which improvement in paralysis is to occur if there is to be any
improvement. There was certainly not the slightest improvement in the
paralysis of this case before eight or nine months of treatment had
elapsed, and it took ten months to secure the marked improvement
mentioned.
1. What is the significance of the prodromal symptoms? The headache
and dizziness should have been viewed with great gravity. They are
characteristic in MENINGOVASCULAR NEUROSYPHILIS.
Moreover in this case there had also been difficulties with speech
and other transient symptoms which should have called attention
far earlier to the possibility of neurosyphilis.
2. What is the significance of the high cell count: 176 per cubic
millimeter? Such high cell counts are frequent enough in diffuse
neurosyphilis, but low cell counts are frequent also. But although
the high cell count taken alone is of lesser significance, the
fact that the high cell count in this case is associated with a
“syphilitic” gold sol reaction is of far greater significance for
diagnosis. These associated findings are characteristic of
meningovascular neurosyphilis.
3. What kind of recovery may be expected in successful examples of
treatment in meningovascular cases? Recovery with defect. It will
be noted that ten months elapsed before any marked improvement
occurred on the paralyzed side. We could not expect a complete
recovery from this paralysis.
4. Was inadequacy of treatment following the chancre responsible for
the early cerebrospinal involvement? In this connection one must
remember that such neural involvements occur occasionally even
during active treatment (neurorecidives). The discontinuance of
treatment after a short period, in this case less than a year, is
always a risk to say the least. And this is true even though the
W. R. becomes negative, for trouble of a neurosyphilitic nature
may occur later; this when both blood and spinal fluid have
previously been found negative. The old rule of following and
treating a syphilitic for several years despite the disappearance
of symptoms is still a good rule.
=The results of systematic, intensive, intravenous salvarsan therapy
in atypical neurosyphilis (cases not certainly paretic, tabetic or
the common types of meningovascular neurosyphilis) may be in our
experience as good as the results of treatment in common
meningovascular cases: example.=
=Case 105.= Henri Lepère, a machinist, 48 years of age, came voluntarily
to the Psychopathic Hospital for a gradually failing memory and
inability to work. He had had indigestion for four years (epigastric
distress, nausea, no vomiting). He was still suffering from epigastric
distress and from headaches. At times he had had difficulty in walking.
=Physically=, Lepère looked older than he was; he was very poorly
developed and nourished, and seemed very weak. There was a slight
visceroptosis.
=Neurologically=, there was considerable speech defect, particularly
well marked in test phrases. The pupils were contracted and gave the
Argyll-Robertson reaction. Neurologically there were no other signs.
=Mentally=, there was a depression with worry; but it was a question
whether these phenomena were not entirely natural. The special complaint
was of failing memory.
The Argyll-Robertson pupil also _prima facie_ signifies neurosyphilis.
Lepère, in fact, admitted syphilitic infection at 23. The gastric
symptoms at once suggested tabes. The knee-jerks and ankle-jerks were,
to be sure, preserved; however, this is not very unusual in tabes. The
amnesia and aphasia naturally suggested paresis. Without resort to
laboratory findings, accordingly, the diagnosis of taboparetic
neurosyphilis (“taboparesis”) was suggested.
=EFFECT OF EARLY TREATMENT ON THE DEVELOPMENT OF NEUROSYPHILIS=
TOTAL CASES 4134
DEVELOPED GENERAL PARESIS 198 = 4.8%
DEVELOPED TABES DORSALIS 113 = 2.7%
DEVELOPED CEREBROSPINAL SYPHILIS 132 = 3.2%
———————————
443 = 10.5%
=EFFECT OF TREATMENT=
Repeated
None 1 course energetic
NUMBER OF CASES 100 134 924
DEVELOPED G.P. 25 = 25% 31 = 23.1% 30 = 3.2%
DEVELOPED TABES 11 = 11% 16 = 11.9% 25 = 2.7%
DEVELOPED C.S.S. 3 = 3% 21 = 15.6% 71 = 7.6%
Poorly Better
treated treated
1880–84 1895–99
NUMBER OF CASES 617 1139
DEVELOPED G.P. 60 = 9.7% 37 = 3.2%
DEVELOPED TABES 22 = 3.5% 16 = 1.4%
DEVELOPED C.S.S. 15 = 2.4% 28 = 2.4%
MATTAUSCHEK AND PILCZ
CHART 24
The serum W. R. proved positive, but the spinal fluid W. R. very
slightly so (yielding only moderate reaction with 1 cc., 0.7 and 0.5
cc., and a negative reaction with 0.3 and 0.1 cc.). Globulin was
moderate, and albumin was found in only moderate excess. There were 21
cells per cmm. in the spinal fluid. The gold sol reaction was that which
we regard as typical of syphilis or tabes. If we were to rely upon the
weakness of the fluid W. R. and the nature of the gold sol reaction, we
should be inclined to favor the diagnosis of DIFFUSE NEUROSYPHILIS
(“cerebrospinal syphilis”) rather than resort to the diagnosis of
paretic neurosyphilis.
Salvarsan treatment was attended by the rapid disappearance of headaches
and gastric symptoms and by a rapid gain in weight and feeling of
well-being. Salvarsan was continued twice a week for two months,
whereupon Lepère returned to work. He has been successfully at work now
for seven months without return of symptoms. Four months after beginning
of treatment, the spinal fluid was examined and found entirely negative.
Nevertheless, the serum W. R. has remained positive despite eight months
of salvarsan treatment.
1. What is the meaning of the titrations in the spinal fluid
Wassermann reaction? When Plaut originally applied the Wassermann
reaction to spinal fluids, he used 0.2 of a cc. of spinal fluid.
With this amount of fluid he found that cases of general paresis
gave a positive reaction in about 100% of the cases while this
positive reaction was only given by 40 to 60% of the cases of
cerebrospinal syphilis and tabes dorsalis, hence he promulgated a
differential point that a negative reaction in spinal fluid
indicated that the case was not general paresis. Hauptmann later
showed that if 1 cc. of spinal fluid were used, a positive
reaction would occur in practically 100% of the cases of general
paresis, cerebrospinal syphilis and tabes. Therefore, at present,
we use the different titers of spinal fluid from which we draw the
following conclusions: If the reaction in the untreated case is
negative with 0.1 and 0.3 of a cc. and positive with the 0.5, 0.7
and 1 cc. dilutions as in the case of Lepère, we are probably
dealing with non-paretic neurosyphilis. With this method of
titration we are also better able to watch the progress of
treatment as the dilutions of 0.1 and 0.3 cc. become negative
first.
2. How soon can one expect improvement after commencement of
salvarsan therapy in cases of diffuse neurosyphilis? The time
relation of results in treatment varies with each individual case.
