier of spirits, was admitted
to the Psychopathic Hospital in a tremulous, mentally confused,
depressed, and unhappy state. He was particularly concerned because he
could not give an accurate account of his past life and because he found
that he was continually contradicting himself.
Superficial examination shortly discovered the pupils to be much
contracted, irregular, and non-reactive either to light or distance.
Although these pupils showed more than the Argyll-Robertson phenomenon,
yet the suspicion of syphilis was important.
Throat examination showed a large area of ulceration involving the whole
of the right tonsil and extending even to the left side of the median
line so that the whole of the faucial pillar was involved. In the midst
of this ulcerative area was a mass of purulent necrotic tissue, about
which the edges of the ulcer stood out sharply. There was, however, very
little acute reaction about the margin of the area.
The association of pupillary changes (especially stiffness to light),
what looked like tonsillar gumma, and mental disorder (including memory
disturbance) heightened the impression of syphilis.
However, the remainder of the examination was not especially
confirmatory of the diagnosis. The man was well developed and obese,
with a slightly enlarged heart, with sounds of poor quality and the
aortic second sound accentuated. The systolic blood pressure was 130;
the diastolic, 90. There was no disorder of reflexes except that the arm
reflexes were very lively.
After a time, a few facts concerning the patient’s life became
available. Although a rectifier of spirits, Mason could not be found to
have over-indulged in alcohol. It appears that some five months before
his admission to the hospital, a wisdom tooth had been extracted. About
four months before admission, the ulceration of the faucial pillar had
begun, and this ulceration was immediately laid to infection from the
wisdom tooth cavity. Mason then had to discontinue work and a depression
followed. But the account of this depression led us to think that he was
a victim more of natural sadness than psychopathic depression. There was
much worry and insomnia. To meet the insomnia, large amounts of
hypnotics were administered. The sequence of these hypnotics was a
tremendous disturbance and continual crying out by the patient. In fact,
Mason became so excited that he was removed to the Psychopathic Hospital
for temporary care in the condition above mentioned.
We naturally awaited the outcome of the serum W. R. The return was
negative. However, the typical position of the ulcerative lesion and the
non-reacting pupils,—to say nothing of the mental symptoms and the
associated tremors, with incoördination (this incoördination was
non-characteristic and apparently due largely to the tremor),—led to
lumbar puncture.
The spinal fluid yielded a weakly positive W. R. There was a slight
positive albumin, the globulin test was slightly positive, there were 14
cells per cmm., and the gold sol reaction was of the syphilitic type. We
were, then, probably entitled to conclude that syphilis was active not
only in the body at large but also in the nervous system. Looking back
upon the case, we considered that large doses of morphine and hyoscyamus
might well have produced the marked mental confusion and possibly the
tremors that characterized Mason on his arrival at the hospital.
Improvement followed after a few days of rest; the confusion disappeared
and the tremors diminished; the pupils returned to their normal size and
reaction; depression persisted, and the patient was very properly much
concerned about the tonsillar lesion. However, further improvement did
not take place under antisyphilitic treatment and patient died after
several weeks from what was believed to be an embolus from the tonsil.
1. What was the true interpretation of Frank Mason’s pupillary
changes? They were probably due to the opiates, despite the fact
that, taken in association with the gummatous lesion of the
faucial pillar, we had regarded them as possibly syphilitic.
2. How shall the negative serum W. R. be explained? Such a reaction
is consistent with the diagnosis _gumma_. It is, however, a little
surprising that with active neurosyphilis and a relatively active
non-nervous syphilitic lesion like that in this case, the serum W.
R. should have been negative. Possibly a repetition of the test at
various times would have shown a positive serum W. R. In any
event, the fluid reaction was positive.
3. Could the tonsillar ulceration be due to dental infection? The
chances are against this on account of the interval (2 months)
between extraction of the wisdom tooth and the ulceration, which
itself seems to be of a tertiary syphilitic nature. In point of
fact, the patient admitted a syphilitic infection 21 years
previously namely, at 28 years of age. At that time he took large
quantities of mercury and potassium iodid by mouth.
4. Relation of the case of Frank Mason to the so-called _lues
maligna_? The case closely resembled the cases reported by Bly.
Frank Mason showed great destruction of tissue, toxemia, failure
to react to antisyphilitic treatment. In both of Bly’s cases, the
tonsil was the starting point of the illness; and in both cases
there was a trauma of the tonsil or peri-tonsillar structures
(tonsillectomy and application of caustic). In our case there not
only had been extraction of a wisdom tooth, but the tonsil had
been cauterized.
=Neurosyphilis versus multiple sclerosis.=
=Case 70.= Annie Kelly is a young Irish woman, 21 years of age, who was
perfectly well until three months before her admission to the
Psychopathic Hospital, when suddenly one evening she became very dizzy.
