thirty-third year. The records show that at that time he was somewhat
depressed, and his speech was even then, according to his own statement,
stammering. However, we found the W. R. at that time to be negative in
the blood serum. It appeared that his mother had died of consumption;
his father was said to have committed suicide. A brother had once
recovered from an attack of depression, presumably an attack of
manic-depressive psychosis. Accordingly, we thought at the time that the
case was probably one of manic-depressive psychosis. Moreover, our
routine serum W. R. failed to indicate any syphilitic process. As for
the so-called stammering of speech, this appeared to be a matter of the
patient’s own recollection rather than of our observation. In any event,
the patient had gone into the country and appears to have entirely
recovered; falling, again, however, into mental difficulties after a
short period, and finally arriving at the hospital in the
above-mentioned classical condition.
The W. R. in the blood serum proved again negative. The test was
repeated a number of times; also, after salvarsan had been given. The
salvarsan did not act provocatively, and the blood serum has remained
consistently negative.
In cases of syphilis the W. R. is at times negative. Swift claims that
in such cases an injection of salvarsan will often produce a positive W.
R. if the blood is tested on several days following the injection.
The spinal fluid, however, did show a positive W. R. as well as a gold
sol reaction of a “paretic” type. There were at the first examination
194 cells per cmm., there was a moderate excess of albumin, and a
positive globulin test. In short, there was no question of any other
diagnosis than GENERAL PARESIS.
1. How can the negative W. R. of the blood serum be explained? It is
difficult or impossible to explain this. Figures differ as to the
percentage of cases of general paresis with negative blood serum;
perhaps 3 to 5% of these cases yield a negative serum W. R.
It is important to note the long preparetic period: at least a year
and a half. Could our diagnostic methods be sharpened a trifle,
such cases as these could be obtained early in this preparetic
period and it might then be safe to promise good therapeutic
results.
2. What is the nature of the preparesis of Dana? When Dana’s brief
paper on preparesis was written, there was of course hardly any
idea that cases of paretic neurosyphilis could be cured or would
recover, except possibly vanishingly few _curiosa_ about which
there would always rage a diagnostic question. Accordingly, Dana,
having found certain cases that seemed to him to have early signs
of paresis but had apparently been cured by treatment, proposed to
call them cases of preparesis. His idea was that he would thereby
not offend those who held that general paresis was theoretically a
fatal disease. With modern work and the display of more and more
atypical cases of neurosyphilis, and the observation of relatively
numerous cures or remissions under treatment, the designation of
preparesis for a separate entity, or even for a sub-form of
neurosyphilis, becomes superfluous.
3. What is the percentage of cases of paretic neurosyphilis that
show a negative serum W. R.? Among the best figures are those of
Müller, who found that of 386 examples of paretic neurosyphilis,
379 showed all reactions positive, or 98.5%.
4. What is the meaning and value of the so-called provocative
salvarsan injection? In practice, there may be a series of
negative W. R.’s in the blood serum before a positive reaction is
finally obtained, owing to technical difficulties or biological
peculiarities. Where intensive work is being done upon the
neurosyphilis problem, it is beyond question desirable to make the
W. R. test upon at least three separate samples of blood drawn at
intervals, for the second or third test may prove positive. This
situation makes the interpretation of the so-called provocative
salvarsan injection exceedingly doubtful; that is, the reaction
might have been positive on repetition without the injection of
salvarsan. The present case, as above stated, failed to yield a
serum W. R. even after repeated tests and the “provocative.”
5. What is the significance of the irregular pupils in this group?
Paretic neurosyphilis shows inequality of the pupils in a high per
cent of cases. Irregularity of outline of the pupils is commonly
thought to be an important sign and to suggest neurosyphilis. It
is true that many cases of pupillary irregularity are syphilitic,
but the sign is of little or no differential value since
congenital malformations and relics of old injuries and adhesions
may produce effects identical with those of neurosyphilis.
=DIFFUSE (that is, meningovasculoparenchymatous[5]) NEUROSYPHILIS is
typically associated with six positive tests (serum Wassermann
reaction, fluid Wassermann reaction, spinal fluid gold sol reaction,
pleocytosis, positive globulin, excessive albumin); but one or more,
and frequently several, of these tests are likely to run mild as
compared with the tests in PARETIC NEUROSYPHILIS (“general
paresis”). The clinical course of the diffuse (and especially the
meningovascular) cases is likely to be protracted, with a good
prognosis as to life (barring fatal vascular insults).=
=Case 14.= We shall present the case of John Jackson, a surveyor, 31
years of age, suffering from a left hemiplegia, with this in mind: To
exhibit difficulties in diagnosis in the presence of an embarrassment of
symptomatic riches.
The patient arrived at the hospital, in the first place, because he had
been threatening a woman who lived next door to him. He believed that
this neighbor had been talking about him and circulating reports against
him. Excited by these ideas, he had threatened to cut her throat.
Now the occurrence of hemiplegia in adult life before the approach of
senium is always suspicious of syphilis, and this suspicion we naturally
entertained from the beginning. However, there was upon the scalp a
crooked linear furrow about six inches long, running from the vertex to
the right parietal eminence. Another furrow about an inch long was
present upon the forehead. These furrows appeared to be of a bony nature
and were not tender. There was evidence of an old decompression
operation on the right side of the head; there were also large scars on
both sides of the neck, evidently the result of old operations; and
there were numerous palpable glands—the largest about the size of a lima
bean—all firm and not tender.
[Illustration:
Station in syphilitic hemiplegia. Syphilitic pigmentation of skin.
]
It seems that at the age of eight, according to the patient’s mother,
Jackson had received a head injury and had remained unconscious for
three weeks. Upon recovery, he had to relearn both to walk and to talk;
however, he was able to begin school where he left off. He became more
nervous and irritable after the accident than previously. Nothing
further had developed until, at about 25 years of age, a tubercle was
discovered in his eye (the right pupil was smaller than the left,
reacting more slowly; right iris bound down by adhesions, with white
opacity of anterior chamber). For two years, 25 to 27, the patient was
under medical treatment for tuberculosis, and at the conclusion of this
period numerous glands were removed from the neck and diagnosticated
tuberculous. However, the neck did not heal and he carried bandages upon
it for two years.
At 28, the patient’s mother described the occurrence of a slight shock,
with head retraction, for a minute or two, and inability to speak.
Thereafter there had been five or six similar attacks, less severe, and
without loss of speech. The attacks were never accompanied by convulsive
movements. Then occurred a paralytic stroke, leaving the patient with a
left hemiplegia, which had somewhat improved. Mentally, the patient had
gone down hill, becoming less alert and more apathetic, and to some
extent amnestic. One had to consider, accordingly, the somewhat doubtful
possibility of post-traumatic and post-operative conditions, and the
question of tuberculosis (possibly errors in diagnosis; the lungs showed
no evidence of tuberculosis).
=Physically,= the signs of a left hemiplegia were appropriate.
Spasticity on the left side was found; there were Babinski, Gordon,
Oppenheim reflexes and ankle clonus on the left side (all absent on the
right). Speech defect was present. =Mentally,= aside from the delusions
noted at the beginning of our analysis, a striking feature was the
patient’s childishness. While reciting delusions, the patient was
overactive and evinced a somewhat childish interest. Arithmetically,
Jackson had preserved a fair ability but his apathy and lack of interest
interfered with tests, and possibly also with the exercise of memory. As
above noted, we were compelled to maintain the suspicion of syphilis
throughout despite the attractive hypotheses of traumatic and
post-decompressive effects and cerebral tuberculosis. A history of the
acquisition of syphilis an unknown number of years before admission
entered to strengthen the suspicion of the syphilitic nature of the
mental symptoms.
TYPICAL LABORATORY FINDINGS IN NEUROSYPHILIS (NONNE, 1915)
─────────────┬─────────┬─────────┬─────────┬───────────────┬───────────
DIAGNOSIS │ W. R., │ W. R. │ SPINAL │ PHASE I, │PLEOCYTOSIS
│ BLOOD │0.22 CC. │ FLUID, │ GLOBULIN │
│ SERUM │ BLOOD │ 1.0 CC. │ │
│ │ SERUM │ │ │
─────────────┼─────────┼─────────┼─────────┼───────────────┼───────────
PARESIS OR │POSITIVE │POSITIVE,│POSITIVE,│POSITIVE, │POSITIVE,
TABOPARESIS│ IN │ 85–90% │ 100% │ 95–100% │ ABOUT 95%
│ ALMOST │ │ │ │
│ 100% │ │ │ │
│ │ │ │ │
TABES (not │POSITIVE,│POSITIVE,│POSITIVE,│POSITIVE, │POSITIVE,
combined │ 60–70% │ 20% │ 100% │ 90–95% │ 90%
with │ │ │ │ │
paresis) │ │ │ │ │
│ │ │ │ │
CEREBROSPINAL│POSITIVE,│POSITIVE,│POSITIVE │POSITIVE almost│POSITIVE
SYPHILIS │ 70–80% │ 20–30% │ ALMOST │ always; │ ALMOST
│ │ │ ALWAYS │ NEGATIVE only│ ALWAYS
│ │ │ │ EXCEPTIONALLY│
─────────────┴─────────┴─────────┴─────────┴───────────────┴───────────
CHART 8
[Illustration:
Syphilitic thrombosis. Contours of brain preserved.
]
The W. R. proved positive in blood and spinal fluid. The gold sol
reaction was of the syphilitic type; 37 cells were found per cmm.; there
was a slight amount of globulin and a slight excess of albumin.
We made a diagnosis of CEREBROSPINAL SYPHILIS rather than general
paresis on account of, first, the slow course of the disease; second,
the vascular type of the cerebral insult, hardly typical of paresis; and
third, the mild spinal fluid reaction. Treatment will hardly cure the
hemiplegia, at least so far as restoration of cerebral tissues lost in
the insult is concerned. We were perhaps entitled to consider that, as
in the cases of Petrofski (17), O’Neil (19), Robinson (45), the
meningitic process could be arrested. Unfortunately, our treatment of 20
injections of salvarsan over a period of 10 weeks, followed by a number
of months of bi-weekly injections of mercury salicylate, proved
incapable of making any change in the mental and physical picture or in
the laboratory findings.
1. Can we explain the apparently poor reaction to treatment of the
cerebrospinal syphilis in the case of Jackson by supposing a more
deep-seated involvement than the meningovascular involvement
indicated by the hemiplegia and the signs in the fluid? Autopsied
cases in our experience show focal parenchymatous involvements
that have not caused obvious clinical symptoms at any time during
the course of the disease. These symptomatically silent lesions
may have been present.
2. What is the comparative prognostic value of seizures in paretic
neurosyphilis and in such a meningovascular case as that of
Jackson? Paretic seizures are often and indeed characteristically
recovered from. Moreover, autopsies in paretic neurosyphilis
characteristically show no gross focal destructive lesions to
correspond with the seizures. The paretic seizures are apparently
more irritative than paralytic. However, the seizures of the
meningovascular group of neurosyphilis are also, though less
commonly, recovered from, so that the differential diagnosis on
the basis of the outcome of seizures is not safe. Rarely paretic
neurosyphilis itself also develops seizures from which no recovery
is made.
3. What is the relation of neuropathic heredity to neurosyphilis?
The family history of John Jackson is undoubtedly poor, since his
father died of diabetes and a paternal uncle was insane; and on
the mother’s side, the grandmother died of tuberculosis and an
aunt died insane. This general question was more interesting in
the days before the syphilitic nature of general paresis and of
allied diseases was known. However, we may still hold perhaps that
not only syphilis but also various intoxications, especially
alcoholism, do flourish upon a neuropathic soil. This question,
like that of Krafft-Ebing’s celebrated claim of the relation
between syphilization and civilization, needs revision in the
light of more extensive applications of the W. R. in larger and
larger groups of persons under various community conditions.
=The SIX TESTS (serum Wassermann reaction, fluid Wassermann
reaction, pleocytosis, gold sol reaction, globulin, excess albumin)
are likely to run STRONGER in PARETIC NEUROSYPHILIS (“general
paresis”) than in DIFFUSE (especially meningovascular)
NEUROSYPHILIS; in particular, the gold sol reaction is likely to
prove “paretic” rather than “syphilitic.” The clinical course of
paretic neurosyphilis (“general paresis”) is likely to terminate in
death within a few years.=
=Case 15.= Pietro Martiro was a well developed and nourished man, 30
years of age, who had been doing erratic things and acting peculiarly
for a few weeks before entering the hospital. In the hospital, Martiro
proved to be very excitable and given to violence. He had marked
delusions of grandeur, saying he was worth many millions of dollars, was
the greatest singer in the world, the greatest athlete in the world, and
the like.