In the case of Lepère gastric symptoms that had been present for a
number of months disappeared as if by magic after the first
injection of salvarsan. As a rule, it is true that the more acute
the symptoms the quicker their disappearance but this does not
hold for all cases, as in this particular instance the
long-standing symptoms disappeared very rapidly. The symptoms
often disappear very much more rapidly than the laboratory, tests
change.
3. How can the mental symptoms (depression and failing memory) of
which patient complained be explained? In the first place, as has
been stated, it is doubtful if these are more than subjective and
the result of the patient’s feeling of discomfort and pain.
However, it is also possible that there may be intracranial
involvement of the meninges or of the brain itself. And, if such
were the case, the improvement might be the result of the
treatment.
=The Argyll-Robertson pupil should not be used as a basis for a
necessarily bad prognosis if treatment can be given.=
=Case 106.= Frederick Stone was a business man of large interests. He
had been in the hands of physicians for several years for a variety of
disorders such as renal, respiratory, cardiovascular, and so on. No
suspicion of syphilis had apparently been uttered by the physicians
despite the fact that Mr. Stone readily stated that he had had a chancre
thirty years before, and that he had received several years’ treatment
of mercury and potassium iodid by mouth.
It appeared that a few years ago he had begun to have trouble with his
nose, which was cauterized and operatively interfered with without
satisfactory results. This nasal condition had later been diagnosticated
as gummatous, and had improved considerably under a mild antisyphilitic
treatment. However, this nasal condition had been considered and treated
quite separately from the remainder of Mr. Stone’s troubles.
What brought him to attention was a sudden diplopia with ptosis. There
was a paralysis of the external rectus of the left eye, as well as a
drooping of the lid on this side. The left eye was much inflamed. The
diplopia greatly bothered the patient, and there was also considerable
pain in the left frontal region, confined chiefly to the distribution of
the first division of the trigeminal nerve. According to the patient
this headache was periodic. There was considerable tenderness to
pinprick over the area and a diminution of sensory discrimination of
fine touch. Both the pupils failed to react to light.
The remainder of the neurological symptomatic examination was
surprisingly clear of disorder, nor was there anything in the history
suggestive of tabes. There was ozena as well as evidence of the
operative work upon nares and throat. Possibly the arteries were
slightly hardened; blood pressure was 165 systolic. There was a large
trace of albumin, and there were numerous hyalin casts in the urine.
=PARETIC NEUROSYPHILIS=
=(GENERAL PARESIS)=
Cases systematically treated 50
CLINICAL REMISSIONS 34 68%
C.S.F. ALTERED TO NEGATIVE 4 8%
C.S.F. ALTERED TO WEAKER 16 32%
C.S.F. UNALTERED 14 28%
CLINICALLY UNIMPROVED 16 32%
C.S.F. WEAKER 7 14%
C.S.F. UNALTERED 9 18%
MASSACHUSETTS COMMISSION ON MENTAL DISEASES
NOVEMBER, 1916
CHART 25
=Mentally=, there was a degree of depression and worry hardly out of
keeping with the general situation. Despite the preservation of memory,
Mr. Stone failed to do rather simple arithmetical calculations; this was
the more remarkable as in his business he had to handle figures a great
deal and had been doing so until recently. There was a slight tremor in
his writing, as well as a certain difficulty in enunciating test
phrases. Insomnia, irritability, and a feeling of nervousness and of
being tired out, completed the picture.
A suggestion for diagnosis would be classically offered by the
Argyll-Robertson pupils. Should not a patient with the Argyll-Robertson
pupils have either tabes or paresis? However, in favor of tabes, besides
the pupil, are to be counted merely the troubles with the eyes. In the
direction of paresis we have to consider speech defect, to say nothing
of less definite symptoms such as insomnia and increased irritability.
We are inclined to think, however, that the disease in this case is
meningovascular. This diagnosis is suggested by the cranial nerve
palsies and by the headache. Headache is much more rarely a phenomenon
in the paretic type of neurosyphilis than in the meningovascular type.
In point of fact, the spinal fluid phenomena bore out the diagnosis of
MENINGOVASCULAR NEUROSYPHILIS inasmuch as the globulin, albumin,
cellular content, gold sol, and W. R.’s were all weakly positive.
1. How far can we regard the cardiorenal defects as syphilitic?
Perhaps we may do so on the general principle of parsimony in
scientific interpretation.
The diagnostic lumbar puncture led to an extremely severe exacerbation
of the pains on the left side of the head. In fact, these pains could
not be held in check by the exhibition of pyramidon. Mr. Stone regarded
the pain as due to the lumbar puncture. However, there was no
improvement in the pain in the prone position,—a feature characteristic
of lumbar puncture pains. Upon administration of salvarsan, this local
pain rapidly disappeared. In fact, there was a startling improvement;
the ocular palsies disappeared in a few weeks, although these palsies
had been present for several months before the administration of
salvarsan. The blood pressure was reduced; the urine became negative.
Perhaps the most startling feature of all (although of this we are not
sure) was that the patient states he was accepted by a life insurance
company although he had been twice refused previously.
=Note= in this case the 30–year interval between infection and
generalized neurosyphilitic involvement. Note also the amenability of
the process despite this duration. We are perhaps entitled also to note
that a neurological examination careful enough to detect an
Argyll-Robertson pupil should have been made by a number of examiners
long before the particular crisis which we have sketched. It is also
permissible to note that the rhinological work should not have been
carried out independently of all other medical work.
2. What are the untoward results of lumbar puncture? It is true that
there is always a possibility of setting up a septic meningitis by
lumbar puncture, but this is a very remote possibility and with
any reasonable care it is not to be considered. Lumbar puncture
also has a considerable danger in cases of increased intracranial
pressure. In cases of brain tumor where the tumor is located in
the posterior fossa, sudden death may occur from withdrawal of
spinal fluid. This is supposed to be due to the medulla being
pressed down into the foramen magnum and causing paralysis of
respiration. Therefore lumbar puncture should never be performed
except with the greatest caution in a case in which brain tumor is
suspected.
However, aside from these remote serious consequences which play
very little rôle in the ordinary procedure of lumbar puncture,
certain unpleasant symptoms do frequently arise. These symptoms
are chiefly headache and nausea, but, however, may go as far as
vomiting. These symptoms occur almost entirely in the cases in
which there is no abnormal condition producing increased spinal
fluid pressure. Such unpleasant symptoms may last as long as four
or five days; as a rule, however, last only for a period of a day
or two.
3. What is the treatment of discomfort following lumbar puncture? It
is a rule well worth observing that the patient after lumbar
puncture should remain flat on his back without a pillow for 24
hours in order to avoid any unpleasant symptoms. If any symptoms
do occur, it will be almost certainly when the patient arises, and
in nearly every instance they will be overcome if the patient
again assumes the prone position. Raising the foot of the bed so
as to lower the head also helps. Veronal or bromides may be given
but as a rule are not very satisfactory.