This was followed by a chill and vomiting. The next day she had a sore
throat but was able to be about and do her work. The dizziness, however,
continued and she began to feel rather queer. Gradually it became
difficult for her to walk on account of staggering.
A little later she noticed a weakness of the left side, involving face,
arm, and leg; then she began to find it difficult to talk. Finally the
right leg became weak, making walking practically impossible. All these
symptoms grew worse and the dizziness increased. At times her vision
would be blurred; there were somewhat frequent attacks of diplopia.
Finally she had to take to her bed, and at last she lost control of her
sphincters.
At no time did she suffer any pain. She was taken to a hospital, and
after a time improved somewhat; but she was told she had a brain tumor
and had better be in a large city, where she could have surgical aid if
this became necessary; consequently, she was brought from Montana to
Boston.
On admission to the hospital, the examination disclosed no important
symptoms outside of the nervous and locomotor systems. She was unable to
walk unless assisted. The pupils were large but reacted well to both
light and accommodation, were equal in size, and regular. Slight
nystagmus was present; there was no ptosis or strabismus; vision in the
left eye was poor. The other cranial nerves showed no involvement. The
tendon reflexes were all present and very lively; Babinski, Gordon, and
Oppenheim signs were present on either side. The ataxia was marked,
especially of the lower arms, and she had some difficulty in the
alignment of the fingers. The sense of position of the limbs was very
poor. There was some tremor, which was not of the intention type. The
writing showed some incoördination. The speech showed nothing abnormal.
=Mental examination= disclosed nothing of note objectively, but patient
stated she could not think so clearly as she could formerly.
The =diagnosis= would seem to lie between brain tumor,—which had been
suggested to the patient by her physician,—multiple sclerosis, and
neurosyphilis. The numerous neurological symptoms without any definite
evidence of intracranial pressure were sufficient to rule out for the
moment the consideration of brain tumor. The syndrome of multiple
sclerosis is not complete, but the race, age, and onset, with the
increasing and decreasing intensity of symptoms are very suggestive of
this diagnosis. The symptoms, of course, are all consistent with
neurosyphilis also, and while the patient denied any knowledge of
syphilitic involvement, the examination of the blood and spinal fluid
was made. The W. R. was negative in both the blood serum and spinal
fluid. Further examination of the spinal fluid showed presence of
globulin and an increase in the albumin content, 43 cells per cmm. and a
“paretic” type of gold sol reaction. With the negative W. R. of both
blood serum and spinal fluid, and with so much in favor of MULTIPLE
SCLEROSIS, this diagnosis was made.
1. What is the relation of multiple sclerosis to syphilis? There is
no definite relationship between multiple sclerosis and
syphilis,—that is, multiple sclerosis is not a syphilitic disease;
but the complete syndrome of multiple sclerosis is often given by
a syphilitic involvement of the central nervous system (see case
Lauder, 71).
2. Is the spinal fluid finding in this case consistent with multiple
sclerosis? According to Nonne, about 19% of the cases of multiple
sclerosis show globulin and pleocytosis in the spinal fluid. As a
rule, the number of cells ranges between 10 and 20 per cmm. and
the globulin is not present in large amounts. In this case, the
amount of globulin, which was given as 2+, is only a moderate
amount,—less than is usually found in cases of general paresis.
There are not very many cases of multiple sclerosis in the
literature in which a gold sol reaction has been performed, but in
the majority of those tested, the reaction is reported as mild.
However, cases of multiple sclerosis giving a typical paretic
curve have been described by a number of observers, among whom may
be mentioned Kaplan and Solomon.
3. How frequently is it necessary to make a differential diagnosis
between multiple sclerosis and neurosyphilis? Before the days of
the W. R. this differentiation was much more difficult than at
present. But we, however, still have to face a not very rare
difficulty in separating the two conditions. Syphilis is prone to
cause small localized lesions in the nervous system. The changes
in the patient’s condition, with improvements and regressions are
equally characteristic of both diseases. How closely the
symptomatology of neurosyphilis may simulate that of typical
multiple sclerosis is shown in the next case (Lauder, 71). When
the sclerotic area of multiple sclerosis occurs in appropriate
parts of the cerebrum, symptoms of mental disturbances will occur.
In its histological picture multiple sclerosis is at times highly
suggestive of syphilis, even showing mononucleosis and meningitis.
=Optic atrophy; nystagmus; spasticity; intention tremor. Diagnosis:
?=
=Case 71.= James Lauder began to lose his eyesight at 32 years, and was
shortly determined to be suffering from primary optic atrophy. In the
course of a year, he had become completely blind. No mental symptoms had
developed.