=Physically=, there was no disorder except overactivity of some
reflexes. The diagnosis of GENERAL PARESIS offered no difficulties, and
it was confirmed by the laboratory tests (positive serum and fluid W.
R., “paretic” gold sol reaction, 42 cells per cmm., an excess of
albumin, and a positive globulin test).
=Treatment=: The perfect physique of this case and the extremely brief
clinical duration (a few weeks) would naturally suggest a probably
favorable outcome. However, cases with marked delusions of grandeur have
very frequently proved to be cases with extensive brain tissue loss as
shown in certain studies with Danvers material.
In any event, the treatment in this case proved unavailing. Enormous
doses of salvarsan, twice a week, aided by mercury and potassium iodid,
were given. Although other cases had been helped by such intensive
treatment, Martiro went steadily downhill, nor was there the slightest
diminution in the intensity of any of the spinal fluid reactions. After
50 injections of salvarsan over a period of 30 weeks without
improvement, treatment was discontinued. A few months later, the patient
died.
=PARETIC NEUROSYPHILIS (GENERAL PARESIS)=
=PHYSICAL SYMPTOMS=
EARLY HEADACHE
VISUAL DISORDER
HYPALGESIA
ADIADOCHOKINESIS
ATAXIA
NASOLABIAL FLATTENING
VOCAL CHANGE
SPEECH DISORDER
WRITING DISORDER
LOSS OF MANUAL DEXTERITY
PUPILLARY CHANGES
REFLEX CHANGES
SEIZURES
LATE: PARALYSIS, CONTRACTURE
CHART 9
=PARETIC NEUROSYPHILIS (GENERAL PARESIS)=
=MENTAL SYMPTOMS=
INTAKE IMPAIRED
CONSCIOUSNESS CLOUDED
FATIGUABILITY INCREASED
HALLUCINOSIS RARE
AMNESIA—RECENT! CHRONOLOGY AND STORAGE IMPAIRED. FABULATION
OVER-SUGGESTIBILITY
JUDGMENT IMPAIRED
FANTASTIC DELUSIONS
INSIGHT INTO ILLNESS NIL
EARLY IRRITABILITY OR HEBETUDE
QUICK SHIFTING EMOTION
CHARACTER CHANGE
CONDUCT SLUMP
CHART 10
1. What is the duration of paretic neurosyphilis (“general
paresis”)? If we omit the doubtful, early, and prodromal stages
and count the beginning of the disease with the occurrence of
definite symptoms, we find (Kraepelin) that almost half the
patients with pronounced paretic signs die within the first two
years of their disease. Kraepelin’s observations upon 244 cases
are as follows:
Year: 1 2 3 4 5 6 7 8 9 10 14
Cases: 51 63 52 41 22 4 5 2 2 1 1
The average duration of the disease in months has been calculated
as varying from 24 to 32 months. Juvenile paresis runs a slower
and more insidious course. The duration of paresis, according to
many observers, diminishes with the increasing age of the patient.
It is now held that a combination of tabes with paresis does not
prolong the duration of the paresis. As noted above in the
discussion of Case Harrison (9), our conceptions of the
characteristic duration of paretic neurosyphilis must alter with
the increase of our knowledge due to the early application of
laboratory tests.
2. What is the significance of the term _general paresis_? The case
of Martiro is, of course, a good instance to show that the term is
sometimes a misnomer. The characteristic generalized motor
incapacity denoted by the term _general paresis_ is shown in
patients in the institutions for the chronic insane in their last
few months of life. The term _paresis_ is perhaps to be preferred
to the term _paralysis_ because the paralysis is not complete but
partial; but perhaps the best reason is that the word _paresis_ is
a shorter word. When the mental side is to be emphasized, the term
_paralytic dementia_ is employed. In this book we have used the
term _paretic neurosyphilis_ to mean a more precise statement of
the etiology of general paresis (general paralysis, paralytic
dementia). The lay term, _softening of the brain_, like the terms
_metasyphilis_ and _parasyphilis_ is in the present phase of our
knowledge to be eschewed.
[Illustration:
Euphoria in paretic neurosyphilis (“general paresis”). The head, arms
and trunk were shaking with mirth; hence, the indistinct outlines of
the photograph.
]
3. If this fatal case be typical of general paresis (for more
favorable results, see Part V), what is the toll of deaths from
this disease in the community at large? A striking statement may
be quoted from Dr. Thomas W. Salmon’s “Analysis of General
Paralysis as a Public Health Problem:”
“With the information in our possession at the present time, we
are able to state that not fewer than 1000 persons in whom general
paralysis is recognized die in New York State every year. Let us
compare this with the lives lost from some other important
preventable diseases. It means that _one in nine_ of the 6909 men
who died between the ages of 40 and 60 in New York last year died
from recognized general paralysis and that _one in thirty_ of the
5299 women who died in the same age-period died from this disease.
“The number of deaths from general paralysis in New York last year
about equalled the number of deaths from typhoid fever. The
following table gives the number of deaths due to the ten most
important specific infectious diseases. Of course, deaths in
measles, typhoid fever and scarlet fever will be found also under
the names of some of the complications of these diseases, but it
should be remembered that these primary diseases are not
invariably fatal as general paralysis is. Many of the patients
with measles who died from bronchopneumonia would have recovered
but for this complication, while the paretics with
bronchopneumonia would have died even if this complication had not
arisen. No attempt is being made to compare the _prevalence_ of
general paralysis with that of other diseases—we are trying only
to estimate its share in the _mortality_.
“1. Tuberculosis (all forms) 16,133
2. Pneumonia 9,302
3. Bronchopneumonia 7,217
4. Diphtheria and croup 1,854
5. Influenza 1,381
6. Measles 1,071
7. Typhoid Fever 1,018
_General paralysis (recognized)_ 1,000
8. Scarlet fever 837
9. Whooping cough 818
10. Syphilis 782”
=PARETIC NEUROSYPHILIS (GENERAL PARESIS)=
=CHARACTERISTICS=
AMNESIA
QUICK SHIFTING EMOTIONS
CHARACTER CHANGE
CONDUCT SLUMP
NERVOUS DISORDERS
SPEECH DISORDERS
PUPILLARY CHANGES
REFLEX CHANGES
SEIZURES
CEREBROSPINAL FLUID PICTURE
CHART 11
=SYPHILITIC PSYCHOSES=
SYPHILITIC NEURASTHENIA
GUMMA
SYPHILITIC PSEUDOPARESIS
APOPLECTIC CEREBRAL SYPHILIS
SYPHILITIC EPILEPSY
SYPHILITIC PARANOIA
TABETIC PSYCHOSIS
HEREDITARY
PARESIS
KRAEPELIN, 1910
CHART 12
=TABOPARETIC NEUROSYPHILIS (“taboparesis”) is CLINICALLY a
combination of the symptoms of TABES DORSALIS and those of GENERAL
PARESIS. The COURSE of TABOPARESIS is likely to be from a
characteristic tabes dorsalis (often of years’ standing) to a
characteristic general paresis; the ultimate paretic picture is
likely to retain various characteristics of tabes. The LABORATORY
TESTS in the paretic phase are characteristic of ordinary
(non-tabetic) general paresis. The PROGNOSIS, after the paretic
phase has arrived, is apt to be that of general paresis.=
=Case 16.= Joseph Sullivan, a waiter, 50 years of age, sought assistance
at the Psychopathic Hospital voluntarily. His complaint of severe and
lancinating pains in the legs, difficulty with his gait, and a feeling
of constriction about the waist, was forthwith suggestive of tabes
dorsalis. He was a rather poorly nourished, white-haired man, with a
drooping of the left side of the face. The pupils reacted sluggishly to
light, the right somewhat better than the left. A marked Romberg
reaction could be demonstrated. Ataxia in walking was marked. There was
some incoördination of the hands, considerable tremor, and writing was
poorly performed. The ankle-jerks and knee-jerks were absent. On the
whole, the diagnosis of TABES DORSALIS was clear enough.
The most appealing situation was =mental=. Sullivan was exceedingly
apprehensive about his condition on the ground that it was growing
progressively worse; if it was to get worse, Sullivan feared he would
commit suicide. From his own account, he had become irritable,
quick-tempered, and often unreasonable. As usual in these cases, the
question arose whether the depression was psychopathic or natural.
=TABETIC SYMPTOMS AND SIGNS IN ORDER OF THEIR FREQUENCY=
=ANALYSIS OF 250 CASES=
PER CENT
1. ROMBERG SIGN 96.4
2. ABSENT KNEE-JERKS 90.0
3. LANCINATING PAINS 88.4
4. STAGGERING GAIT 87.2
5. ARGYLL-ROBERTSON PUPIL 80.0
6. ATAXIA IN UPPER EXTREMITIES 68.2
7. SPHINCTER DISTURBANCES 67.6
8. SENSORY DISTURBANCES 58.2
9. VISUAL DISTURBANCES 43.6
10. PARESTHESIA AND NUMBNESS OF FEET AND LOWER EXTREMITIES 42.8
11. GIRDLE SENSE 31.2
12. PTOSIS OF EYE-LIDS 23.2
13. PARESTHESIA OR NUMBNESS IN HANDS OR UPPER EXTREMITIES 13.6
14. STRABISMUS 12.0
15. VISCERAL CRISES 12.0
16. LOSS OF SEXUAL DESIRE 11.5
17. CHARCOT JOINTS 9.2
18. VERTIGO 4.0
19. MAL PERFORANS 3.2
20. PAIN IN JOINTS 2.8
21. RECTAL TENESMUS 2.8
22. MENTAL DEGENERATION (other than paresis) 2.4
23. HEMIPLEGIA 2.4
24. VESICAL TENESMUS 2.0
25. DIFFICULTY IN ARTICULATION 2.0
26. DEAFNESS 1.2
27. ANOSMIA 0.8
BALDWIN LUCKE.
CHART 13
While in the hospital things shortly came to a crisis. In the midst of a
fit of depression, Sullivan attempted suicide by beating his head
against the wall. Whether this attempt could be regarded psychopathic,
however, remained in question. Sullivan had been drinking very heavily
although he had stopped about six weeks before admission, fearing that
the alcohol was causing a development of symptoms. The remedy was almost
worse than the disease because he then became more nervous, lost his
appetite, and had a marked insomnia.
According to the patient’s own history, he had had several attacks of
gonorrhœa and a syphilitic infection at the age of 19; that is, some 31
years before admission to the hospital. However, the first
_neurological_ symptoms of which the patient was aware came about 27 or
28 years after infection, namely, 3 or 4 years before admission, when
facial paralysis developed. At that time, he had suddenly felt a
peculiar sensation in the throat and became unable to swallow for a
time. His voice remained hoarse and low for some time, and his face
began to droop. The lancinating pains and the ataxia also dated back
several years.
1. How shall we evaluate the mental symptoms? The prognosis of tabes
dorsalis is relatively good so far as life is concerned, and it
might even be possible for Sullivan by training to remain capable
of being a waiter. The manual incoördination was not marked, and
possibly the manual tremor was in part due to alcohol.
Accordingly, the mental symptoms, such as emotional lability and
memory defect, were in the foreground of attention. In point of
fact, the laboratory examinations showed positive W. R. in the
serum and the spinal fluid, which latter also contained 60 cells
per cmm., positive globulin, and an excess of albumin. THE
DIAGNOSIS MADE WAS THAT OF TABOPARESIS, meaning thereby a tabes
associated with appropriate symptoms of a mental nature.
2. How shall the term _taboparesis_ be used? Some use the term, as
we feel erroneously, for instances of general paresis which happen
to show crural areflexia (absence of knee-jerks). We feel that the
best usage of the term is for instances in which well-defined
symptoms of tabes (as well as of paresis) are present, namely,
characteristic ataxia, lightning pains, and the like. If the term
is used more loosely, as above mentioned, then practically every
case of general paresis might perhaps be termed _taboparesis_,
since almost every case of paresis does show involvement of the
cord as well as of the cerebrum. Such involvement may lead to
hyperreflexia, hyporeflexia, or areflexia according to the
localization of the process. In true taboparesis, in which there
is a commingling of the features of tabes with those of paresis,
we should find the posterior roots of the spinal cord affected.
The spinal lesions of paresis itself are more apt to be
intraspinal; that is, confined to the nervous system within the
pial investment.