4. How permanent is the improvement obtained in the case of Mr.
Stone likely to be? As a matter of fact, the patient discontinued
treatment as soon as he felt well again, but after two months the
pain returned to be again quickly dispelled by salvarsan. This
improvement must be considered as only temporary. Under continued
treatment there may be no further relapse. There is, however,
evidence that much damage has been done to the body by the
spirochetes, much of which is irreparable. It is even possible
that further disintegration might occur even while undergoing
treatment. Still treatment offers much in such a case and is to be
highly recommended.
=In DIFFUSE NEUROSYPHILIS, rendering the spinal fluid negative by
treatment may mean neither cure nor disappearance of symptoms.=
=Case 107.= Greta Meyer, a widow, 51 years of age, came voluntarily to
the hospital, seeking medical aid for a marked depression. She was also
suffering from a right hemiplegia. It appeared, according to Mrs. Meyer,
that she was married at 16, and lived with her husband until 29,
whereupon she left him on account of his alcoholism, his abuse of her,
and the discovery through his physician that he was suffering from
venereal disease. She had had two healthy children and there never had
been miscarriages or stillbirths. Six years after the separation, namely
at 35 years of age, and 16 years before resort to the Psychopathic
Hospital, Mrs. Meyer developed certain red areas on her hand, and
learned at a hospital that these were due to syphilis. She kept up
treatment for these lesions for a year, until she seemed perfectly well.
She had, in fact, remained perfectly well for some 14 years, until at
49, a small tumor had appeared on the right side of the forehead, near
the hair line. This tumor was firm and not sore. Medical treatment
reduced it, leaving, however, a depression in the bone. One day, about a
month after the appearance of the tumor, the patient lay down for a nap,
and upon awaking found she could only with difficulty move her right arm
and leg. Her face was not affected; she was not in pain; and there was
no disorder of speech. In a few days she got much better and she had
been improving for some time past through the administration of further
medicine.
However, since the onset of the hemiplegia Mrs. Meyer had been very
despondent. There had been ups and downs but she had rarely felt well.
The depression was a mild one and in point of fact may perhaps be
regarded as non-psychopathic, since at her age with her disability,
there might well be a degree of sadness and unhappiness concerning the
future. =Mentally=, there was no other disorder of note, and in
particular no disorder of memory.
=METHODS OF TREATMENT=
I. BY MOUTH.
1. MERCURY
2. IODIDES
3. ARSENIC
II. INTRAMUSCULAR INJECTIONS
1. MERCURY
2. SALVARSAN, NEOSALVARSAN, OTHER ARSENIC PREPARATIONS
3. SODIUM NUCLEINATE
4. ANTIMONY
III. INTRAVENOUS
1. MERCURY
2. MERCURIALIZED SERUM
3. SALVARSAN, NEOSALVARSAN, ARSENIC
4. IODIDES
IV. SPINAL INTRADURAL
1. SALVARSANIZED SERUM (IN VIVO—SWIFT-ELLIS)
2. SALVARSANIZED SERUM (IN VITRO—MARINESCO-OGILVIE)
3. MERCURIALIZED SERUM (BYRNES)
V. CEREBRAL SUBDURAL AND INTRAVENTRICULAR
1. SALVARSANIZED SERUM (IN VIVO)
2. SALVARSANIZED SERUM (IN VITRO)
3. MERCURIALIZED SERUM
CHART 26
=Physically=, the patient showed a right-sided hemiplegia with excessive
right knee-jerk, but without Babinski or other abnormal reflex
phenomena. The extraocular movements were somewhat restricted in range
but there was neither strabismus nor nystagmus.
The question arose whether the hemiplegia was of hemorrhagic or
thrombotic origin. After all, at 51 years, hemiplegia is rather unlikely
to be of a non-syphilitic arteriosclerotic origin; moreover, we had a
clear history of syphilis. The serum W. R. proved positive as well as
the spinal fluid W. R. The finding of 77 cells per cmm., excess albumin,
and positive globulin test, taken in connection with the entire picture
seems to warrant a diagnosis of CEREBROSPINAL SYPHILIS. If we proceed on
statistical grounds, it might be regarded as more probable that the
hemiplegia is THROMBOTIC in origin rather than hemorrhagic. It appears
that syphilitic cerebral thrombosis rather characteristically occurs
without preliminary symptoms, despite the fact that many cases do show
headache, dizziness, and restlessness as prodromal symptoms.
1. What is the treatment indicated in the case of Mrs. Meyer?
It would appear that little or nothing can be done for the
hemiplegia unless the claims of Franz with respect to
reëstablishment of a degree of function in certain hemiplegics are
substantiated. However, the indication of meningitic process as
shown by the spinal fluid, suggests that the case is not a purely
vascular one but may be regarded as meningovascular. (Possibly,
also, we should regard the left frontal depression and scar as
indicative of a non-parenchymatous and non-vascular process.)
Accordingly, antisyphilitic treatment should be theoretically of
some value.
In point of fact, the patient was given injections of mercury
salicylate, mercury by mouth, and potassium iodid. Her
psychopathic depression under this treatment, supported by proper
hygiene and rest, diminished. However, six months later, the
patient slipped on a wet floor and fell. Though the impact seemed
hardly sufficient to cause a fracture, the pelvis was somewhat
severely fractured. Very probably there was a syphilitic
rarefaction of the bone. Six months later the patient’s depression
was still in evidence, though somewhat less than upon admission.
The blood serum remained positive but the spinal fluid had become
entirely negative, both in respect to the W. R. and in respect to
the other findings.
2. How may one explain the continuance of the depression after the
spinal fluid had become entirely negative under treatment? It may
be that while the active process had been stopped, as seems
probable from the negative spinal fluid, that a permanent
destruction of brain tissue may account for the depression. We
recognize this readily in instances of vascular disturbance where
(as also in this case) the active process being stopped, a
residual defect remains.
3. Should treatment have been discontinued on reduction of the
gumma? It cannot be too often emphasized that the disappearance of
symptoms in cases of syphilis can not be considered as evidence of
cure. The neurologist and psychiatrist see only too often cases of
neurosyphilis occurring in patients who have been declared cured
at some time previous because the symptoms then present had
cleared up and remain in abeyance for years.
=Contrary to various warnings, arteriosclerosis by no means
absolutely contraindicates intensive salvarsan therapy.=
=Case 108.= Victor Friedberg, 42 years of age, gave the following
history. He acquired syphilis at 22 years. He had “adequate” medical
treatment for two years with inunctions of mercury and mercury by mouth
and potassium iodid. The only secondary symptoms were skin lesions of
the legs; these disappeared upon treatment. Married, Friedberg has one
child, apparently normal. There had been no miscarriages or stillbirths.