=Physically=, Lauder was in very good condition. =Neurologically=, there
was a complete optic atrophy with paralysis of the internal rectus
muscle, marked nystagmus, and absent pupillary reactions. All the tendon
reflexes were exceedingly lively, though the right arm reflexes were
more lively than the left, and the left leg reflexes more lively than
the right. There was an ankle clonus on both sides. The abdominal and
cremasteric reflexes were lively. There was a slight intention tremor.
There was, however, no ataxia and no speech defect.
=Diagnosis=: The nystagmus, optic atrophy, and the reflex disorder
suggested multiple sclerosis, although the liveliness of the superficial
reflexes, especially the abdominal reflexes, was a point somewhat
against any advanced degree of multiple sclerosis. It would appear that
the absence of pupillary reaction to accommodation is also rather
unusual in multiple sclerosis.
The serum and spinal fluid W. R.’s proved positive. There were 25 cells
per cmm., albumin was in excess, and there was a positive globulin
reaction.
1. What is the significance of optic atrophy and other optic changes
with respect to neurosyphilis? Canavan, from our laboratory, has
reported that she found that 40 of 58 unselected cases of mental
disease exhibited obvious and undeniably important changes in the
optic nerve. She found that optic nerve changes were even more
frequent than chronic spinal cord changes as detectable by the
same method (Weigert myelin sheath method); there were only 34 of
the 58 cases which showed chronic spinal cord changes. Eighteen
cases very probably syphilitic (although the clinical evidence was
not in all cases supported by the W. R.) failed to show optic
nerve changes in but three instances. The 15 syphilitic cases that
did show optic nerve changes showed them in but one eye in three
cases, in both eyes in 12 cases. Canavan incidentally demonstrated
a spirochetosis in the pial sheath of the optic nerve in a case of
neurosyphilis, possibly paretic.
2. What is the frequency of eye changes in neurosyphilis? Posey and
Spiller (“The Eye and the Nervous System,” 1906) quote Kéraval as
finding 42 instances of fundus change in 51 cases of paresis.
Clifford Allbutt found 41 cases of atrophy in 53 of paresis; other
authors have found far fewer. Optic atrophy sufficiently marked to
cause blindness is relatively rare in paresis. Compare table of
eye changes from Joffroy under Case Falvey (55).
As for optic atrophy in tabes, Posey and Spiller record statistics
as so various as to be on the whole unsatisfactory. The highest
percentages found appear to be those of Mott, 80%, and Gross, 88%.
It is evident that the standards for measuring optic atrophy must
differ very much.
=Atypical case of neurosyphilis. Picture of Huntington’s chorea.=
=Case 72.= Margaret Green, 28, was received at Danvers State Hospital in
an excited and frightened state. She was very talkative and said that
she was being bitten by snakes and serpents. She thought every one
approaching her was the devil, and sprinkled what she called “holy
water” about her for protection. It was clear that she was hallucinated.
She heard her child crying, and she saw a woman carrying it away.
After a few weeks, Mrs. Green grew quiet and more rational except for a
few spells of violence and noise; she gave the impression of a rather
pleasant and agreeable, though somewhat demented, patient. Physically,
beyond a tremor of fingers and tongue and lively knee-jerks and some
evidence of enlargement of the heart, there was nothing to be found.
Margaret Green is still in the Danvers Hospital, being now 48 years of
age. During the twenty years, she has presented,—besides the mental
picture of impairment of memory—occasional spells of confusion, a
variety of delusions based, at least in part, upon auditory and vivid
visual hallucinations, a certain irritability and psychomotor
excitement, and a picture of Huntington’s chorea. The diagnosis of
Huntington’s chorea has always been in doubt by reason of the lack of
any evidences of hereditary taint; it has, however, not been possible to
secure a properly intensive account of her relatives.
It appears that the choreic movements were first observed—in the
hospital at least—about 16 years ago. The patient has always been
decidedly mixed upon dates. From internal evidence derived from her
obviously in part erroneous statements, it may be that the chorea began
at the age of 23. It appears that she had been often termed a victim of
St. Vitus’ dance, and had had to leave her work in the mill on account
of the disease. From one source of information, it would appear that the
patient began to have what was called St. Vitus’ dance when she was 14
or 15 years of age; so far as this informant knew, no other member of
the family had had the affliction.
The first movements observed in the hospital were irregular, jerking
movements, more marked in the left arm but also occurring in the other
extremities, as well as in the face, wherein were produced peculiar
grimaces. The twitching movements would become decidedly worse during
spells of irritability. Observation in the patient’s early thirties left
the question in doubt whether the left pupil reacted to light or not. In
1904, when the patient was 36, both pupils failed to react to light
either directly or consensually. At this time, the jerky movements
continued, especially in the left hand and forearm, the tongue was
tremulous, test phrases were poorly pronounced, the knee-jerks were
exaggerated (especially the left), and both wrist-jerks were
exaggerated. The systematic examination, however, revealed no other
neurological disorder. Within a year, slight spurious ankle clonus
developed on both sides; the eyes, especially the left, gave the
appearance of developing cataracts. A slight consensual light reaction
was demonstrable on the right side, but all light reactions were absent
in the left eye.