3. Bearing in mind that Sullivan was a waiter, what shall be said
about the infectivity of these cases? It is counted as a rule as
negative, since there are no open spirochete-bearing lesions. The
longer the period since infection the less, as a rule, is the
chance of contagion in syphilis; and as tabes and paresis occur
fairly late in the disease, the infectiousness at this stage is
practically negligible.
4. Of what differential value is the insight shown by Sullivan into
the nature of his symptoms? Kraepelin remarks that a genuine
insight into the nature of the disease does not as a rule occur in
paresis. At the beginning of the disease, there may sometimes be a
correct understanding of the nature of the disease and of its
probable outcome; but the presence or absence of insight into the
fact of mental disease is by no means a differential sign of
practical value.
5. What is to be said of the occurrence of depression and excited
states in paretic neurosyphilis? A variety of classifications of
sub-forms of paretic neurosyphilis have been propounded.
Kraepelin, for example, deals with four: the demented, depressive,
expansive, and agitated forms, but remarks that the division is
merely convenient for exposition. The institutional intake does
not accurately represent the distribution of cases. Under
psychopathic hospital conditions with the relatively easy resort
to such institutions, the number of quiet cases increases; under
the less advanced conditions in Heidelberg, Kraepelin took in 53%
demented paretics as against 56% at Munich (73% women) under the
easier conditions of admission. The admissions of demented
paretics varied from 37 to 56%. The variations depend much upon
the facility with which the cases can be brought to institutions.
Where admission is beset with various legal restrictions, the
quiet and demented cases are more apt to be treated for long
periods at home. The depressive type of paretic neurosyphilis
forms a much smaller group, according to Kraepelin, as only about
12% of his Heidelberg admissions were of this type, and still
fewer of his Munich admissions. Other authors give percentages as
high as 16 and 19. The so-called expansive group is larger,
Kraepelin finding 30% of his Heidelberg cases to be of this group,
and 21 to 22% of his Munich cases. The rarest sub-form of paretic
neurosyphilis is the agitated form: 6% of Kraepelin’s Heidelberg
admissions; 14% among males and 5% among females in his Munich
admissions, where the diagnosis of agitated paresis was entered on
somewhat broader lines. French authors (Sérieux and Ducaste) have
enlarged the number of sub-forms of paretic neurosyphilis as
follows: Expansive 27%; sensory 24%; demented 24%; persecutory 3%;
depressive 2%; circular 7%; hypochondriacal 7%; and maniacal 6%.
=DIFFUSE (meningovasculoparenchymatous) NEUROSYPHILIS may look
precisely like PARETIC NEUROSYPHILIS (“general paresis”) at certain
periods of clinical and laboratory examination.=
=Case 17.= The police found Gregorian Petrofski crouching on his knees
on a Boston sidewalk, attempting to take pickets off a fence. Petrofski
knew little English; he said that he had slept in Poland the night
before. He did not appear to be alcoholic.
When he was examined, through an interpreter, he told how he had been in
America two days, and in Boston two years; that he was at the present
time in Poland, and that his brother had brought him to the hospital and
left him there.
The =physical examination= showed Petrofski to be well developed and
nourished. His pupils were somewhat dilated and reacted somewhat slowly
to light and accommodation. =Neurologically,= there was nothing else
abnormal found upon systematic examination although, through lack of
coöperation, sensory and coördination tests proved difficult if not
impossible. There was a large ulcer on the under surface of the glans
penis, with several small smooth scars on the upper surface. There was a
purulent discharge from the external meatus. There were exostoses of
both tibiae.
The initial diagnosis had to consider uremia and diabetes, which could
be easily excluded on examination. Alcoholism was excluded through
absence of alcohol on the breath. There remained such diagnoses as
epilepsy, some post-traumatic condition, or meningitis, to say nothing
of the hypothesis of syphilis raised by the tibial exostoses and the
lesions of the penis. The hypothesis of trauma was given up, as well as
epilepsy and meningitis upon the data of the lumbar puncture. The spinal
fluid proved to be clear but with enormous amounts of globulin and
albumin, 80 cells per cmm., a “paretic” gold sol reaction, and a
positive spinal fluid W. R. (the serum W. R. was also positive).
Accordingly, it was clear that the case was one of neurosyphilis.
Treatment was instituted with injections of mercury salicylate, a grain
and a half twice a week, and potassium iodid. After some weeks,
diarrhoea and salivation with marked symptoms of mercury poisoning set
in; the treatment was suspended, but later re-instituted. In a few weeks
Petrofski was apparently quite well, the spinal fluid tests had all
become negative, as had the serum W. R.
Petrofski now began to pick up a good deal of English, and gave a
consistent narrative of his past life, although the period just prior to
and during his early stay in the hospital has remained blank. Without
further treatment Petrofski has remained well for over a year.
1. Does the “paretic” gold sol reaction mean general paresis? In
connection with this general question, a brief summary of the
significance of the gold sol reaction in this group may be made.
(1) Fluids from cases of general paresis in the vast majority of
cases will give a strong and fairly characteristic reaction,
especially if more than one sample is tested. (2) Very rarely
general paresis fluid will give a reaction weaker than the
characteristic one. (3) Fluids from cases of syphilitic
involvement of the central nervous system other than general
paresis often give a weaker reaction than the paretic, but in a
fairly high percentage of cases give the same reaction as the
paretics. (4) Non-syphilitic cases may give the same reaction as
the paretics; these cases are usually chronic inflammatory
conditions of the central nervous system. (5) When a syphilitic
fluid does not give the strong “paretic reaction” it is
presumptive evidence that the case is not general paresis, and
this test offers a very valuable differential diagnostic aid
between general paresis, tabes, and cerebrospinal syphilis. (6)
The term “syphilitic zone” is a misnomer, as non-syphilitic as
well as syphilitic cases give reactions in this zone, but no fluid
of a case with syphilitic central nervous system disease has given
a reaction out of this zone, so that the finding may be used
negatively; and any fluid giving a reaction outside of this zone
may be considered non-syphilitic. (7) Mild reactions may occur
without any evident significance, while a reaction of no greater
strength may mean marked inflammatory reaction. (8) Tuberculous
meningitis, brain tumor, and purulent meningitis fluids
characteristically, though not invariably, give reactions in
higher dilutions than syphilitic fluids. (9) The unsupplemented
gold sol test is insufficient evidence on which to make any
diagnosis, but used in conjunction with the W. R., chemical and
cytological examinations, it offers much information, aiding in
the differential diagnosis of general paresis, cerebrospinal
syphilis, tabes dorsalis, brain tumor, tuberculous meningitis, and
purulent meningitis. (10) We believe that no cerebrospinal fluid
examination is complete for clinical purposes without the gold sol
test.
=FREQUENT SYMPTOMS IN DIFFUSE AND VASCULAR NEUROSYPHILIS=
=(“CEREBRAL” AND “CEREBROSPINAL SYPHILIS”)=
PUPILLARY DISORDER
HEADACHE
VERTIGO
INSOMNIA
DROWSINESS
CHANGE IN DISPOSITION
Irritability Slow thinking
SEIZURES
PARALYSES
Permanent Transient
APHASIA
HEMIANOPSIA
SENSORY DISTURBANCES
GASTRIC CRISES
SPHINCTER DISTURBANCES
INTRACRANIAL PRESSURE SYMPTOMS
POLYURIA, POLYDIPSIA, GLYCOSURIA
MÉNIÈRE’S SYNDROME
NYSTAGMUS
CHART 14
See Appendix B for technical details.
2. What is the relation of the tibial exostosis to neurosyphilis?
The syphilographers have always stressed the tibial lesions in the
diagnosis of syphilis. Although not so much attention has been
paid to these and kindred osseous lesions in neurosyphilis, yet we
have frequently found such lesions and they afford an important
auxiliary means of diagnosis.
=A POSITIVE SERUM Wassermann reaction with a NEGATIVE FLUID
Wassermann Reaction may be found in NEUROSYPHILIS, particularly in
VASCULAR NEUROSYPHILIS: the remaining signs in the fluid, although
frequently positive, may even be negative.=
=Case 18.= Frederick Wescott was a promoter, an elderly looking man of
60 years. His health had been failing for 18 months. There had been
shortness of breath, dizziness, a tired feeling, inability to “get the
words he wanted,” and forgetfulness of names. About eight weeks before
examination, Wescott had had a convulsion, following which he had been
unable to express himself at all well. This convulsion was not
accompanied by loss of consciousness. Besides a marked motor aphasia,
there was agraphia.
=Physically=, Wescott showed arteriosclerosis and a blood pressure of
135 systolic, but, except very lively knee-jerks, no other reflex
disorders or anomalies were discovered. In particular, the pupils
reacted fairly well.
There was, perhaps, no special reason to implicate syphilis in the case,
yet Wescott gave a history of syphilis at 35 years. The W. R. of the
blood serum proved positive; that of the spinal fluid was negative, and
the albumin was but slightly increased; there was a very slight amount
of globulin, and there were 16 cells per cmm. in the fluid. The gold sol
reaction suggested syphilis.
We felt entitled to make a diagnosis of SYPHILITIC CEREBRAL
ARTERIOSCLEROSIS, regarding the convulsion or seizure eight weeks before
as due to a vascular insult. The laboratory picture in the spinal fluid
in Wescott’s case seems to be rather characteristic of this group of
syphilitic arteriosclerotics.
1. What is the reason for the negative spinal fluid W. R.? The
theory would be that the syphilitic lesion is localized in the
vascular system and that the parenchyma is only secondarily, if at
all, involved. The W. R. producing bodies are accordingly not
found in the fluid.
2. How frequently are several of the spinal fluid tests negative,
while others are positive? Whereas, clinically speaking, the five
tests in the spinal fluid (W. R., globulin reaction, excess
albumin, pleocytosis, and gold sol reaction) are each indicative
of a pathological condition in the central nervous system, yet a
specially intensive study of the distribution of these tests has
shown that they are prone to occur independently. Consequently, we
must concede that they do not all represent the same inflammatory
products and chemical conditions. The W. R. producing bodies, the
gold sol reaction producing bodies, as well as the globulins and
albumins, have been proved to be separate. Special work has also
shown that _these tests disappear under treatment at different
rates_. There is, unfortunately, no doubt that the rate and
intensity, presence or absence, and the order of disappearance of
these tests in either treated or untreated cases, do not at all
parallel the clinical conditions of the patients.
3. What is the prognosis in vascular neurosyphilis, such as in the
case of Wescott? The prognosis is identical with that of cerebral
arteriosclerosis in general, that is to say, bad, but with
frequent periods of improvement. In the neurosyphilitic type of
arterial disease thromboid formation is frequent. Where the lesion
is chiefly perivascular infiltration, rather than disintegration
of the vessel wall, improvement may very well occur as a result of
treatment. Wescott showed slight improvement under treatment. He
has already lived two years since his first convulsion, and three
and a half years since the onset of symptoms.
=DIFFUSE NEUROSYPHILIS (so-called “cerebrospinal syphilis”) is often
marked by SEIZURES.=
=Case 19.= Agnes O’Neil, an unmarried woman of 28 years, was first
examined five weeks after the initial symptoms. It appears that she had
had certain seizures, with unconsciousness and twitching of the limbs
(otherwise not well described), followed by confusion of mind and
sometimes by a weakness of the left side and a difficulty in speaking.
Headache had been almost constant, as well as pains in the arms and
legs.
=Physically=, both in general and =neurologically=, there were no signs
or symptoms; mentally, we could discover no symptoms. Syphilis was
denied, although possible exposure to syphilis was admitted.
The =diagnosis= of some form of organic brain disease was clear with the
picture of convulsions followed by slight aphasia with headaches and
limb pains. With onset at 28, the most frequent cause for such
epileptiform seizures is certainly syphilis. Examination of the blood
and spinal fluid showed a positive W. R., in both. The albumin was also
somewhat increased. The clinical picture suggested a fairly generalized
meningitic involvement.
The =prognosis= in such cases of generalized meningitic involvement is
in general good, and this principle was illustrated in the O’Neil case,
in which the symptoms soon disappeared under intensive antisyphilitic
treatment. In fact the spinal fluid W. R. became negative in the course
of four weeks. The blood serum W. R., however, has remained positive
despite eight months of active treatment.
=CONDITIONS IN WHICH CONVULSIONS OCCUR=
NEUROSYPHILIS
HYSTERIA
EPILEPSY MAJOR (GRAND MAL)
EPILEPSY MINOR (PETIT MAL)
DEMENTIA PRAECOX
TOXIC CONDITIONS:
Asphyxia, Uremia, Alcohol, Absinthe, Lead, Mercury, etc.