At about 34 years, there began to be shooting pains in the legs,
occurring at first about once in three months, but later much more
frequently. These pains were severe, lightning in character, lasting
several days at a time, at which period his head would feel heavy; but
there were no disturbances, crises, or difficulty in locomotion.
At 36 years of age, Friedberg waked up with pain one night, and found he
was unable to move his left leg or hand, and he felt his mouth drawn to
the left. Upon trying to get out of bed, he fell to the floor. In five
hours, however, he was entirely recovered, able to get up and walk
about, and to use his left arm quite normally. He went to sleep, but
upon waking up after an hour, discovered that his left side was again
paralyzed. After two weeks in a hospital, he was able to walk with a
crutch. The arm remained helpless for about a year. Both arm and leg
improved slowly for two years, after which time his condition had
remained stationary. For four years past, there had been no more pain,
but at 42—about two years before admission—the pains returned in his
legs, back, and side. At that time he received four injections of
salvarsan, mercury tablets, and potassium iodid. Three weeks before
admission to the hospital, Friedberg again began having headaches, very
much worse than formerly. At first these headaches were frontal, then
occipital, and there was a feeling as if something were growling inside
of the head. There was a feeling of pressure in front on the head and at
the base of the nose.
=Physically=, Friedberg appeared somewhat older than his assigned age.
There was a degree of general peripheral arteriosclerosis, but in
general the physical examination was negative. _Neurologically_, there
was a left hemiplegia with appropriate increase of the reflexes on that
side, spasticity, Babinski reflex, and an Oppenheim; the pupils reacted
properly; there was no Romberg reaction.
_Mentally_, Friedberg was entirely negative.
The W. R. of the blood serum was doubtful, as was that of the spinal
fluid. There were but two cells per cmm. and there was neither globulin
nor excess albumin in the spinal fluid.
The =differential diagnosis= might lie between cerebral hemorrhage and
syphilitic thrombosis. Thrombosis is much more common as a result of
syphilis than is hemorrhage. The occurrence of the thrombosis during
sleep without premonitory symptoms is also characteristic in syphilis.
Possibly there was a low-grade spinal meningitis at the bottom of the
lancinating pains. Whether the headache is an arteriosclerotic effect or
due to a meningitis not shown in the cerebrospinal fluid is doubtful.
However, the absence of inflammatory products in the cerebrospinal fluid
rather indicates that the headache is of arteriosclerotic origin.
Autopsies, however, warn us that we may have a localized meningitis in
various parts of the cranial cavity without the determination of any
inflammatory products in the spinal fluid.
1. How shall we explain the doubtful (slightly positive) W. R. in
the spinal fluid if the case is one of VASCULAR BRAIN SYPHILIS?
The finding is not unusual in these cases. The W. R. producing
body is recognized to be of a separate nature from the globulin
and albumin bodies, and is probably also separate from the gold
sol reaction producing bodies.
Treatment: The theory of treatment is that any spirochetes that may be
still active in the body should be destroyed. Accordingly, although
salvarsan can certainly have no effect in reproducing nerve tissue, it
nevertheless seems indicated. It is frequently stated, however, that
salvarsan is dangerous in cases of this group. We have not found this
statement correct. In this case, there was a symptomatic improvement, as
far as pain and discomfort went, under salvarsan and iodids.
2. What precautions should be taken in intensive salvarsan treatment
of syphilitic arteriosclerosis? Treatment should be begun with
very small doses of salvarsan, that is, about 0.1 of a gram and
then the amount slowly increased. The injection should be given
slowly so as not to put too great a load upon the cardiovascular
system.
3. What rôle does the mental attitude of the patient play in a case
like that of Friedberg? It was quite evident that Friedberg was
neurotic and that he had a syphilophobia. Consequently some of the
symptomatic improvement may have been more results of assurances
offered by the physician and knowledge that he was being treated,
than results of salvarsan. In some cases mental anguish suffered
by the patient is of more importance than the actual symptoms of
the disease and this point must be always borne in mind in
handling syphilitic patients.
=Symptoms of intracranial pressure cured by antisyphilitic
treatment.=
=Case 109.= Mrs. Annie Rivers, a housewife 36 years of age, sought
advice and treatment for severe convulsions which she had had during a
period of several weeks. She left the hospital before being properly
examined, and had several more convulsions, after which she was brought
back in a state of marked confusion. The confusion shortly disappeared
almost completely, and a good history was obtained.
It appears that the patient led a normal life and had had six children,
the last of whom was born about four months before her coming to the
hospital. The first symptoms appeared about a month after the birth of
the child, when, one afternoon, Mrs. Rivers suddenly fell unconscious
while ironing. She remained unconscious for nearly three hours. During
this attack there were no convulsive movements or tongue-biting; and
after the spell, she felt neither lame nor sore, but merely tired. This
was Mrs. Rivers’ statement; but her daughter stated that the patient
really did have convulsive movements. A week later came a second
convulsion, followed by daze and stupor. This second attack lasted two
hours.
About a week before entrance, the patient had remained in bed on account
of dull grinding pain in the left side of the head, below the ear, and
upon this day the patient vomited twice. In addition to the dull
grinding pain, there were pains referred to the ear itself and to the
left side of the head, especially over the left eye; there were no pains
on the right side of the head. The next day the patient was better, but
the day thereafter again remained in bed. The only other symptoms were
cold feelings at times and bright spots in the field of vision.
No =mental symptoms= were observed in Mrs. Rivers except a bit of
depression after her hasty retreat from the hospital the first time.
Upon her second admission, however, after a week or ten days’ residence,
apathy developed together with considerable amnesia for the same facts
she had quite readily remembered a few days previously. Along with the
apathy and amnesia developed considerable headache; and there were
attacks of vomiting.