At the age of 42, the patient was still disoriented for time, place, and
persons and subject to a deep amnesia; was tidy, tranquil, and of a
pleasant demeanor, but many of her muscles were in continual motion.
There were chewing movements and both hands and feet were rarely still.
There were no longer any spells of irritability or violence; and once
when found crying on the piazza, Mrs. Green, on being asked the reason,
replied that a gray cat had come and looked at her so hard it made her
cry. There were other crying spells at times for equally good reasons,
or for no reason.
More recently, the patient has become fairly well oriented for time and
place, and has acquired a fairly good insight into her condition and a
good memory for past events. She has had occasionally auditory
hallucinations, as of water running. In 1914, it was reported that the
pupils reacted to light, and the rest of the systematic neurological
examination was negative except that the knee-jerks were exaggerated;
and a re-examination in 1916 showed the pupils still reacted to light.
At present, the patient is disoriented for time, stating that her age is
about 25; she is no longer subject to auditory hallucinations; she has a
marked difficulty in enunciation, emphasized by the lack of teeth and in
part due to continual movements of the tongue; the movements appear to
be part of a generalized chorea.
In a systematic review of the Wassermann findings in the hospital
population, the blood of Margaret Green was examined and found to be
positive. Lumbar puncture forthwith performed showed a positive W. R. in
the fluid; there was a positive globulin and an excess of albumin; the
gold sol was characteristic of paresis; there were, however, but three
cells per cmm.
1. Are the choreiform movements related to the demonstrable syphilis
of the nervous system? Neither the fluid W. R. nor the gold sol
reaction should be regarded as necessarily an indicator of tissue
loss. The fluid W. R. is commonly thought to signify merely that
the fluid contains substances which are somehow due to the
presence of spirochetes in some region pretty closely related with
the fluid. The gold sol reaction, although well established to be
characteristic of neurosyphilis, is perhaps not so strong an
evidence of the existence of spirochetes in the region from which
fluid constituents are derived. There is no pleocytosis. However,
the positive globulin test and the excess of albumin do indicate a
certain amount of destructive process somewhere in the neural
tissues. Are we to suppose that these substances have been
continually found during the course of this disease? This question
cannot be answered with the data in hand, and we can only suspect
that these positive tests for albumin and globulin are an effect
of tissue destruction caused by neurosyphilis. It must be admitted
that the argument here is a little tenuous. The lesson is plain:
that in the present stage of our knowledge the W. R. should not be
omitted even in cases which present a fairly convincing picture of
some well-known entity. Thus, a disease, which looks like
Huntington’s chorea, as well as a disease suggestive of multiple
sclerosis, requires investigation by the methods of the
syphilographer.
2. How shall we explain the changes in pupillary reaction in this
case? They cannot yet be explained. A few observers have reported
changes in pupillary reflexes in the direction of normality. In
our experience such changes have not been noted. It cannot be too
strongly emphasized that it is very easy to make errors in judging
pupillary reaction if care is not used. For instance, if the
patient is accommodating for near vision, light will probably not
cause contraction. A frequent cause of error in testing the light
reflex arises from using a weak electric light. An electric
flash-light is much less efficient than daylight. Probably the
most satisfactory method is to take the patient to a window, ask
him to look at a distant object, shade the eye with the hand,
remove hand, and observe.
3. What is the chief triad of symptoms in Huntington’s chorea? (1)
Choreiform movements associated with (2) progressive mental
enfeeblement, (3) occurring in a patient whose family history
shows a similar condition in a preceding generation.
=Differential diagnosis between NEUROSYPHILIS and SENILE
ARTERIOSCLEROTIC PSYCHOSIS.=
=Case 73.= Marcus Chatterton was a retired sea captain, 75 years of age.
At the age of 71, he had had a seizure with a slight right hemiplegia
and inability to talk. He had been slightly confused for a short time
but had rapidly recovered. During the intervening four years, there had
been three similar attacks, and the last one had caused him to come to
the hospital. He was, in fact, confused upon admission but had become
perfectly clear by the next day. There was a considerable memory defect,
which the patient himself did not entirely appreciate. Possibly his
judgment had been deteriorating slightly. He had been irritable of late
and sometimes sleepless.