ORGANIC BRAIN LESIONS
Apoplexy, Meningitis, Intracranial Growths
STOKES-ADAMS DISEASE
MALINGERING
DISSEMINATED SCLEROSIS
CHART 15
1. Are certain cases of syphilitic epilepsy really cases of
Jacksonian epilepsy? As a matter of nomenclature, Jacksonian
cortical epilepsy is usually the result of a focal and
circumscribed irritative lesion in the cortex. Gumma, local
syphilitic meningitis, and syphilitic vascular lesions, as well as
scars consequent upon the latter, are among the causes of
Jacksonian epilepsy, along with such other focal lesions as
trauma, tumor abscess, tubercle, and the like. Even non-syphilitic
Jacksonian epilepsy has been observed from time to time in cases
of diffuse intracranial pressure. Jacksonian attacks also have
been found in so-called genuine epilepsy. Accordingly, we must not
conclude from the occurrence of Jacksonian convulsions, even
though in a proved syphilitic case, that the convulsions in
question are surely due to a focal lesion, for they may be due to
diffuse syphilitic lesions.
2. What is the significance of aphasia in Agnes O’Neil? Aphasia is
not a characteristic symptom in ordinary Jacksonian epilepsy, but
the aphasia is another sign of focal lesion and forms an added
argument against the diagnosis of genuine or idiopathic epilepsy.
See also discussion of aphasia in paretic neurosyphilis under Case
Levenson (22).
3. What is the behavior of the serum W. R. and the spinal fluid W.
R. under systematic treatment? Sometimes, as in this case, the
serum W. R. remains positive and the fluid W. R. becomes negative;
but in other equally well-defined cases, the reverse holds true,
and the serum W. R. reaction becomes negative whereas the spinal
fluid reaction remains positive. The obvious conclusion is that we
cannot always be sure even by faithful tests of either the serum
or the fluid alone, whether the treatment has succeeded in
abolishing the laboratory signs.
4. Can this case be regarded as one of cure? Not by the definition
adopted in this book or by the syphilographers who take into
account not only the nervous system but the body which contains
it. To be sure, the spinal fluid of Agnes O’Neil is now entirely
negative and she is clinically free from symptoms; yet from the
broad standpoint of syphilis therapy in general, this patient is
not cured, as is evidenced by the positive serum W. R.
=PARETIC NEUROSYPHILIS (“general paresis”) is often marked by
SEIZURES.=
=Case 20.= Lester Crane, a plumber, 37 years of age, came to the
hospital with a slow and defective speech. Moreover, there seemed to be
some mental disorder since his answers to questions were not always
relevant. It appeared that he was seeing bugs on the wall.
=Physically=, Crane was a well developed and nourished man, with
overactive knee-jerks and a Babinski reaction on the left side.
It developed that there was an impairment in hearing. The pupils reacted
well both to light and to distance. The patient was very restless and
smiled in a silly fashion. His memory was decidedly defective in all
spheres, and he was very slow in the intake of ideas.
The plumber’s wife said that, at about the age of 23 or 24, he had a
spell of confusion lasting two or three days, with peculiar conduct,
unintelligible talk, and a good deal of weeping. The medical diagnosis
at that time took into account the fact that Crane was a plumber and was
“lead encephalopathy.”
However, according to his wife, Crane had acquired chancre at about 26
years, was treated mercurially for about three years and declared well.
He had remained well up to about 18 months before entrance, when,
without previous warning, the patient had a convulsion with the
continuous movements for about half an hour. He was semi-conscious for
about 18 hours and vomited continuously. There was amnesia for the whole
affair on regaining consciousness. In a week’s time, Crane was entirely
well. But six weeks later there was another convulsion. Upon removal to
a hospital, the diagnosis of general paresis was made, and the patient
was given the Swift-Ellis intraspinous treatment. This seemed to be very
successful, and the patient discontinued treatment after 14 weeks
(during which time there had been seven treatments) on the ground that
he was entirely well.
However, after discontinuing treatment, there was another convulsion in
about a month, and further convulsions occurred once a month. For six
months, however, the patient took no treatment, but finally returned to
the hospital and was given mercury. This treatment appeared to suspend
convulsions again for three months, but at the expiration of six months,
the patient had three convulsions in one day, and several more during
the following days. After the last of these convulsions, there had been
numbness on the right side of the body and considerable headache.
The diagnosis of PARETIC NEUROSYPHILIS (“general paresis”) is borne out
by the laboratory tests. The W. R. of the blood serum was, to be sure,
negative, but the W. R. of the spinal fluid was positive, and there was
a “paretic” type of gold reaction, together with other laboratory signs.
The case well demonstrates that group of paretic cases in which
convulsions periodically occur, leaving the patient worse after each
convulsion. Treatment with salvarsan was instituted, and mercury and
iodid was given by mouth. During the period of eight months which have
now elapsed since the beginning of this treatment, there have been no
convulsions; there has been a great improvement in the memory, the
hearing has improved, the W. R. in the spinal fluid is much less
intense, the gold sol test has become negative, and the other tests are
all less intense.
The patient, however, has not been entirely well, for in place of the
generalized convulsions, he has had minor seizures, beginning as a rule
with a tingling sensation in the right hand, extending up the arm, down
the trunk and leg, and through the right side of the face, with a bitter
sensation on the right half of the tongue. The patient maintains that
this sensation is absolutely confined to the right half of the body (in
this connection we may recall case Morton (1), in which there was also a
hemiplegia together with other apparently hysterical symptoms at several
times during the long course of a disease with abundant structural
correlations). During these minor seizures, the patient is unable to
talk, although he does not lose consciousness and is entirely aware of
everything going on about him. These attacks have of late been growing
somewhat less frequent.
=LOSS OF DEEP REFLEXES=
NEUROSYPHILIS
NEURITIS
(alcohol, diabetes, diphtheria, lead, arsenic, tubercle,
cachexia, etc.)
Peripheral nerves sensory or motor
PERIPHERAL NERVE PALSIES
TEMPORARILY FROM COMPRESSION BY TOURNIQUET
FRIEDREICH’S ATAXIA
SUBACUTE COMBINED DEGENERATION OF POSTERIOR AND LATERAL COLUMNS
Posterior column disease
FOCAL LESION IN GRAY MATTER OF CORD
INFANTILE PARALYSIS (ACUTE ANTERIOR POLIOMYELITIS)
PROGRESSIVE MUSCULAR ATROPHY
(chronic anterior poliomyelitis)
Anterior cornua of cord
AMYOTROPHIC LATERAL SCLEROSIS
SYRINGOMYELIA
THROMBOSIS OF ANTERIOR SPINAL ARTERY
LANDRY’S PARALYSIS
Anterior cornua and peripheral motor nerves
MYOPATHIES
(pseudohypertrophic and atrophic types)
MuscLe itself
AMYOTONIA CONGENITA
FAMILY PERIODIC PARALYSIS
(during attacks)
INCREASED INTRACRANIAL PRESSURE
(especially hydrocephalus and tumors of posterior fossa)
PNEUMONIA
IMMEDIATELY AFTER ATTACK OF MAJOR EPILEPSY
(post-epileptic coma)
TOXIC COMA
(uremia, morphine, etc.)
DURING SPINAL ANESTHESIA
COMPLETE TRANSVERSE LESION OF CORD
PURVES STUART
CHART 16
1. What is the cause of the negative serum W. R.? It is claimed that
3 to 5% of all cases of general paresis yield a negative blood
serum. In this particular case, there had been considerable
treatment, including some Swift-Ellis treatment, so that it may be
that this treatment had reduced a formerly positive blood serum W.
R. to a negative one.
2. What is the nature of the typical seizures of general paresis?
The most frequent seizures are epileptiform and bear a general
resemblance to cortical epilepsy; but more rarely these seizures
resemble the ordinary epileptic attack or consist of a violent
general shaking of the whole body. A variety of initial minor
disorders usher in the attacks: the temperature is often
increased. The attacks are over after one or at most after a few
hours. Kraepelin speaks of one that lasted 14 days. Sometimes a
_status paralyticus_ develops, suggestive of the _status
epilepticus_. Another rarer form of characteristic seizure is the
apoplectiform, which can hardly be told from an ordinary stroke,
and may be followed by the usual post-apoplectic phenomena. A good
many of the strokes leading to sudden death in middle life are
probably cases of neurosyphilis although often set down as early
arteriosclerosis of a non-syphilitic nature. Besides the
epileptiform and apoplectiform seizures, there are certain
seizures of a less definite and complete nature, ranging from
simple fainting spells, dizzy spells and petit mal attacks, to
various special forms of irritative muscular contractions and
temporary speech disorders. Sometimes these attacks occur with
complete preservation of consciousness. Transient paresthesias,
visual field defects, and especially attacks of vomiting, which,
according to Kraepelin, may precede paresis by years (of course in
this connection gastric crises of tabes must be thought of), may
be counted as sensory seizures.
3. What is the proportion of paretic cases developing seizures?
Figures vary from 30 to 90%. According to Kraepelin, seizures
occurred in 30 to 40% of his cases at Heidelberg; he was of the
impression that treatment in bed had reduced the number of
seizures. 65% of paretics admitted to Munich (under very free
conditions of admission) were determined to have shown seizures
before their admission to the hospital. Seizures are said to be
somewhat more frequent in men than in women. These paretic
seizures are not due to either hemorrhages or vascular plugging—at
least in the vast majority of cases—and must be ascribed to the
effects of microscopic injuries.
4. What is the effect of seizures upon the future course of paretic
neurosyphilis? The current idea as expressed, for example, by
Mercier, is that “immediately after each crisis the patient is
much worse than he was before it, and thereafter there is some
improvement, but he never improves up to the point at which he was
before the occurrence of the crisis.” That is, “The course of the
disease is one of sudden plunges, each deeper than the last, each
followed by a gradual recovery that is less complete than the
recovery from the previous plunge.”
5. During what period of the disease are seizures most common? Late
in the disease many cases have convulsions, even though there were
none for the first year or two. In other cases the convulsion is
the first indication of paresis.
=DIFFUSE (non-paretic) NEUROSYPHILIS (“cerebrospinal syphilis”) is
often marked by APHASIA.=
=Case 21.= Martha Bartlett, a woman of 40 years, was brought to the
Psychopathic Hospital aphasic, or at least unable to talk distinctly
enough to be understood, or even to give name and address. The police
had found her wandering aimlessly about the streets. Although she was
well-dressed, she was mud-bespattered and apparently had not changed her
garments for several days. It shortly developed that the patient,
although unable to express herself either in words or by writing, could
understand everything that was said to her and could indicate by the
monosyllables _yes_ or _no_ whether she agreed or disagreed with
statements made. It was thus determined that she was pretty well
oriented. She was able to understand both speech and printed words.
Although she approximated more than is at all common a pure type of
_motor aphasia_, it appeared that there was a slight involvement on the
sensory side, especially in the sphere of visual imagery.
=Neurologically=, the patient showed moderate strabismus, slight
deviation of the tongue to the right, and considerable tremor on
protrusion of the tongue. The right side of the palate hung lower than
the left. The ankle and arm reflexes were possibly more active on the
left side, and the left grasp was somewhat better than the right. Both
knee-jerks were active, but again the reflex on the left side was more
active than the right. No other abnormalities of reflex were determined.
There was no Rombergism but the gait was somewhat ataxic. For the rest,
the physical examination was normal. The blood pressure was 120
systolic, 85 diastolic.
=CONDITIONS IN WHICH SPEECH DEFECT IS FOUND=
NEUROSYPHILIS
HYPOGLOSSAL PARALYSIS
FACIAL PALSY
PARALYSIS OF PALATE (POST-DIPTHERITIC)
BULBAR PALSY
PSEUDOBULBAR PALSY
MYOPATHY—FACIO-SCAPULO-HUMERAL TYPE OF LANDOUZY AND DEJERINE
MYASTHENIA GRAVIS
FRIEDREICH’S ATAXIA
LARYNGEAL TABES
ALCOHOLIC INTOXICATION
POST HEMIPLEGIC
LENTICULAR DISEASE
BILATERAL ATHETOSIS
MULTIPLE SCLEROSIS
DEAF MUTISM
PARALYSIS AGITANS
CHOREA
STAMMERING
TICS
HYSTERICAL APHONIA
CHART 17
The ready suspicion was that the case was one of apoplexy of slight
degree with post-apoplectic phenomena. Upon investigation, this
suspicion was confirmed since it appeared that Mrs. B. had been
apparently quite well until about six months before admission, when
without particular warning she began to act strangely and promptly fell
into a series of convulsions. These convulsions would begin with
twitchings of the face, and then spread throughout the body. There would
be a period of unconsciousness for two or three hours. It is not certain
how many of these convulsive seizures the patient had. At all events she
is reported to have recovered therefrom completely, remaining well for
three months; whereupon, suddenly, while visiting a friend, she suffered
a paralysis of the left side of the body. She remained dazed and had
hospital treatment for about a week. Ever since this left-sided
paralysis, the aphasic condition above described has persisted.