=UNTOWARD SYMPTOMS OF THERAPEUTIC AGENTS=
=A. SALVARSAN=
CYANOSIS MALAISE
RAPID PULSE
PERSPIRATION
RESPIRATORY DIFFICULTIES
FEVER
NAUSEA, VOMITING, DIARRHOEA
DERMATOSES
EDEMA
KIDNEY IRRITATION
LIVER IRRITATION
INTENSIFICATION OF SYMPTOMS
COLLAPSE
=B. MERCURY=
SALIVATION
FETID BREATH
EXCESS FLOW OF SALIVA
TENDERNESS OF TEETH—LOOSENING AND FALLING OUT
SPONGY GUMS—EROSION
METALLIC TASTE
NECROSIS OF BONES OF JAW
SORENESS OF PARETIC AND MAXILLARY GLANDS
SWELLING AND EROSION OF TONGUE AND MUCOUS MEMBRANES
GASTRO-INTESTINAL SYMPTOMS
ANEMIA
PAIN IN JOINTS
NEPHRITIS
=C. IODINE=
SKIN LESIONS
METALLIC TASTE
SALIVATION
CORYZA
URTICARIA (EVEN TO GRADE OF ANGIONEUROTIC EDEMA)
PAINS
CONSTIPATION
INVOLVEMENT OF JOINTS
FEVER
SOFTENING AND BLEEDING OF GUMS
EROSION OF MUCOUS MEMBRANES
GASTRO-INTESTINAL SYMPTOMS
ANOREXIA
WEAKNESS
CHART 27
On the =physical= side, it is interesting to note that the
ophthalmoscopic examination upon Mrs. Rivers’ first admission to the
hospital was entirely negative, whereas a week later, pronounced
difficulty with vision appeared so that in a few days she was able to
make out only very large type. The fundi now showed hazy and indistinct
disc outlines, with small yellowish areas of fatty degeneration above
the disc, reduction of arterial calibre, and dilated and somewhat
tortuous veins (no projection of papillæ), so that the ophthalmological
diagnosis was chronic neuritis.
The physical examination otherwise was mostly negative. The skin
presented irregular areas covered with silvery scales over the arms and
chest, back, abdomen, and legs (the patient had had psoriasis several
years before). Both pupils reacted to light and distance, though the
right was slightly larger than the left and somewhat irregular. There
was a slight tremor of the tongue and extended fingers. The reflexes
were active, especially the knee-jerks; no abdominal reflexes could be
obtained. The serum W. R. was positive, but the spinal fluid W. R. was
negative. The spinal fluid showed but 3 cells per cmm., but there was a
positive globulin test and an excess of albumin.
=Diagnosis=: After the symptoms had fully developed, it became clear
from the optic neuritis, headaches, and vomiting that a condition of
intracranial pressure existed. In view of the positive serum W. R., it
is natural to conceive that the agent producing the intracranial
pressure was a gumma.
It is, of course, possible that a marked degree of meningitis might be
so localized as to produce the same symptoms. The diagnostician would
crave a pleocytosis of the spinal fluid if a diagnosis of meningitis is
to be made; and there was no such pleocytosis. On the whole, we do not
feel that it is possible to make a diagnosis either of MENINGITIS or of
GUMMA.
=Treatment=: Treatment, however, caused a disappearance of all symptoms.
The treatment consisted of but one injection of 0.3 gram of salvarsan,
followed by a few injections of mercury; whereupon Mrs. Rivers became
much brighter, recovered her vision, lost her headaches, ceased to have
convulsions or vomiting spells.
1. Is salvarsan contraindicated in cases with involvement of the
optic or auditory nerves? Such a contraindication exists according
to prevailing opinion. In this particular case, a hemorrhagic
retinitis occurred after the injection of salvarsan, but this
retinitis disappeared along with the other symptoms. On the whole
we believe that in many cases of optic or auditory nerve
involvement salvarsan should be used. However, one should never
lose sight of the possibility of untoward results and should
advise such treatment only when other treatment seems inefficient.
=TABETIC NEUROSYPHILIS (“tabes dorsalis”) may show very marked
improvement as a result of intraspinous therapy.=
=Case 110.= Mr. McKenzie[18] was a retired merchant of 42 years whose
complaint was that he tired very easily, could not make his legs go
where he wished, was unsteady and felt a numbness in his legs. These
symptoms had been in progress for a few months only when the examination
was made. This disclosed Argyll-Robertson pupils, absent knee-jerks and
ankle-jerks, Romberg sign, unsteady gait, moderate ataxia and dysmetria.
The W. R. was negative in the blood serum but positive in the spinal
fluid with 0.2 cc., and there were 107 cells per cmm. With the symptoms
and signs it was therefore easy to make the diagnosis of TABETIC
NEUROSYPHILIS (“tabes dorsalis”).
The patient was given five intraspinous injections of mercuric chloride
in blood serum (mercurialized serum) according to the method of Byrnes.
The dose was 0.001 gm. of mercury. Two weeks after the first injection
the cell count was 58 cells per cmm., the Wassermann was positive only
with 0.4 cc. After the fourth injection there were but 18 cells and the
Wassermann reaction was negative even with 1½ cc. of spinal fluids. The
symptoms had improved to such a degree that the patient had no complaint
whatsoever and considered himself cured.
1. What are the unpleasant results of intraspinous therapy?
Frequently there is an exacerbation of symptoms and pain may be
quite severe after intraspinous injections. This, however, lasts
only a short period, that is, as a rule less than 24 hours. There
may be other symptoms of cord irritation as retention of urine or
lack of sphincter control. A rise of temperature is not unusual.
=Treatment may alter the W. R. to negative in blood and spinal fluid
in TABES DORSALIS.=
=Case 111.= Ivan Rokicki was a baker, 43 years of age, who came
complaining of exceedingly severe attacks of abdominal pain with
vomiting. He described these attacks as having occurred periodically for
a number of years, lasting sometimes as long as a week, during which
time Rokicki could not eat or get relief short of large doses of
morphine.
Upon his arrival, Rokicki was seen in one of his attacks; he was curled
up with excruciating pain, and the abdomen was rigid, though it was
impossible to produce additional pain by external pressure. There was
spasmodic vomiting, frequently followed by slight relief from the pain,
which however shortly recurred and caused the patient to cry out in his
suffering. The condition was controlled by opiates but lasted a full
week. The leucocytes remained normal and there was no rise of
temperature. The attack ceased spontaneously.
Save for the pain, Rokicki’s =mental examination= proved entirely
negative. =Physically=, Rokicki was fairly well developed and nourished.
His pupils were slightly irregular: the left markedly larger than the
right; both pupils failed to react to light, and the left pupil also
failed to react in accommodation. There were no other reflex disorders
evident to systematic examination, nor was there sensory disturbance or
speech defect. The heart seemed somewhat enlarged but there were no
murmurs; blood pressure: systolic 150; diastolic 110.
The correct symptomatic diagnosis in Rokicki’s case proved to be gastric
crises, and this diagnosis must perforce be the first to entertain in
view of the chronicity, the periodicity, the non-relation to diet, and
the spontaneous cessation of the seizures. The observation of
Argyll-Robertson pupils was naturally held to substantiate the diagnosis
of TABES DORSALIS.
The possibility of abdominal inflammation could be shortly dismissed on
account of the absence of tenderness (the rigidity in this case was not
accompanied by tenderness), fever, and other characteristic signs. There
was no diarrhoea, such as is found in lead colic, and there was no other
sign of plumbism. Jaundice was absent and there was no special radiation
of pain from the abdomen. One had to think of gastric ulcer and
hyperchlorhydria, and possibly malaria or gastroenteritis.