=Physical examination= showed a rather well preserved man with but
slight senile changes. The pupils were equal and reacted readily to
light and accommodation. There was no sensory disorder and no
disturbance of coördination. There were no tremors. The systolic blood
pressure was 205, the diastolic 135. The arteries were sclerotic upon
palpation. A sufficient diagnosis would have seemed to be
arteriosclerosis, and the hypothesis of syphilis would hardly have been
raised off-hand by most practitioners. The W. R. of the serum was
negative. What led to lumbar puncture in this case was the fact that the
sea captain’s wife had died 15 years before of general paresis. The
lumbar puncture was rewarding since the W. R. was positive. There was an
increase of albumin and globulin, a “paretic” type of gold sol reaction,
and 56 cells per cmm.
Accordingly, we must regard the condition as one of neurosyphilis.
Perhaps the arteriosclerosis was of syphilitic origin. If this is a case
of general paresis as we suppose, it is one of very long-standing
syphilis.
1. Do delusions of grandeur in the senile period suggest syphilis?
Not necessarily; it appears that there is a small group of senile
cases which might be called cases of senile pseudoparesis in which
extravagant delusions of grandeur are entertained, and in which
frontal atrophy is found although entirely without evidence of
chronic inflammation. It has not been proved that these cases are
of syphilitic origin. It is suggestive that the site of the most
extensive lesion is precisely the site of the most extensive
lesion classically found in paretic neurosyphilis, viz., in the
frontal regions.
2. Is neurosyphilis frequently found in both mates? It can hardly be
said that this is a usual finding. However, it is far from rare,
and it occurs frequently enough to be used in support of the
theory that there is a special strain of spirochete that has a
predilection for nervous tissue. It must be remembered, however,
that the wives of syphilitics are frequently infected without
being aware of it. In such cases they receive no treatment and
consequently have a larger chance of developing neurosyphilis. It
is a good rule to consider the mate of every syphilitic a
candidate for neurosyphilis.
=An atypical case of recurrent dazed states resembling HYSTERICAL
FUGUES. Probably an instance of NEUROSYPHILIS.=
=Case 74.= Abel Bachmann, a man of 40 years, remains doubtful and
perhaps belongs to the still unresolved group of mental cases due to
syphilis that cannot be placed in any of the well-known categories.
Bachmann had been found by the police, working in front of a cowbarn
without the consent or even the knowledge of the owner. Bachmann had, in
fact, spent the night in the cowbarn and was working with the idea of
paying for his night’s lodgings. The situation struck the police as so
peculiar, and Bachmann was so confused and irresponsive, that he was
brought to the Psychopathic Hospital. The afternoon of his admission,
however, he entirely cleared up and was able to give a good account of
himself.
His story was that he had been worrying a good deal about a divorce
suit, and the morning of his episode he had awakened with peculiar
feelings. He walked from Boston to Cambridge, feeling that he was in a
strange city. He recognized the places he passed, yet they all seemed to
be changed. Upon reaching Harvard Square, he determined to return to
Boston and walked and walked, failing to reach Boston. All day he had
eaten nothing; when night fell he stole into a field and dug out
radishes. A postman stopped and said, “Hello, Bill,” which awakened him
as by an electric shock. A barn presented itself, in which he spent the
night. In the morning, the barn looked different. In fact, his entire
surroundings appeared mysterious. As he felt like working, he went to
work in front of the barn.
It seems that in his life there had been two other episodes of a similar
nature; in fact, Bachmann had been in a state hospital for six weeks
after the first episode. The first episode had lasted a few days only,
and followed worry when he learned that the girl with whom he was in
love was married. The second attack followed the death of his mother,
whereupon he was taken to a state hospital although the total duration
of symptoms was only three days. Bachmann had had a chancre or some
other form of genital disease at 26, and had at that time been treated
with mercury.
Except for irregular and absolutely rigid pupils, reacting neither to
light nor to accommodation, Bachmann showed no physical and especially
no neurological disease whatever. Moreover, the W. R. in the blood serum
was negative.
As to diagnosis, one might consider hysteria, of which, however, there
are no visible stigmata. It would not appear that brain tumor would be
likely to have lasted so long as eight or nine years, even if we should
attempt to make the hypothesis of tumor cover both the non-reacting
pupils and the episodes. Bachmann was non-alcoholic, and there was no
sign of any other form of intoxication. The spinal fluid showed a
negative gold sol reaction, there were no cells in the fluid, there was
no globulin; albumin was normal. However, the W. R. was strongly
positive.
The situation, then, in this case is that we have somewhat peculiar
psychopathic episodes, pupils rigid to light and accommodation, a
positive W. R. in the spinal fluid, and extremely little else to permit
a diagnosis. We are ignorant as to the course and pathology of such
cases. However, we cannot resist the temptation of the diagnosis of
neurosyphilis, although further classification is not ventured.