Such a phenomenon has often been dismissed in the past as due to an
early arteriosclerosis, but most neurologists and internists of today
would look beyond the diagnosis of mere arteriosclerosis and consider
syphilis. The only suggestive feature in the case, aside from the
post-apoplectic reflex disorder and spastic phenomena, is the
irregularity and diminished light reaction of the pupils. Our suspicions
were confirmed by the positive serum W. R. The W. R. of the spinal fluid
proved, however, to be negative. There was a moderately strong gold sol
reaction of the syphilitic type. There was a slight excess of albumin,
and there was an exceedingly slight amount of globulin. There was but
one cell per cmm.
On the whole, it would seem best to consider the case of Mrs. Bartlett
to be one of CEREBRAL ARTERIOSCLEROSIS OF SYPHILITIC ORIGIN, and a case
in which there is no evidence of meningitis or meningoencephalitis.
1. What is the explanation of the negative spinal fluid W. R.? It
may be that none of the W. R. producing bodies have gone over into
the spinal fluid. It has been shown by the work of Weston that the
W. R. producing body is not identical with the bodies responsible
for the other tests in cerebrospinal syphilis. Moreover, it has
been clearly shown that these several tests of the spinal fluid do
not run at all parallel with one another. Especially is it true
that the chemical tests do not correspond at all with the degree
or nature of the pleocytosis. On the whole, when involvement of
the nervous system is entirely vascular, it is not only
theoretically proper but also practically common, to find a spinal
fluid negative to several tests.
2. Omitting consideration of the syphilitic gold sol of this case,
what conclusion could be drawn from the albumin and globulin
findings? It would not be warrantable to assume syphilis since it
is a common finding after cerebral hemorrhage due to
non-syphilitic arteriosclerosis to find excess albumin and also
globulin in the spinal fluid. Occasionally, also, pleocytosis
occurs in cases of cerebral hemorrhage even when the hypothesis of
an active meningitis can be excluded. We may recall in this
connection the pleocytosis in so-called meningitis sympathica of
certain brain tumors. (See also the case of Milton Safsky (48), a
case of brain tumor in which there was an excess of albumin, a
large quantity of globulin, and a pleocytosis of 146 cells per
cmm.)
3. What can be expected from treatment in these cases of vascular
cerebral syphilis? The condition offers very little opportunity
for therapeutic results. However, antisyphilitic therapy is
indicated to prevent if possible further progress of the lesions.
Since the lesions are, however, vascular, and since it must remain
a question how far these vascular lesions are due directly to
spirochetal action, and since in any event it may be difficult to
reach the spirochetes thus active, perhaps it is best to place
most reliance on potassium iodid. In any event, potassium iodid
should be given. Salvarsan and mercury are also indicated. It is
common to warn against administration of large doses of salvarsan
in this type of case on the ground that further vascular ruptures
may be produced. (See Friedberg, 108.)
4. If we conclude that the aphasia of the Bartlett case is due to
vascular disease, can we conclude a relation between this vascular
disease and vascular tension? It is not safe to draw such a
conclusion. The Bartlett case itself showed low blood pressure. To
be sure, some cases of neurosyphilis show high blood pressure from
which one draws the _à la mode_ clinical conclusion to the effect
that the kidneys are probably involved in the arteriosclerosis;
but other cases do not show a high blood pressure but may in fact
show a low blood pressure. The vascular disease doubtless
responsible for the aphasia in the Bartlett case is probably not
at all an effect of blood pressure conditions, but is, on the
contrary, an effect of local syphilitic vascular lesions.
=PARETIC NEUROSYPHILIS (“general paresis”) is often marked by
APHASIA.=
=Case 22.= Meyer Levenson, a traveling salesman of 36 years, had for the
last two or three years been undergoing a change of disposition, quite
interfering with his work. He had begun to take unreasonable aversions
to people, had become irritable and emotionally depressed, and often
fell to weeping without cause.
About nine months before hospital observation, it seems that a
trunk-cover had fallen on Levenson’s head, and there is some question as
to whether he did not have a convulsion at that time. However, a month
later he had a definite seizure, followed by speech disorder, a slight
paralysis, and a staggering gait. Four weeks later, however, he had
gotten over these post-convulsive difficulties and had gone back to
work.
At his work, he became tired easily, his gait and speech did not seem
entirely normal, and there was a considerable memory disorder. After
five more months, another attack of a convulsive nature, with twitching
of hands and face and tongue-biting occurred, and the attending
unconsciousness remained for two days. Again improvement followed,
though without ability to return to work. Four (?) months later there
were several severe convulsions and Levenson would remain unconscious
for a day or two at a time. Restlessness, irritability, and irrational
talking followed.
=Physically=, the patient was fairly well developed and nourished; blood
pressure 168 systolic, 68 diastolic; pupils reacted very sluggishly to
light. There was a marked motor aphasia, which the patient recognized as
a speech difficulty. On the whole, however, Levenson was very euphoric
and was entirely sure that he was improving and would surely get well.
Shortly after entrance, Levenson had a severe convulsion, with
unconsciousness. The movements were mainly on the right side of the
body, and there was a post-convulsive weakness of the right side for
several days, followed by a slow recovery of strength.
The course of the disease—convulsions followed by improvement—is very
characteristic of a paretic onset. The =laboratory findings= were in all
respects confirmatory. It was rather striking that a permanent _motor
aphasia_ followed the convulsions in this case, since the seizures of
paresis do not in the vast majority of cases leave permanent paralyses.
The course of the disease continued to show convulsions, which would in
each instance leave him at a lower terrace of capacity than had been
before shown. The patient died four years after the onset of symptoms of
a general asthenia. With the exception of the permanent motor aphasia,
this case might be regarded as a fairly typical one of general paresis.
1. What is the general nature of speech disorder in paretic
neurosyphilis? Speech disorder is, along with the pupillary
changes, one of the most important clinical symptoms in paretic
neurosyphilis. There are aphasic and articulatory disturbances.
The aphasia that accompanies paretic seizures is of a transient
nature as a rule. A case with such long-standing motor aphasia as
shown by Levenson is not common. Paraphasia, with incorrect naming
of objects, may last longer. The so-called “sticking” phenomenon
is often observed.
Word-deafness is said to be rarer but is difficult to test on
account of the patient’s dementia. Agrammatism (incapacity to form
correct sentences) is sometimes observed. But the most
characteristic disorder is in the syllabic composition of words.
Syllables are left out (“medaltricity” for medical electricity),
or fused (“exity”), or doubled (“electricicity”). Besides the
central speech disorders of which the above are examples, there
are disorders in articulation, which at first occur as a
consequence of paretic seizures or in states of excitement, but
later become permanent. These are divided into paretic and ataxic
disturbances.
2. What is the structural basis of these forms of aphasia? It is
believed that they are due to microscopic changes, not to coarse
destructive lesions.
[Illustration:
BROOKLINE, MASS.
]
[Illustration:
BROOKLINE, MASS.
Mss. of Levenson, case 22. Paretic neurosyphilis. Tremor, misspelling.
Metathesis of letters (Bk, not Br) omission of letters (Book).
]
[Illustration]
[Illustration:
God save the Commonwealth of Massachusetts
Mss. of Safsky, case 48, brain tumor. Tremor not marked. Misspelling,
omission of letters. Wrong letters (h in hweth).
]
[Illustration:
Mss. of Halleck, case 31, cervical tabes. No brain disorder.
Pen-holding and bearing on difficulties. Crowding of phrases result
of ataxia.
]
[Illustration:
Mss. of Collins, case 61, paretic neurosyphilis. One misspelling
(-chussetts); not psychopathic? Characteristic tremor.
]
=REMISSIONS of identical appearance occur in PARETIC and in DIFFUSE
(non-paretic meningovascular) NEUROSYPHILIS.=
=Case 23.= Thomas Donovan, a merchant 44 years of age, acquired syphilis
according to his own story at the age of 31, and he was at that time
treated at a well-known watering-place with mercurial injections. Later
he continued treatment under his family physician, and at 34 was
pronounced cured. However, four years later—that is seven years after
his initial infection and in his 38th year—he had his blood examined and
it proved positive. He was accordingly treated by salvarsan and his W.
R. became negative. The story did not end there, however, for at 43,
mental symptoms appeared of the nature of depression and a diagnosis of
paresis was made. He was released from the institution against advice at
that time, and without treatment, made a partial recovery.
A sudden outburst of violence brought Mr. Donovan to the Psychopathic
Hospital; he was very surly, combative, and difficult to manage,
standing 6′ 2″, and weighing 210 pounds. He was oriented only fairly
well and his surliness was streaked with humor. He facetiously said that
the Psychopathic Hospital was the largest hospital in the country, and
that it was, in fact, a horse hospital; that he had come because he
liked the surroundings, not to make money; that he was the healthiest
man in the world, never having been sick; that the Psychopathic Hospital
was a club, for which you have to get somebody to propose your name.
There was amnesia and no knowledge of current events. He regarded the
food as poisoned, refused to eat, and was very irritable and untidy.
=Physically=, there were few abnormalities, but the pupils failed to
react either to light or accommodation, and the knee-jerks and
ankle-jerks were absent. There was a slight Rombergism. There was a
marked speech defect to test phrases. Both serum and spinal fluid W.
R.’s were positive; the fluid showed 41 cells per cmm., there were large
amounts of globulin and albumin, and the gold sol reaction was of the
“paretic” type.
=ATAXIA OR INCOÖRDINATION=
NEUROSYPHILIS
LESION OF PERIPHERAL SENSORY NERVES
DIVISION OF POSTERIOR ROOTS
TUMORS OR CHRONIC SCLEROSIS OF POSTERIOR COLUMNS
SUBACUTE COMBINED DEGENERATION
VESTIBULAR ATAXIA
FRIEDREICH’S ATAXIA
FAMILY PROGRESSIVE HYPERTROPHIC NEURITIS
THROMBOSIS POSTERIOR INFERIOR CEREBELLAR ARTERY
MARIE’S HEREDITARY CEREBELLAR ATAXIA
LESIONS OF CEREBELLUM, TUMORS, ETC.
WRITERS’ CRAMP
PREHEMIPLEGIA
MULTIPLE SCLEROSIS
PSEUDO-SCLEROSIS
HYSTERIA
CHART 18
=CONDITIONS IN WHICH VERTIGO IS FOUND=
NEUROSYPHILIS
HEAD TRAUMA
CEREBRAL ANEMIA AND HYPEREMIA
MENOPAUSE
ARTERIOSCLEROSIS
RENAL DISEASE
CEREBRAL HEMORRHAGE AND THROMBOSIS
INTRACRANIAL TUMORS
MULTIPLE SCLEROSIS
EPILEPSY (AURA)
TOXIC CONDITIONS:
alcohol, tobacco, constipation
PSYCHONEUROSIS
OCULAR DISTURBANCES
EAR DISEASE
MÉNIÈRE’S DISEASE
MIGRAINE
CHART 19
Salvarsanized serum was injected intraventricularly through a trephine
opening in the right frontal region. Injections were made through the
corpus callosum into the third ventricle. There was progressive
symptomatic improvement after each of four injections. In fact, after
the fourth injection the patient was allowed to leave the hospital
despite the fact that there was only a slight improvement in the spinal
fluid findings. The speech defect had entirely disappeared. (Speech
defect, according to many authorities, including Kraepelin, is of very
grave diagnostic significance.) His memory returned. Mr. Donovan is now
able to handle figures rather extraordinarily well. He now has a good
insight into his delusions and tells stories about them with great
humor.
1. What is the definition of a remission in general paresis?
Remissions form a foil to seizures; just as seizures mark a sudden
advance in the severity of the disease or may even lead to death;
so remissions may cause a sudden cessation of both mental and
nervous phenomena in the disease. Whereas the seizures occur most
often, according to Kraepelin, in the demented types of paresis,
the remissions occur in all cases except in the terminal phase.