The pupillary reactions pointed to a syphilitic condition despite the
fact that the lack of reaction to accommodation (over and above the
Argyll-Robertson phenomenon) in the right pupil is not entirely typical.
Accordingly, although there was no areflexia, Romberg sign, or ataxia,
resort was had to the W. R. This however proved negative, in blood and
spinal fluid; nor was there any globulin or excess albumin; there were 5
cells to the cmm., in the spinal fluid.
We are left, accordingly, with characteristic gastric crises;
Argyll-Robertson pupils, slightly irregular; and a somewhat enlarged
heart.
Upon investigation, it appeared, however, that a year before the attack
above described, the patient had been examined and both blood and spinal
fluid found positive to the W. R. At that time, treatment, consisting of
intravenous injections of salvarsan and intraspinous injections of
salvarsanized serum (Swift-Ellis), had been instituted. Whereupon the
laboratory tests had become negative, as above stated, and there had
been no alleviation of the symptoms.
1. How can Rokicki’s normal deep leg reflexes be explained? The
abolition of the deep reflexes is of course due to lesions
properly localized. It is probable that this particular case of
tabes dorsalis is more truly “dorsal” than most cases; for most
cases exhibit lesions involving regions lower than the dorsal.
Both in these dorsal cases and in certain rare cases of cervical
tabes, the deep leg reflexes are preserved. (See cases Green (30)
and Halleck (31).)
2. What is the mechanism by which a characteristic gastric crisis is
produced? The mechanism is unknown. Some endeavors have been made
to meet gastric crises by surgery of the posterior roots, on the
assumption that the irritation causing the pain was located either
in the posterior ganglion or in the passage of the nerve through
the meninges. In only a few instances, however, has the result
been what was desired. In many instances the gastric crises and
pain continued uninterrupted and in addition came discomfort due
to the lack of sensation in the part supplied by the severed
nerve. At present this treatment is seldom carried out.
3. Should antisyphilitic treatment be continued in such a case? As
far as our present knowledge of syphilis goes one would hesitate
to suggest further antisyphilitic treatment, feeling that the
active process had been entirely stopped as suggested by the
absence of any positive findings either in the blood serum or in
the spinal fluid. We should perhaps conclude that there was no
more activity in this case and that the crises were due to the
changes that had already taken place in the nerve tissue and which
could no longer be changed.
=The literature is in doubt concerning (in fact is preponderantly
against) the success of treatment in PARETIC NEUROSYPHILIS (“general
paresis”). Our experience has yielded a number of apparently
successful results through systematic intensive intravenous
salvarsan therapy. Example.=
=Case 112.= Albert Forest had always been a successful salesman, but in
the middle of March, in his 46th year, he was arrested for grabbing a
purse from a woman in front of a theatre and running down the street
with it. In court, Forest acted strangely and he was sent to the
Psychopathic Hospital for observation. Upon investigation, it appeared
that his wife thought he had been showing mental changes for about a
year. For example, he would embrace his wife on a street car, or refuse
to pay her fare. He once attempted to hit his son on the head with a
red-hot poker. Now and then he would become sleepy and stupid. He looked
rather older than his age and had a coarse tremor of the hands.
Otherwise, no change could be detected in the physical examination,
either neurologically or otherwise. As for the manual tremor, Forest’s
wife gave a history of considerable alcoholic indulgence on his part.
For several days, nothing abnormal could be detected in the man; and in
particular, his memory for both remote and recent events was very good
and his knowledge of current events was good. Simple arithmetic was easy
to him.
One evening his temperature was found to be 104° F. and no cause could
be discerned for this. The next morning, Forest was discovered in a
stupor, with a complete right hemiplegia. The Babinski reflex, the
Oppenheim reflex, and ankle clonus had appeared on the right side, and
the right arm was spastic.
However, all symptoms of this paralysis had disappeared by four o’clock
in the afternoon, and the paralytic phenomena were replaced with
violence. The patient fought with the attendants and for some time
remained extremely difficult to manage, being confused and subject to
outbreaks of violence with destruction of furniture and other property
about the ward.
=Diagnosis.= At first we were naturally inclined to dismiss the case
with a diagnosis of alcoholism. The transient hemiplegia at once raised
a considerable question of brain syphilis or of brain tumor.
The W. R. of the serum was doubtful. The spinal fluid yielded, besides
marked excess of albumin and much globulin, also a “paretic” gold sol
reaction and 75 cells per cmm. The W. R. was positive.
=Treatment.= The patient was given injections of salvarsan, 0.6 gram,
twice a week, with potassium iodid. After a few weeks improvement
followed, and after several months all the laboratory tests became
negative, the patient was apparently perfectly normal mentally and was
discharged from the hospital, and has remained well for 18 months
without further treatment. The serum W. R. has continued to be negative.
1. What is the significance of the so-called “doubtful” W. R.? Where
there is not a complete uniformity the results of the strong and
weak antigens (see appendix on technique of Wassermann reaction)
the result is reported as doubtful. In the majority of instances
repetitions will give a strong positive reaction.
2. Is the case of Forest to be regarded as one of general paresis?
Sometimes such cases are termed in the literature _syphilitic
pseudoparesis_ (see case Burkhardt (58)). The differential
diagnosis of this group is entirely therapeutic. There are,
unhappily, no laboratory tests which will suffice in the present
stage of knowledge to differentiate a case of so-called
pseudoparesis from general paresis. We are inclined to term the
case one of GENERAL PARESIS, with recovery, or, at all events,
with remission.
=The literature is in doubt concerning (in fact is preponderantly
against) the success of treatment in PARETIC NEUROSYPHILIS (“general
paresis”). Our experience has yielded a number of apparently
successful results through systematic intensive intravenous
salvarsan therapy. Example.=
=Case 113.= We present the case of Gussie Silverman, a housewife, 35
years of age, among other reasons, for its social interest. The case is,
on the whole, sufficiently typical of GENERAL PARESIS. =Physically=, for
example, the pupils failed to react to light and accommodation and were
unequal, the right being larger than the left. The knee-jerks were
sluggish though equal. The ankle-jerks could not be obtained. The
abdominal reflexes were not obtained. Otherwise, there was no reflex
disorder.
From the =laboratory= point of view, the W. R. was positive in the blood
and in the spinal fluid. There were 80 cells per cmm. and there were an
appropriate globulin and albumin reactions. Mrs. Silverman was rather
poorly nourished and had a slight edema of the ankles.