1. What is the significance of stiff pupil as an isolated symptom?
Nonne finds that in the end, after years of observation, the
Argyll-Robertson pupil turns out to be an advance courier of other
more functionally serious signs and symptoms of neurosyphilis. We
can confirm this experience and regard it as an established
clinical proposition that the Argyll-Robertson pupil cannot be
neglected. In this connection, refer to the case of alcoholic
pseudoparesis (Murphy, 60), and also to the case of pineal tumor
(Donald Falvey, 35). Enthusiastic reports have occasionally been
made upon apparent restoration of the true syphilitic
Argyll-Robertson pupil to normal light reaction. The difficulties
in rendering the symptomatic diagnosis of Argyll-Robertson pupil
in a given case are so great, and the chances of complication so
numerous, that we are inclined to attach little significance at
present to these claims.
It may not be amiss to mention a somewhat humorous incident familiar
to some local neurologists. A case was reported by the interne for
a number of months as a victim of a pupil stiff to light and
accommodation, and the entirely adequate cause of this phenomenon
was actually only discovered at autopsy by the triumphant medical
examiner, who demonstrated that the patient in question was
possessed of a =glass eye=.
=TABETIC NEUROSYPHILIS (“tabes dorsalis”) versus PERNICIOUS ANEMIA
with spinal symptoms.=
=Case 75.= Mrs. Brown was a woman of 56, who for the past eight or ten
years had been complaining of trouble in her legs. As she described it,
at times her legs were so weak she could hardly stand; at other times
there was considerable pain and numbness. She has always been considered
“high strung”; that is, she had a very bad temper and lost control of
herself almost entirely when she became excited. Her legs had been
growing progressively worse, and for about a month prior to admission
she had been unable to stand or walk. She had also lost control of her
bladder. On account of her temper, it had been almost impossible to
nurse her; no nurse would stay with her because of her scolding and
fault-finding. Recently, she had been having fits of the blues.
Her husband, who was seen before Mrs. Brown, was an old gentleman, over
70, who was chiefly remarkable from the fact that he had unequal,
irregular pupils, which reacted neither to light nor accommodation;
there was also a speech defect.
The patient herself proved to be extremely irritable, as had been
stated,—so much so that at times it seemed almost impossible to do
anything for her. She was very querulous, constantly complaining, and
not satisfied with anything that was done. Aside from this, her =mental
examination= proved to be entirely negative; that is, there were no
psychotic symptoms.
The systematic =physical examination= gave the following significant
findings: blood pressure, 160 systolic, 90 diastolic; no evidences,
however, of peripheral arteriosclerosis. Patient was unable to walk or
stand, and had no control over her bladder. The knee-jerks and
ankle-jerks absent on both sides; ataxia in the leg movements; loss of
sense of localization, with no tenderness over the nerve trunks; no
atrophy, paralyses, or muscular asymmetry of the parts. The vibratory
sense was maintained. Subjectively, the patient thought that the
vibratory sense differed in the legs from that in the arms.
Localization, touch, pain, heat, and cold responded to correctly. The
arms showed nothing abnormal; there was no incoördination, dysmetria, or
dysdiadochokinesis. Her pupils were equal, regular, and both reacted
normally to light and accommodation.
=Diagnosis=: The first consideration in the case is naturally tabes
dorsalis, especially when one considers that the husband had signs which
suggested syphilis of the nervous system. The rapid onset of the acute
symptoms in this case, and the absence of the characteristic signs of
pain were at least atypical for this diagnosis, as was the absence of
any pupillary signs. Further, the W. R. was negative in the blood and
spinal fluid; there were no definite signs of inflammatory reaction as
shown by the other spinal fluid tests. These findings made a diagnosis
of tabes entirely improbable. After tabes, the most frequent cause of
the symptoms above enumerated is perhaps to be found in pernicious
anemia. Examination of the blood showed that the patient had 2,500,000
erythrocytes per cmm. The hemoglobin by Tallquist scale was 80%. The
smear was practically negative; no blasts could be seen. Although this
picture is not a typical one for pernicious anemia, at least it is
significant in the low number of red cells to be found, and as no causes
for anemia were to be found, it seemed probable that we were dealing
with a primary anemia. The diagnosis in the case, therefore, is spinal
sclerosis of primary anemia. The mental picture was not uncharacteristic
of PERNICIOUS ANEMIA.
1. Could the diagnosis be rendered in this case without a lumbar
puncture? In the first place, the emaciation is not entirely
characteristic. The pupils react normally to light. Probably such
a case might well have been regarded as one of tabes dorsalis in
former days, or even at the present time, if a lumbar puncture had
not been resorted to.
2. Could this case possibly have been one of tabes dorsalis with
negative spinal findings? Such cases have been reported
frequently, but, unlike the present case, are apt to be of
long-standing and non-progressive, in which the active
inflammation is no longer present. The negative findings would not
be consistent with tabes, in which the symptoms are of short
duration and of sudden onset.