Kraepelin quotes Hoppe as observing pronounced remissions of long
duration in 17% of male and 15% of female paretics. Gaupp observed
marked improvement in less than 10%, and very marked improvement
indeed in only 1% of his cases. Kraepelin states that such
improvements are most frequent in agitated and especially in
expansive forms of paresis, and that they are rarer and less
complete in the depressive and demented forms. Sometimes the
improvement occurs over night, although the full extent of the
remission becomes complete only gradually, perhaps in the course
of months. The sensorium clears, the disorientation disappears,
the delusions retreat, and the former delusions are treated as
dreams and imaginations. There is often a good deal of persistent
uncertainty as to events during the height of the disease. The
nervous disorders are far more obstinate than the mental. Still,
both speech and writing may often greatly improve.
Cotton in New Jersey found, among 127 cases of paresis
diagnosticated by modern methods during seven years, that
remissions occurred in but five, or about 4%, lasting from a half
to three years.
2. Does a remission ever amount to a cure? The classical case quoted
in this connection is one observed by Tuczek. This case developed
a picture of paresis in 1876, at the age of 36; and a remission,
or cessation, of symptoms, occurred in 1878; but in 1883, at 43
years, the patient developed a tabes without any trace of mental
disorder, which tabes gradually advanced. By the middle of 1898,
when the patient was 58, certain symptoms of excitement and
confusion occurred, which led to death with dementia, 22 years
after the beginning of the disease. Nissl pronounced the cortex to
be undoubtedly the characteristic cortex of a paretic. This
observation seems to indicate that a clinical remission tantamount
to a clinical recovery may occur without the death of the
spirochetes engaged. This observation is to be held in mind in
connection with all therapeutic work with neurosyphilis.
Nonne states that during his clinical experience of 19 years he
had followed 10 cases of paresis with apparent recovery; but of
these ten cases, four had to be thrown out by Nonne because the
apparent recoveries turned out to be only long and almost complete
remissions, finally issuing in characteristic dementia. Of the
remaining six cases, perhaps two should hardly be counted as
paretic and Nonne rather preferred to term them cases of
syphilitic dementia in the sense of a non-paretic cerebral
syphilis. At the end, therefore, of his review of observations,
Nonne found himself with four cases of true recovery from paresis.
Spielmeyer holds that there is no theoretical reason why paresis
might not be cured, since all the different changes that have been
described in the disease can be halted, and many of them can be
repaired. In particular, he reminds us that the acute infiltrative
process, the neuroglia reaction, and the phagocytic action of the
large mononuclear cells are distinctly removable processes. (See
discussion below under Section V, for apparent cures and
remissions occasionally secured under treatment.)
=REMISSIONS of identical appearance occur in PARETIC (“general
paresis”) and in DIFFUSE (non-paretic) NEUROSYPHILIS.=
=Case 24.= Michael O’Donnell, a laborer of 48 years, came home, one day,
at 5:30, complaining of severe headache. His wife told him he should lie
down and, taking him by the arm, tried to help him to the bed. At this
moment, O’Donnell lost control of both left arm and left leg, and fell,
unable to move but with consciousness preserved. The wife noted that the
left side of his face was drawn up and that he drooled. He was at once
carried to a general hospital, remaining there for about three weeks,
talking at random in a delirious manner and tied in bed. Two
intraspinous injections of salvarsan were given, and O’Donnell showed
considerable improvement and went home.
However, upon his return from the hospital, he became very wilful, would
not remain in bed, and on one occasion actually took the mattress from
the bed, carried it to another room, and then returned to his own room
and slept upon the springs. He became irritable and emotional, insisted
upon going to the hospital, did not go there but upon returning home
insisted that he had been there. That night, O’Donnell left the house
only partly dressed.
It appears that O’Donnell had been excessively alcoholic, but that
before August 15, when he sustained the left-sided hemiplegia above
mentioned, there had been no symptoms except that in February he had
once been very dizzy. It appears that there had been another dizzy
spell, three nights before the paralysis, accompanied by a fall and
unconsciousness for about 15 minutes.
=TRANSIENT OR FLEETING PARALYSES=
NEUROSYPHILIS
MYASTHENIA GRAVIS
MYOTONIA CONGENITA (THOMSEN’S DISEASE)
PARAMYOTONIA CONGENITA
MYOTONIA ATROPHICA
INTERMITTENT CLAUDICATION
OCCUPATION NEUROSES
FAMILY PERIODIC PARALYSES
ETANY
EPILEPSY MINOR
HYSTERIA
MULTIPLE SCLEROSIS
APOPLEXY
CEREBRAL THROMBOSIS
CHART 20
O’Donnell was brought to the Psychopathic Hospital some six weeks after
the paralysis, complaining merely of a slight headache and desirous of
treatment. There were no mental symptoms of any sort. =Physically=,
O’Donnell was in general not abnormal (there was a slight pre-systolic
murmur and a blood pressure of 190 mm. systolic). The pupils were
slightly irregular, the left larger than the right; both reacted
sluggishly. Both ears were moderately deaf; the tendon reflexes of the
left arm and leg were somewhat more lively than those on the right. The
systematic =neurological= examination otherwise revealed no
abnormalities. The urine was negative. The serum W. R. was positive but
the spinal fluid reaction was negative. There were but 2 cells per cmm.,
and there was a very slight trace of albumin.
1. How shall we account for O’Donnell’s transient paralysis? We
might invoke brain tumor, alcoholic pseudoparesis, or some form of
neurosyphilis. The diagnosis of brain tumor seems quite untenable
in view of the absence of premonitory symptoms and in the absence
of intracranial pressure. As for alcoholic pseudoparesis it is
true that the patient was excessively alcoholic.
However, against these two diagnoses and in favor of the diagnosis
of NEUROSYPHILIS, are the positive serum W. R. and the pupillary
reactions (although these are short of the true Argyll-Robertson
phenomenon). Dizziness with retention of consciousness and
associated with the paralyses mentioned suggests rather a
subcortical than a cortical lesion. We are inclined to regard this
lesion as probably THROMBOTIC, and to place it possibly in the
region of the internal capsule. We are inclined to regard the
phenomenon as purely vascular and as not in this case associated
with an encephalitis. We are, however, not entirely satisfied with
the diagnosis.
2. What shall be said as to treatment? A full-blown left-sided
hemiplegia may be produced even when the thrombotic lesion is
itself exceedingly small. It is common to explain this on the
basis that there is an area of collateral edema about the small
necrotic, thrombotic, or hemorrhagic area responsible for the
lesion. In short, numerous neurones are functionally rather than
structurally affected, or at all events capable of early
restitution of function.
3. What is the prognosis in such cases? It appears that now and
again patients run for several years without further trouble, both
with and without treatment. We are inclined, however, to advocate
treatment rather than absence of treatment for a variety of
reasons. In the first place, vascular lesions may at any time
become associated with meningitic lesions, and treatment by
salvarsan may perhaps be counted on to head off this process;
secondly, the treatment with iodids may possibly aid in the
resolution of a local thrombotic process.
4. What are the prodromal symptoms of cerebrospinal syphilis?
According to Nonne, headache, dizziness, sleeplessness, mental
symptoms of the irritability group, loss of capacity as to mental
work, whether severe or not, and loss of capacity for difficult
thinking; also impairment of memory. Nonne does not regard these
phenomena as characteristic of syphilitic vascular disease, and
calls attention to the fact that in every organic disease the same
subjective symptoms occur. The triad—headache, dizziness, and
impairment of memory—is for example now counted as a prodromal
symptom complex for arteriosclerotic apoplexy (Cramer). Of course,
apoplectic attacks occur without such preliminary symptoms:
particularly, according to Nonne, the nocturnal attacks.
5. Can the fleeting paralysis be of service in differentiating the
diffuse from the paretic form of neurosyphilis? Probably not. In
both forms transient paralyses occur as well as the permanent
ones. In general, however, the transient paralyses are more
frequent in paretic neurosyphilis, whereas the permanent ones
occur more often in diffuse neurosyphilis.
=There are cases of NEUROSYPHILIS in which the laboratory signs are
positive but in which there are no clinical signs or symptoms
(PARESIS SINE PARESI?).=
=Case 25.= Richard Lawlor[6] was admitted to the Psychopathic Hospital,
October 29, 1914, being sent there from a general hospital where he had
gone on account of a self-inflicted wound of the wrist, apparently made
in a period of depression with suicidal intent. Routine notes follow.
=Family History.= Paternal grandparents both died of heart disease.
Maternal grandfather died at seventy-two of dropsy. Moderately
alcoholic. Maternal grandmother died of shock at fifty-six. Father died
at age of forty, after an illness of eight years, from heart disease.
Father all his life was subject to fainting spells and headaches. The
only paternal cousin died at thirteen months of brain fever. Mother,
aged forty-seven, is, to say the least, eccentric. Says “she has several
times been given up from tuberculosis.” Two maternal uncles died of
tuberculosis, one from rupture, one from heart disease. One uncle who
“doesn’t know anything after he has a teaspoonful of liquor.” Several
other uncles and aunts whose history is not obtained. Patient is
mother’s only child. Mother was twice married. There were several
miscarriages by both husbands; patient child by first marriage.
=Past History.= Patient born thirty-two years ago, full term, normal
delivery and development. Measles, mumps, and chickenpox in childhood.
Subject to headaches since seven or eight years old. Kicked in the face
by horse at seventeen or eighteen, not considered serious. Hit by a
baseball three or four years ago, leaving him hard of hearing on left
side. Married ten years ago; no children because he says his wife needed
an operation. He denies venereal disease by name and symptoms. For past
ten years has had attacks of depression lasting but a short time, but
quite severe. Never caused him to quit work as a barber and he felt
better when working. His married life he says was fairly happy except
for his wife’s extravagances, and on this account he left her a little
over a year ago, and she has applied for a divorce, which he is willing
that she should have, but does not wish to give her alimony. He admits
moderate alcoholism.
=Present Trouble.= Patient states that since he left his wife a year ago
he has felt sorry a number of times. He has wished he had her back. He
has felt lonely. He has had six or eight periods of depression in that
time similar to those he has had for many years, lasting two or three
days, and sometimes a week. These were always precipitated by some cause
for worry. In these attacks he feels nervous, sleeps poorly, has little
or no appetite, sweats during his work and everything looks black.
Several times in these attacks he has had suicidal ideas. Ten months ago
he considered taking corrosive sublimate. For a little over a week
before entrance to hospital he had been out of work and had been
“sporting.” The day before entrance he had a telephone message from his
lawyer which upset him somewhat and he walked the floor all night. He
had just been shaving when the idea of suicide came to him. He sat down
a minute when suddenly the thought “to hell with the world” came to him;
he took the razor and slashed his wrist. He does not remember drawing
the razor across his wrist. As soon as he saw the blood he felt sorry,
called his mother, and was taken to an emergency hospital and then sent
to the Psychopathic Hospital.
=Physical Examination.= Patient is a well developed and nourished man
thirty-two years of age. Head is normal as to size and shape; there are
no scars or marks of injury. Hair and skin not remarkable in any way.
Ears negative to external examination. Teeth well kept; two missing,
several gold fillings. Tongue very slightly coated. Throat negative.
Tonsils easily visible without evidence of inflammation or exudation.
Neck, no thyroid enlargement, no abnormal pulsations, no adenopathy.
Chest, symmetrical, expansion good, resonant throughout. Breath sounds
transmitted normally. No râles or rubs heard. Heart, no enlargement or
cardiac dulness. Sounds of good quality, no murmurs heard. Rate regular.
Pulses equal, regular and synchronous, and of good volume and tension.
Systolic blood pressure 130, diastolic 65. Abdomen, flat, soft and
tympanitic throughout; no masses; no tenderness. Liver edge not felt,
below costal margin. Spleen not palpable. Extremities negative, except
for incised wound on left wrist.
=Neuromuscular Examination.= Pupils are large, round, regular, equal and
react readily to light and accommodation. No nystagmus, strabismus or
ptosis. No weaknesses or paresis of facial muscles. The tongue projects
medially and shows no tremor. The triceps and biceps reflexes are
readily elicited, and are quite active, as are the knee-jerks and
ankle-jerks. On one occasion it was thought that the tendon reflexes
were slightly more active on the left than on the right. This was never
confirmed; always afterwards found equal. There was no tremor of
extended hands. Abdominal reflexes not elicited. Cremasteric present on
both sides. The plantar response is flexor. There is no Babinski, Gordon
or Oppenheim. No Romberg. Coördination tests well performed. No speech
defect. No sensory disturbances. Urine examination negative.