=Mentally=, she was found on admission to be markedly depressed. It
appeared that during a recent pregnancy, terminated by the birth of a
7–months child, she had fainted several times a day, that since the
confinement she had been very nervous, that she had been asking her
husband not to send her away, that she had refused to leave the house,
that she had become excited even to the point of injuring herself,
especially at night, and that she would go so far as to scratch her
husband, shortly afterward being very sorry for her performances. Before
this last pregnancy there had been four others and the resulting
children were all apparently in good health. Except for the fainting
spells during the pregnancy, it would not appear that the story just
told is at all characteristic of paresis.
However, in the hospital Mrs. Silverman could hardly be got to answer
questions, continually saying, “You know what it is; I don’t have to
tell you.” She claimed so marked a degree of confusion as not to know
where she was and what she was doing. She would beg despondently that
something be done for her, and iterate and re-iterate these claims.
There appeared to be a marked degree of amnesia. Some one, she felt, had
controlled her thoughts and made her do things she did not want to do
and say things she did not want to say, things she did not know she was
about to say. She said, “I feel like jumping around. I couldn’t believe
myself as if I am me. Some one is making me jump around. I used to hear
him talking. I don’t know who it is. I used to keep my eyes open and I
couldn’t move. I feel only I would like to talk, and talk, and talk, and
talk all the time. It seems to me that some one talks in me. I couldn’t
sleep for five minutes. My God, I wish I could sleep! I used to feel
something in my heart. I used to faint. It seems to me I used to see a
funny thing. What it was I can’t tell. It used to talk to me, make me
get out of bed, throw me about, make me do things. O, I don’t know what
it was.”
These not entirely characteristic mental symptoms, together with the
suggestive physical signs and the laboratory examination, caused
treatment to be instituted; under which treatment (intravenous
injections of salvarsan) she improved rapidly. Mental symptoms
disappeared under the administration of 12 injections of salvarsan
within two months. Moreover, the spinal fluid became entirely negative.
Two and a half years have now elapsed since her discharge and she has
shown no return of symptoms. The serum W. R. has always remained
negative although there has been no treatment since leaving the
hospital. There has, however, been no change in the reflexes, which
remain as on admission. The 7–months baby has continued to be perfectly
healthy. Its W. R. is negative, as are the W. R.’s of the husband and
the other three children. It must seem surprising that a healthy child
could have been born from a mother with generalized syphilis as in this
case. However, perhaps there are more instances than we imagine like the
case of baby Silverman.
1. May a patient be considered permanently cured although there has
been no recurrence of symptoms for 2½ years and although the
Wassermann has remained negative? One would hesitate to give a
definite statement that the patient was cured until more time had
elapsed. It is quite possible that spirochetes may be lurking in
some portion of the body without causing the production of
symptoms or Wassermann bodies and yet ready to break out at any
time. This hypothesis has added weight from the recent work of
Warthin already quoted. We advise examination of this patient at
intervals of not longer than six months for a good many years.
2. Should the course under treatment cause us to change the
diagnosis? It has often been stated that a differential point
between cerebrospinal syphilis and general paresis is the reaction
to treatment, that is, that a case which recovers could not be
general paresis. Head and Fearnsides state that if six months
after beginning of treatment the spinal fluid has become negative,
the case should be considered as one of cerebrospinal syphilis and
not general paresis. We do not feel ready to concur in this view
as we know of no similar logic in medicine. We have many cases in
which a spinal fluid has remained positive for six months and
later become negative, so that where the symptoms shown are those
of paretic neurosyphilis, we are inclined to consider the case
such until such time as more definite evidence checked by post
mortem examination causes us to change this point of view.
3. Do the reflexes change under treatment? The signs of spasticity
often do disappear under treatment and also when there is no
treatment. A few instances have been reported in the literature
where Argyll-Robertson pupils are said to have altered to normal.
It has never been our good fortune to see such a change nor have
we seen an absent knee-jerk become normal, as has also been
reported, except where it is the result of pyramidal tract disease
superimposed upon the posterior column sclerosis causing a return
of reflex. This, of course, is not to be considered as a return of
the normal. (See Case 1.)
=Some RESULTS of systematic intravenous salvarsan therapy are
PARTIAL (_e.g._, clinical recovery and persistence of positive
laboratory tests).=
=Case 114.= Walter Henry was an undertaker in a small town. He was
married and the father of two healthy children. In May, 1914, he began
to lose his appetite. He felt restless and seemed to be losing his grip,
and in August he repaired to a sanatorium, where he remained for two
months. Shortly after leaving the sanatorium, he fainted one day, while
digging a grave, during a spell of great heat. Since that time there had
been numerous “weak spells,” with headaches and general debility,
insomnia, and loss of weight.
In February, 1916, Mr. Henry came to the hospital for advice, but the
trip from a distant part of the state was apparently such a strain for
him that shortly after admission he collapsed. There were no convulsive
movements in this collapse, but the patient was confused and his
breathing was rapid and stertorous. The semi-stupor lasted for about 48
hours. Upon recovery from the stupor, Henry was found entirely
disoriented, much confused, and laboring under the belief that he was
digging a grave. After a time he again fell into a stupor and his
temperature rose to 103° F.
The emaciation of this man was striking and unusual, but systematic
=physical examination= showed no special disease. =Neurologically=,
there were marked tremors, and there were purposeless movements of the
arms. There was a marked speech defect. The pupils were dilated,
regular, and equal, and reacted, though slightly, to light. Nothing
abnormal was noted upon systematic examination of the reflexes.
The W. R. was strongly positive in the blood and in the spinal fluid;
the gold sol reaction was typically “paretic”; there were 16 cells per
cmm., globulin was present, and albumin was greatly increased.
The =diagnosis= GENERAL PARESIS was accordingly made, and treatment
instituted. Intravenous injections of arsenobenzol, at first, and later
of diarsenol, were given, as a rule twice a week (usual dose, 0.6 of a
gram). Mercurial injections and potassium iodid were also given. This
treatment was continued as the patient began to improve. The improvement
was of such a degree that at the end of four months, Mr. Henry returned
to his home and his work. He had had 30 intravenous injections of
salvarsan substitutes. Despite the treatment and the clinical
improvement, the laboratory tests remained essentially unchanged. The W.
R.’s of the blood and spinal fluid remained strongly positive, as well
as also the globulin and albumin; the gold sol reaction was still
“paretic”; the cells stood at one per cmm. The patient has continued
antisyphilitic treatment since leaving the hospital, and has remained
apparently well, with good insight into his condition.
1. What is the significance of a temperature of 103° in a paretic
without signs of infection and a normal leucocyte count?
Temperatures of this type are not infrequent in the course of
general paresis. They are usually spoken of as “paretic
temperatures.” Their meaning is not understood, but they are often
stated to be due to a disturbance of the heat-regulating
mechanism. Such temperatures may remain elevated for a
considerable period of time, but the elevation may be very
transitory. At times they vary, like septic temperatures.