3. If the serum W. R. had been positive would the diagnosis have
been neurosyphilis? We are loath to make the diagnosis of spinal
syphilis when the spinal fluid is normal. Syphilis may produce a
marked anemia, however, and thus produce symptoms such as shown by
Mrs. Brown. It is even possible that such is the explanation of
this case, taking into consideration the suggestive findings in
the husband. However, there is insufficient evidence to make such
an hypothesis rock firm, and we do not more than suggest it.
=Atypical case of CONGENITAL NEUROSYPHILIS—peculiar mental state.=
=Case 76.= James Seabrook, 20 years of age, obviously showed a number of
signs of congenital syphilis. The =physical examination= disclosed an
old scar and indentation in the right mastoid region, another on the
right side of the neck, another on the posterior surface of the right
forearm, and two on the outer surface of the right upper arm. The
lesions were about the size of half a dollar. There was a saddle-shaped
nose and a perforation of the palate and uvula; there were palpable
cervical and axillary glands, small but numerous. There was a dulness in
the region of the right scapula, and slight dulness on both sides
behind. There were loud whispering and piping râles and bronchial
breathing throughout the chest, more marked on the left; there was much
coughing, and the sputum was at times blood-stained. The pupils were
irregular but reacted perfectly. The left knee-jerk was slightly more
active than the right. The W. R. in blood and fluid was negative; the
gold sol, globulin and albumin tests were negative. There were, however,
56 cells per cmm. in the fluid.
We learned that the patient had had several spells of great excitement,
with pounding on the door and a desire to fight bystanders. There were
spells of headache and vertigo. =Mentally= the tests showed him to be
subnormal.
The =diagnosis= Of CONGENITAL SYPHILIS seems established; possibly the
pulmonary condition is syphilitic. The mental subnormality as well as
the abnormal traits and episodes are probably to be accounted for on the
basis of syphilitic involvement of the brain.
1. Are the headache and vertigo connected with syphilis? This is
perhaps suggested by the pleocytosis in the spinal fluid.
2. How shall we explain the negative W. R.? This patient had
received, shortly before his entrance to the hospital, salvarsan
and mercury. Possibly the drug treatment has little or nothing to
do with the negative W. R.’s since they not infrequently grow
weaker as congenital syphilitics grow older.
3. What is the explanation of the spells of excitement? Compare the
spells of excitement in a form of neurosyphilis described by
Kraepelin, namely: syphilitic paranoia, discussed in the case of
Bridget Collins (59).
4. Is treatment indicated considering the W. R.’s to be negative in
blood and fluid? Despite the negative W. R.’s in this case
treatment is strongly indicated on account of the pleocytosis.
This would seem to indicate that there is an active inflammatory
process in the cerebrospinal axis, and it is more than probable
that this process is syphilitic. How much real improvement of the
symptoms would result from antisyphilitic treatment it is
impossible to prophesy. Every case is a special problem, and this
case is very unusual in showing a pleocytosis in the absence of
other indications of syphilitic nervous disease, _viz._, globulin,
albumin and W. R.’s.
=CONGENITAL NEUROSYPHILIS resembling an undifferentiated case of
FEEBLEMINDEDNESS—actually PARETIC.=
=Case 77.= John Friedreich, a 7–year old boy, was brought to the
Psychopathic Hospital by agents of a charitable society, who found him a
neglected child and quite evidently a subnormal one.
The dominance of syphilis in the situation was clear. The boy’s father
had died but a few months before of syphilitic heart disease, from which
he is said to have suffered for five years. The boy’s mother (the
parents were first cousins) had also been treated for syphilis and was
excessively alcoholic. The first child of this union—a girl—had died at
6 years, of a disease diagnosticated spinal meningitis. The history
indicates that syphilis was acquired after the birth of this first
child; but in any event it is possible that the meningitic condition of
which the first child had died was syphilitic. The second pregnancy
terminated in a stillbirth; the third issued in a girl, who died two
weeks after birth of what was termed “inward convulsions.” The fourth
pregnancy resulted in a miscarriage; the fifth in our patient, John
Friedreich. The sixth pregnancy resulted in a girl, now 5 years of age,
who is apparently normal. (Her W. R. was negative and she shows no
stigmata of syphilis.)
The patient, John Friedreich, at some very early age had a rash on his
body diagnosticated as syphilis. He also had many seizures called
fainting spells. Ever since birth he had been taking mercury pills. He
had not learned to talk until his third year, and was able then to say
only a few disconnected words. In fact, John has never been able to talk
in complete sentences, mumbling much that is quite unintelligible.
However, he walked at 15 months in a normal fashion and nothing peculiar
in his gait was noted until he was 5 years old, when he began walking on
his toes, particularly those of his left foot. Shortly thereafter, the
seemingly inevitable trauma appeared; John fell out of a window and
severely injured his left leg, whereupon the peculiarity of toe-walking
became more pronounced and associated with a limp.