Wassermann reaction in the serum: Positive, with cholesterinized
antigen; negative, with syphilitic fetal liver antigen.
Wassermann reaction in fluid positive on two occasions. Examination of
spinal fluid, November 4: globulin +++, albumin ++, 100 cells per cubic
millimeter; large lymphocytes, 8 per cent; small lymphocytes, 90 per
cent; plasma cells, 0.7 per cent; endothelial cells, 1.3 per cent.
November 11, globulin +++, albumin +++, cells 18 per cubic millimeter.
November 26, globulin ++, albumin ++, cells 92 per cubic millimeter;
large lymphocytes, 13.1 per cent; small lymphocytes, 82.1 per cent;
plasma, 1.2 per cent; endothelial, 3.6 per cent.
Gold sol, November 4, 5555432100.
Gold sol, November 26, 3332100000.
=Mental Examination.= On entrance to hospital patient seemed slightly
depressed and a bit irritable. This condition lasted two days, after
which he was agreeable and apparently entirely over his depression. Even
during his mild depression, however, he talked freely. There was no
evidence of retardation. He told his story readily. Orientation was
intact. Memory excellent. Educational knowledge well retained. There was
no evidence of any hallucinations or delusions.
1. Was Richard Lawlor insane?
There was, then, on the mental and physical examination nothing to
make a definite suggestion of a psychosis, and the most one could
think of was a psychoneurosis or a cyclothymia of at least ten
years’ duration. The findings in the cerebrospinal fluid and the
Wassermann reactions, however, give us material for thought.
Certainly one cannot call the man insane; all who saw him agreed
on this point.
2. If Richard Lawlor should some day develop mental symptoms, what
would be the genesis of the new psychosis? Though writers such
as Fildes and McIntosh, and Swift, have suggested an
anaphylactic or hyperallergic explanation for the development of
symptoms after a normal interval; such a hypothesis could hardly
obtain in the present case. The hyperallergic hypothesis for the
development of tertiary neurosyphilis would run to the effect
that in the secondary stages there had been a definite disease
of the nervous system, which, however, absolutely cleared up,
leaving no inflammatory vascular or parenchymatous relics of its
existence. Nothing would on this hypothesis remain except a
hypersensitisation of the tissues. In some later period of the
now clinically normal person, one or more spirochetes from a
lesion outside the nervous system are carried into the nerve
tissues and there set up an anaphylactic or hyperallergic
reaction. It is obviously difficult to prove the correctness or
incorrectness of the hyperallergic theory without numerous
examinations of the spinal fluid, in clinically normal persons
after the secondaries have passed. The present case, so far from
demonstrating a normal fluid, demonstrates a highly pathological
fluid, even though there are absolutely no clinical symptoms
which could be regarded as of nervous origin. The burden of
proof at the present time would seem to lie with those who claim
hyperallergy in neurosyphilis. We prefer on present evidence to
think that at the conclusion of the secondaries a disease
process often remains in the nerve tissues despite clinical
quiescence.
3. What is the prognosis in the case of Richard Lawlor? The
prognosis _re_ neurosyphilis is doubtful. We have, however, boldly
termed the condition _PARESIS SINE PARESI_, meaning thereby to
suggest that the patient is in considerable danger of the
efflorescence of a true diffuse or paretic neurosyphilis. We have
no means of telling, however, whether the positive symptoms would
be those of a paretic or a non-paretic neurosyphilis. As data
accumulate regarding these cases of _paresis sine paresi_, we may
be able finally to come upon some case in which trauma shall bring
out the clinical symptoms of neurosyphilis. For discussion of this
matter, see the case of Bessie Vogel (52) in Part III of this
book.
4. Should Lawlor have been brought to a psychopathic hospital? It is
a safe working rule to have any person who attempts suicide
observed. A large percentage of suicides occur in psychotic
individuals and a suicidal attempt is not infrequently the first
recognized abnormality. Immediate observation is a necessary
safeguard against another more successful attempt.
=Demonstrates SYMPTOMS and LESIONS of PARETIC NEUROSYPHILIS
(“general paresis”). Autopsy.=
=Case 26.= John Morrill, 49, an operative in a mill town in Essex
County, Mass., was described as a “Saturday night and Sunday drinker,”
with a history of very serious long sprees at the age of 43. It seems
that he had had what was called “sciatica” at 35, and was treated in
hospital for seven weeks at that time. The nature of this sciatica is in
doubt, but there was a history of syphilitic infection at 36 years (scar
of glans).
Morrill had been married twice, and two of the children were dead; one
daughter was described as “very nervous,” but there were four children
under ten years of age, all regarded as perfectly healthy.
Morrill had been a mill operative of average capacity, was industrious,
and had supported his family despite alcoholism. The syphilis had been
treated with reasonable thoroughness.
Aside from alcoholism, there had been no symptoms up to two months
before admission to Danvers Hospital. Then there had been insomnia,
fatigue, agitation, eruption on foot, loss of ten pounds in weight,
hypochondriacal fears, apprehensiveness for the future of the children,
incoherent talk; and just before admission, his talk was described as
foolish. He had taken to running away and hiding in bushes by a pond and
in the cellars of other people’s houses.
The patient was of medium height and weight, with thin grayish hair and
grayish irides; musculature was slender. The face was blank in
expression, the teeth poorly preserved with atrophy of gums, the tongue
coated, and the breath foul. There was a gummy secretion of the eyelids,
an area of brownish branny eruption over both clavicles, a number of
depressed scars over the limbs and back, and another area of scaly
eruption on the right heel and the sole of the foot. The heart area was
increased, and the sounds were faint at the base, with the first sound
accentuated at the apex. The urine showed a trace of albumin.
=Neurologically=, the Romberg position was maintained with a general
tremor and fluttering of the eyelids. In complicated movements, the
patient was slightly ataxic. The pupils were irregular, the left being
much larger than the right. There were no light reactions to be obtained
in window light. The reaction to accommodation was present, though
slight. Vision was poor, ¼-inch capitals could not be read by left eye
at reading distance. The knee-jerks were diminished equally; the
Achilles jerks were absent; the other reflexes were normal. Upon the
sensory side, the patient gave a history of pains in the legs at
irregular intervals for several years. These pains he described as of a
darting character. There was little or no sensory disorder, although the
outer surface of the right leg required a deeper pressure to elicit
sensation. There were no disorders of muscle sense.
If Morrill was to be trusted, he had been born in Ireland, and had come
to the United States at the age of 17. He married at 18; there had been
seven pregnancies by the first wife, with one stillborn child; one child
had died at five weeks. The four children by the second wife were
healthy. The first signs of neuritis had occurred at 45 and had received
the diagnosis neuritis, although no connection between the neuritis and
the syphilis had been noted.
The patient entered the hospital July 26, 1904, and was discharged,
improved, January 5, 1905. He returned a little more than a year later,
January 15, 1906, and died March 21, 1906. The total duration of the
disease from the onset of mental symptoms may therefore be stated as
somewhat under two years. When the patient appeared at the hospital the
second time, he showed a positive Romberg sign, an unsteady gait, an
ataxia that still was moderate, and somewhat more marked tremors,
involving fingers, tongue, and face. He was now unable to read ½-inch
type with the left eye. The knee-jerks, formerly diminished, were both
exaggerated, the left slightly more so. The Achilles reaction, not
obtained formerly, now appeared on the right side. The pupils reacted as
before. The sensory loss had become more marked, since sharp and dull
points could hardly be distinguished. Deep pinpricks were not felt in
the leg, and heat could not be told from cold.
The speech in 1904 had been somewhat defective (“truly rural” rendered
as “tooly lualal,” “sifted soft thistles” as “thoft thsistles”), and
there had been little further development of the speech defect. The
handwriting had lost appreciably in legibility and had become much more
tremulous. During the first period of hospital observation Morrill had
what might possibly have been visual hallucinations, but it was
impossible to tell whether his story of seeing his wife and children
trying to get in through the window was hallucinatory or a matter of
fabrication. Memory was decidedly imperfect and few details of recent
events could be produced. The association of ideas was almost a
so-called “flight” of apprehensive, fearful ideas, loosely connected,
incoherently expressed, and dealing chiefly with his work and his
children. Judgment was imperfect; the height of the room was estimated
as 24 feet, but the height and weight of persons were estimated with
fair accuracy, and also the length of small objects, whose lengths were
doubtless remembered rather than estimated. The estimate of time
elapsing during a medical examination was accurate, but the estimate of
longer durations involving over-night memories was hopelessly imperfect.
Emotionally, there was a dulling of sensibility, an appearance of
suspicion and apprehensiveness; the patient fancied himself to be in a
hopeless condition as a result of syphilis, but at the same time
accompanied his statement of his hopelessness with laughter. A sample of
his hypochondriacal ideas: “I am all gone; I am good for nothing; I am
all gone now; I can’t drink now; can’t write or talk at all; worse than
when you saw me first; nothing in my inside; all wrong through me again;
I aint got no swallow now; I can’t die even; my heart aint much good; I
can’t hear it beat; I don’t think it flutters; no life in these hands;
they are all cold and dead” (pointing to his arms and moving them
about). During such a portrayal the patient laughed in a silly way.
During the second hospital stay, Morrill was at first restless,
sleepless, profane, imperfectly oriented for time, possibly for place,
and also for the attendants. A few weeks later he became stuporous and
confused, and his feebleness and physical exhaustion were finally ended
by death, March 21, 1906. Death was preceded by a semi-comatose
condition; a left otitis media had developed.
At the =autopsy=, it appeared that death was due to an early
bronchopneumonia associated with acute splenitis and doubtless related
to the otitis media of the left side. The body at large showed, aside
from these acute lesions, a few chronic lesions, including slight scars
of the left apex, and chronic adhesive pleuritis, chronic diffuse
nephritis, and aortic and coronary syphilis. The aorta showed slight
linear and nodular markings, with a single small dark ulcer in the upper
thoracic region, but the aorta did not show the characteristic scarring
which syphilitic aortas often show. The femoral marrow was of a dark red
chocolate color. The thyroid appeared to be smaller than normal. A
slight sacral decubitus had developed.
The description of the head (E.E.S.) is given in full on account of the
encephalitic lesions shown. These encephalitic lesions may be summed up
as follows:
Local cerebral =atrophy= and =sclerosis= of the frontal, orbital,
and central regions, especially of the left operculum and left
supramarginal gyrus.
Extension of sclerosis to hippocampal gyri with effacement of
substantia reticularis alba.
Slight chronic internal =hydrocephalus=.
Granular =ependymitis= (especially of floor of 4th ventricle).
Compensatory edema of frontal and central pia mater.
Cerebellar sclerosis (culmen monticuli, lobus culminis, lobus
cacuminis).
Spinal sclerosis (grossly evident in the posterior columns of the
upper thoracic region and of the lumbar enlargement).
The details are as follows:
Head:—Bald on top. Hair =gray=. Scalp normal. Calvarium thin, deeply
excavated by arachnoidal villi to right of vertex. Diploë absent.
Dura closely adherent in bregmatic region. Dura of usual thickness.
Sinuses contain cruor clot. Arachnoidal villi slight. Pia mater hazy
and over sulcal veins porcelain white over all of vertex except
occipital poles and over flanks (notably left). Thickened also
around circle of Willis, over culmen monticuli and in posterior
cerebellar notch. Edema of pia corresponding to atrophy of frontal
and central regions. Cerebral atrophy most marked in orbital
surfaces of both frontal lobes, in left area of Broca, and in left
supramarginal region. The ascending branch and the ascending ramus
of the posterior limb of the left Sylvian fossæ both readily admit
the thumb by reason of atrophy of adjacent substance. Induration
corresponds closely with atrophy, but is not more marked about the
left Sylvian fossa. There is sclerosis of both hippocampal gyri,
with loss of the substantia reticularis alba. The culmen monticuli
and lobus culminis are firmer than the clival regions, and the lobus
cacuminis is again slightly firmer than the clival region.
Cerebellum a little softer than usual. Pia strips with usual
readiness from all regions. The subpial region of the frontal lobes
is a trifle grayer than that of the rest of cerebrum. Ventricles
slightly dilated. Surfaces evenly sanded. Floor of fourth ventricle
shows numerous coarse, closely set granules. Brain wt. 1200 grms.
Cord shows a slight increase of consistence over one or two upper
thoracic segments and in lumbar enlargement corresponding with a
slight graying out of posterior columns. In places there is a
suggestion of graying out also in lateral columns. A few calcified
plaques in posterior lumbar pia.