[Illustration:
Juvenile paresis. 7 years.
]
The patient strikes one =physically= as having the development of a
child of about five years (actual age, 7). There are a few lymph nodes
palpable in the anterior triangles of the neck. The dilated and slightly
unequal pupils react neither to light nor accommodation. There is
practically complete deafness; loud sounds are not at all noticed.
Withal, the child in a general way presents a somewhat attractive
appearance, being very playful and mischievous, lying about on the floor
and playing with whatever comes to hand, talking to himself or making a
few indistinct remarks to the bystanders. He walks awkwardly, on the
toes of the left foot. He pays little or no attention to his toilet and
needs to be dressed and cared for in all ways. He is quick-tempered and
at times very difficult to manage.
There was, of course, little doubt of the =diagnosis= of CONGENITAL
SYPHILIS and of FEEBLEMINDEDNESS. The W. R. was positive both in the
blood and in the spinal fluid. The gold sol reaction of the fluid was of
the “paretic” type; there were 44 cells per cmm. and there was a large
excess of albumin and much globulin.
As to prognosis, there is doubt.
1. Is, or is not, this a case of juvenile paresis?
2. Is it, perhaps, a relatively permanent case of feeblemindedness
due to congenital syphilis? On the whole, on account of the spinal
fluid symptoms, we should be inclined to give the case a
relatively poor prognosis, namely, of death in a few years.
However, we may perchance be later surprised to learn that the
patient has lived on, at least into early adult age.
Note: Mercury tablets in some cases of congenital syphilis do not
seem effective. John Friedreich was treated most intensively by
syphilographers from birth.
Dr. W. E. Fernald in a personal communication stated that
syphilitic cases of feeblemindedness are rather those of the
imbecile and idiot groups than of the higher levels. This
statement emphasizes again that the true hereditary cases of
feeblemindedness are rather those of the higher group, whereas the
cases in which special causes have operated in the uterus or in
early life eventuate in idiocy and imbecility. However, such a
case as that of Friedreich shows that now and then a case of
feeblemindedness without evidence of neurological disorder and
looking in almost all respects like an hereditary case may be at
times produced by syphilis.
3. How often is the central nervous system involved in hereditary
syphilis? An interesting table bearing on this point is presented
by Veeder.[16] The table concerns the lesions in various parts and
systems of the body in 100 cases of late syphilis. It appears that
in 47, or approximately one-half of Veeder’s series of 100 late
cases, the infection developed some form of lesion of the nervous
system. As Veeder remarks, this result runs counter to the common
statements of pediatricians, notably of Holt.
Bones:
Periostitis tibia 4
Periostitis skull 1
Osteomyelitis 1
Joints:
Acute arthritis knee 8
Acute arthritis ankle 1
Skin:
Macular eruption 1
Condyloma anus 3
Gummata 3
Alopecia 3
Eye:
Interstitial keratitis 24
Choroiditis 1
Ulcerations:
Nasal 2
Laryngeal 1
Pharyngeal 1
Central Nervous System:
Mental deficiency 23
Cerebrospinal syphilis 14
Hemiplegia 6
Epilepsy 5
Spastic paraplegia 4
Chorea 2
Hydrocephalus 2
Miscellaneous Conditions:
Ozena 1
Enlarged spleen (only symptom) 1
Torticollis 1
Aortitis 1
Obscure abdominal pain 1
Obscure pain in legs 2
Endarteritis obliterans 1
Paroxysmal hemoglobinuria 1
Raynaud’s disease 1
Hutchinson’s teeth 4
=Juvenile paretic neurosyphilis. Quadriplegia.=
=Case 78.= Gridley Ringer, 15 years of age, had the facies of a
congenital syphilitic, including Hutchinsonian teeth, rhagades of the
face, and the so-called Olympic brow. No secondary sexual
characteristics had developed. There was a marked speech defect.
=Mentally=, Ringer was a low-grade imbecile. He had been born at full
term, and delivery had been normal. There had never been other
pregnancies. He had never developed normally.
The father admitted syphilis 23 years before, namely, 8 years before the
birth of his son, but the father had been treated for several years and
had been declared cured.
1. What would be expected in the spinal fluid of this case? Without
the history, it would perhaps be impossible to say whether the
case was one of a quiescent imbecility or one of juvenile paresis.
The spinal fluid of the juvenile paretic gives a picture identical
with that in the adult. The spinal fluid in this case showed a
positive W. R. (as did also the serum), a marked increase of
albumin and globulin, 115 cells per cmm., and a “paretic” gold sol
reaction. Accordingly, the diagnosis of GENERAL PARESIS was made.