Analysis of these details shows a number of lesions that characterize
paretic neurosyphilis (among others, granular ependymitis, frontal
atrophy, chronic leptomeningitis), but the lesions are more than merely
frontal, extending as they do back as far as the postcentral regions on
both sides, and even as far as the left supramarginal gyrus. The
cerebellar involvement although frequent, can hardly be said to be
characteristic in paretic neurosyphilis. The spinal involvement is
characteristic of a case which is probably to be regarded as one of
taboparesis; that is, of paretic neurosyphilis following a number of
years after the establishment of tabetic neurosyphilis. The aorta is
almost constantly affected by sclerosis in paretic neurosyphilis. The
absence of diploë in the skull is not infrequent and the adherent dura
mater is often found.
Microscopically, the tissues showed the characteristic lesions of
PARETIC NEUROSYPHILIS; nerve cell destruction, fibrillar and cellular
gliosis, lymphocytic and plasma cell deposits about the small vessels.
1. What are the clinical evidences of syphilis outside the nervous
system? The brownish branny eruptions of the skin, the depressed
scars and the scaly eruption on right heel and sole are very
suggestive of syphilis. Such clinical evidences of syphilis are
very important in systematic examination. Although the laboratory
tests are of the utmost assistance in the diagnosis of syphilis,
the clinical signs should not be neglected, and no physician
should rest satisfied with laboratory signs alone. X-ray diagnosis
of bone conditions sometimes succeeds when all other methods have
failed.
=GUMMA of cerebral cortex verified by operation; death.=
=Case 27.= The presenting picture in the case of David Tannenbaum was
that of deep dementia, in which condition the patient was brought to the
hospital. There was a meagre history to the effect that about four
months before admission, he had lost his job in a hotel through lack of
further work. We heard that at this time he had begun to suffer with
excruciating pains in the head; at first, worse at night, later, worse
by day. It appeared that this pain, though it came and went, was chiefly
localized on the left side of the head. For a fortnight, Tannenbaum had
been dragging his legs, until finally he had become unable to walk at
all.
_Pari passu_ with these developments, Tannenbaum had become mentally
confused and irritable, and his memory had become untrustworthy. For
several days before admission, an appearance of marked dementia was
presented, with slow incoherent, or at all events, irrelevant words, and
a complete disorientation for person. However, his vision had become so
poor that it would have been hard for him to have recognized any one.
It appeared that the family history was entirely negative; that the
patient was without education but had been physically very strong, and
had been fairly successful at first in the junk business, and later in
the clothing business; but latterly he had been less fortunate in the
clothing business, and finally had to resort to work as a laborer around
a hotel.
His wife had had eleven pregnancies with but one miscarriage.
Nevertheless, out of the eleven pregnancies, there were now but four
living children.
=Physically=, Tannenbaum was a rather small man; he was flabby and
looked as if he had recently lost weight. The skin showed areas of
pigmentation on the face and sides of the neck, and some dark
copper-colored circular areas, marble-size, in the neck (syphilitic?).
There was a slight radial arteriosclerosis. The heart was slightly
enlarged with distant and indistinct sounds. There was a small
pedunculated growth on the right side of the abdomen.
The pupils failed to react to flash-light but they reacted to sunlight.
They both were slightly irregular but were equal in size, and reacted in
accommodation. There was apparently almost complete blindness and
extreme deafness. Arm-jerks and knee-jerks were absent; there was an
occasional slight response of the left ankle-jerk, but the right
ankle-jerk was absent; the left abdominal reflex was very feeble; the
right absent; the cremasteric reflexes were absent, but there were no
other abnormalities in the systematic examination. Hand grips weak; gait
awkward, with right leg held somewhat flaccidly.
It was significant that percussion over the left frontal and parietal
regions was able to elicit great pain. Either through the patient’s
deafness or through sensory aphasia, spoken language was not understood.
The serum W. R. was positive, the fluid W. R. negative.
=Diagnosis=: The clinical symptoms seem clearly to indicate syphilis.
The local skull tenderness and impairment of vision might well suggest
intracranial pressure. Uniting these suggestions, we might automatically
arrive at a diagnosis of cerebral gumma. We have learned to be rather
cautious of making a diagnosis of gumma of the brain through its mere
rarity.
Decompression was suggested and executed. A deep growth resembling a
GUMMA, in the view of the surgeon, was discovered. No attempt could be
made to remove it. The patient died without recovering consciousness.
1. What is the significance of the negative fluid W. R. in this case
of cerebral gumma? The W. R. producing substances not infrequently
fail to appear in the spinal fluid from a gumma of the brain. The
serum W. R. was positive in this case, but even the serum W. R.
may be negative in cases of gumma, both of the brain and of the
body at large. It must be remembered that the serum W. R. may be
negative in paretic neurosyphilis (general paresis); the serum W.
R. is even more apt to be negative in cases of gumma.
[Illustration:
Gummatous meningitis. Compression of hemisphere. Tissue destruction of
underlying cortex.
]
2. Is operative procedure to be advised in cerebral gumma? There are
cases in which the acute and threatening symptoms of heightened
intracranial pressure require operative treatment simply because
the therapeutist cannot wait for the effect of antisyphilitic
treatment. Moreover, antisyphilitic treatment of cerebral gumma is
not always as successful as that of most syphilitic lesions.
3. Could the intracranial pressure be caused by other syphilitic
lesions than gumma? A heavy meningitis may cause symptoms such as
produced by an intracranial tumor. In such a case one will usually
find evidences of inflammation in the spinal fluid. Cysts caused
by syphilitic lesions may also produce identical symptoms.
4. What is the significance of cranial tenderness? Where
sensitiveness to cranial percussion is not due to a scalp lesion
it is very suggestive of a tumor underlying this point. A
gummatous lesion of the cranium itself, may occur without causing
pain or increased sensitiveness.
=CRANIAL NEUROSYPHILIS (focal syphilitic extraocular palsy) without
mental symptoms.=
=Case 28.= A chef, Paolo Marini, 28 years of age, reported that on
awaking one morning, everything appeared double to him and that his
right eyelid had begun to drop. In the following month Marini had begun
to feel weak and to have difficulty in swallowing, as well as at times
difficulty in breathing. The diplopia was found to develop when Marini
looked to the right. Mentally, the patient was in all respects normal,
and no other physical signs were found except the diplopia and ptosis
above mentioned. The W. serum test was positive, but the tests of the
spinal fluid were negative.
=Diagnosis=: “CEREBRAL SYPHILIS.”
1. What is the anatomical cause of this condition? It is thought to
be due in a number of cases to a small diffuse gummatous lesion at
the _basis cerebri_. In the case of Marini this lesion appears to
have been a little more extensive and to have interfered with the
tenth and twelfth nerves also.
2. Why is the spinal fluid negative in such a case as that of
Marini? Head and Fearnsides believe that intracerebral lues is
characterized by a negative spinal fluid, under which circumstance
one has always to consider the possibility of brain tumor or
migraine in addition to the suspicion of syphilis.
3. What other causes besides syphilis should one consider for the
sudden diplopia? Brain tumor, multiple sclerosis, cerebral
arteriosclerosis, tuberculous meningitis, trauma and migrainous
ophthalmoplegia, are not infrequently at the bottom of this
condition. Cases also occur in which the etiology remains obscure,
even at autopsy.
Under antisyphilitic treatment, Marini slowly improved.
=The SIX TESTS in TABETIC NEUROSYPHILIS (“tabes dorsalis”) may run
milder than in paretic neurosyphilis (“general paresis”) and
characteristically run somewhat like those of diffuse
(meningovascular) neurosyphilis; in particular, the fluid Wassermann
Reaction and the gold sol reaction are apt to run milder. The
clinical course of tabes dorsalis is protracted and the prognosis as
to life is good.=
=Case 29.= Mario Sanzi, 55 years of age, had been having what he called
rheumatism since his 43d year. This rheumatism affected only the hips
and legs, had at times been very severe, and for two years past had been
almost constant. Before that time, pains had come at intervals, lasted a
variable period, and suddenly disappeared. They were of knife-thrust
character, and could probably be called “lancinating.” In a given
attack, these pains would come at intervals of seconds or more. There
was also a certain unsteadiness in locomotion and inability to control
the vesical sphincter.
=Physically=, the patient was entirely normal so far as could be made
out except =neurologically=. Argyll-Robertson pupils, absence of
knee-jerks, and ankle-jerks, Romberg sign, and characteristic gait, left
no cause for doubting the diagnosis of TABES DORSALIS. The blood and
spinal fluid both proved positive to the W. R., though the W. R. in the
fluid gave a negative reaction with 0.1 cm. and became positive with 0.3
cm. or more. The globulin was somewhat increased though less markedly so
than in paresis. The gold sol reaction was “syphilitic” but weak. It is
to be noted that the disease had run a 12–years’ course before a doctor
had been consulted. The primary infection occurred at 32 years, namely,
11 years before the symptoms began. At the time of his primary
infection, Sanzi had received several years of treatment, chiefly in the
form of mercury by mouth.
1. What is the value of mercurial treatment of syphilis in the
prevention of tabetic or other forms of neurosyphilis? “Fournier
strove for many years to convince the medical profession that a
syphilitic patient should be treated for at least two years after
his infection, whether the syphilis seemed latent or patent. The
method of treating only the symptoms he characterized as the
opportunist method; treatment in the absence of definite symptoms
the preventive method, as preventing the later manifestations.
That prolonged treatment does prevent is shown by Fournier’s
figures analyzing 2396 cases presenting tertiary signs. These he
divides into three groups: Group I, comprising 1878 cases, or 78
per cent of the whole number, having no treatment or inadequate
treatment—that is mercury for less than one year; Group 2,
comprising 455 cases, or 19 per cent, having moderate
treatment—that is, mercury for one to three years; and Group 3,
comprising the remaining 19 cases which represent only 3 per cent
of the whole number, having treatment for more than three
years.”[7]
In the light of what we now know concerning latent neurosyphilis,
it would seem well for patients to be followed from time to time
with the W. R. on blood and spinal fluid after the supposed
completion of the treatment of primary and secondary syphilis. The
examination of the spinal fluid is not superfluous, as our
experience with the so-called _paresis sine paresi_ abundantly
shows. At the present day it is not good practice to assure a
patient that he is cured after two years of ordinary mercurial
treatment without resort to frequent spinal fluid tests, even
though the serum W. R. be negative.
=TABETIC NEUROSYPHILIS (“tabes dorsalis”) is often quite ATYPICAL
clinically and may even show no single symptom warranting the old
clinical name “locomotor ataxia.”=
=Case 30.= Stephen Green is a case of TABES DORSALIS with active
knee-jerks and without locomotor or muscle-sense disorder. When observed
at the age of 45, it appeared that there were but two complaints: lack
of control of the vesical sphincter and shooting pains in the legs. It
appeared that the urinary disorder dated back ten years, when there had
been difficulty in passing the urine. Sounds had been passed at the
time; occasionally there had been incontinence during after years,
ascribed by Mr. Green to the passing of the sound. However, the
physician at that time stated that the incontinence was a symptom of
tabes dorsalis. The incontinence had recently become worse, especially
marked at night, though also occurring in the day; much worse during
excitement, and very much worse after taking alcoholic drinks. Besides
incontinence, there is also difficulty at times in passing the urine, as
well as dysuria.
As for the pains in the legs, they had been first noticed some three or
four years ago and considered to be mild rheumatic effects. Now,
however, they have grown progressively worse and have been the effective
cause of giving up business. The pains are sharp, darting, pinching, and
burning, and last, say, about a second with an interval of about the
same length. The attack will continue sometimes for many hours.
There is a strabismus of the left eye, ascribed by the patient to an
accident with an umbrella (there had been operation without relief). The
pupils showed the Argyll-Robertson effect and were markedly irregular.
Despite the divergent strabismus with diplopia, the eye movements were
well performed although not in parallel axes. Ankle-jerks could not be
obtained even on reinforcement, but the knee-jerks were lively, and the
other deep and skin reflexes proved normal. The blood and spinal fluid
tests were characteristic of tabes dorsalis.
It appears that the syphilis was acquired by this patient 15 years
before; that is, 5 years before neurological symptoms began. Three
courses of treatment had been taken at a well-known watering-place, and
mercury pills had been taken for two years by mouth. The patient is
married; has no children; there have been no pregnancies.
1. What causes may be assigned for the absence of children in the
family of a tabetic? There may be lesions of the genital apparatus
(orchitis, or more specialized toxic lesions). But impotence such
as characterized the present case must also be taken into account.