2. What can be argued from the fact that the cell count became
normal? If thorough antisyphilitic treatment is vigorously given,
it will be found that in the vast majority of cases of
neurosyphilis the cell count will return to normal. It matters not
whether the treatment be intravenous or subdural. It is very
difficult, however, to obtain this result in general paresis by
the use of mercury alone. It cannot, however, be urged that this
finding has any great prognostic significance as it occurs in the
cases which do poorly as well as in those which recover
symptomatically.
3. Is it safe to give large doses of salvarsan to a patient in a
stupor? It is not a good plan to give a large dose to such a
patient on account of the danger of sudden death. This is probably
due as much to the strain put on the heart as it is to any effect
on the nervous system, or specific arsenic effect. In this
particular instance, a dose of 0.15 gm. was the initial injection
and this was increased five centigrams per injection.
=IMPROVEMENT IN PARETIC NEUROSYPHILIS (“general paresis”) may become
evident only after several months of intensive treatment.=
=Case 115.= Henry Ryan was a shipping clerk, 54 years of age, who was
brought to the hospital following a convulsion. For a few months
preceding this period, Mr. Ryan had been failing in his abilities. He
had been very forgetful, showed no energy, and had become very
irritable. He also complained of insomnia and of feeling nervous.
On admission to the hospital, the most striking feature in the mental
situation was that he claimed that he had not slept a wink for three
months, and each day he would solemnly affirm that he had not slept at
all the preceding night, although the records might show that he had
slept eight hours. Argument was of no avail against this conviction. In
addition, his memory was very poor; he showed little knowledge of
current events, and had no ability with arithmetical problems.
=Neurologically= viewed, the points of chief significance were
contracted immobile pupils and a speech defect, especially noticeable on
the repetition of test phrases. The whole picture was suggestive of
general paresis, and this diagnosis was confirmed by the laboratory
findings. It was found that the W. R. was positive in the blood and
spinal fluid, that there was a pleocytosis, positive globulin reaction,
excess of albumin, and a “paretic” gold sol reaction. Consequently, the
diagnosis of GENERAL PARESIS seemed justified, although the patient
denied any knowledge of a syphilitic infection.
Treatment in this case consisted of intravenous injections of salvarsan,
diarsenol, or arsenobenzol, whichever drug was most easily obtainable,
given twice a week in doses of 0.6 gram each. In addition, he was given
occasional injections of mercury salicylate as well as potassium iodid
by mouth. Once or twice a week, 40 to 60 cc. of spinal fluid were
withdrawn. Under this treatment for a period of three months, the
patient showed no improvement whatsoever, either in his mental condition
or in the laboratory findings. However, treatment was faithfully
persevered in, and shortly after the three months, improvement began to
be noticed. At first, the patient began to admit that possibly he may
have slept a few winks some time during the previous six months, for he
said he realized it was not possible for a man to live without sleep for
that period. Then he began to admit that he might have slept a few hours
during the night, and later that he was sleeping pretty fairly. His
memory also showed improvement. His general attitude showed alertness,
and he began to interest himself in his surroundings and in the events
of the world, and finally he gained complete insight into his condition.
In the meantime, that is after three months of treatment, the laboratory
findings began to grow weaker. The gold sol reaction was the first to
decrease in strength, and after four months of treatment, it vacillated
between negative and a mildly positive “syphilitic” reaction. Then the
globulin and albumin became less in amount, and the W. R. began dropping
off in the 0.1 and 0.3 cc. dilutions. As is usually true in those cases
of neurosyphilis that receive adequate treatment, the cell count early
dropped to normal. The W. R. in the blood serum, however, remained
positive.
As the patient’s condition seemed so much better, he was allowed to
leave the hospital at the end of five months. He took things easily for
the following seven months, and then, after being out of employment for
the period of a year, as his health continued good, he decided to return
to work. Before doing so, he entered the hospital again for a lumbar
puncture. At this time, it was found that the cell count was normal,
there was a very faint trace of globulin, possibly a slight increase
above normal albumin content, and a very mild gold reaction. The W. R.
in the spinal fluid was negative including the 1.0 cc. dilution; the
blood serum remained positive.
The patient then returned to his old position and has done
satisfactorily for the past six months. During this entire time, he has
been coming to the hospital for treatment: during the major portion of
the time, about once in two weeks; of late, once in four weeks.
The significant point in this case is that improvement did not show
itself until after more than three months of intensive treatment, and
then the improvement was synchronous with a weakening of the spinal
fluid tests.
It is further significant that his mental and physical condition was
good before the tests had reached anything like normal; and that under
treatment, these tests continued to grow weaker and weaker, until at the
end of a year, they were practically negative.
The case further illustrates the enormous number of injections of
salvarsan preparations that may be given to a patient without causing
any appreciable damage to the general health or to the kidney function.
Mr. Ryan has had more than 60 injections.
1. How soon after treatment is instituted does improvement usually
occur in paretic neurosyphilis? In our experience improvement
usually shows itself in from two or three months of treatment.
Occasionally the improvement may be very marked shortly after
treatment is commenced, that is, after three or four injections of
salvarsan. This is not, however, the rule and as in the case of
Ryan, it may be only after more than three months that improvement
is seen. This means that in the treatment of these cases patience
must be exercised and much work done.
2. What is the point of withdrawing large amounts of spinal fluid as
in the case of Henry Ryan? It has been stated that the withdrawal
of 40 or more cc., of spinal fluid while the patient is under
treatment has the effect of reducing the intraspinous and
intracranial pressure and thereby allowing the drug to diffuse
into the nervous tissue better than it would do under ordinary
conditions. How much truth there is in this contention it is
difficult to say and there is as yet no experimental evidence to
confirm this contention. As a matter of fact, the spinal fluid in
cases of paresis is usually under increased pressure and it is at
least plausible to conceive that a reduction of this pressure may
give some symptomatic relief.
=Evidence of the activity of syphilis outside the central nervous
system may be seen in cases of neurosyphilis despite intensive
treatment.=
=Case 116.= William Rosetti was a speculator, 43 years of age, when he
was brought to the Psychopathic Hospital on account of an outbreak in
which he smashed a showcase at the store where his sweetheart was
employed; he caused so much commotion that he was arrested.
On admission, he was very excited, talking loudly and at length. For
some days it was very difficult to manage him, he was so active. At any
moment, he would insist upon undressing and taking physical culture
exercises. He was very euphoric and expansive, and had no insight into
his condition.
=Physically=, he was a powerfully-built man and in very good physical
condition except for an iritis and moderate thickening of the peripheral
arteries. The =neurological signs= of importance were Argyll-Robertson
pupils, and absent knee-jerks and ankle-jerks. With these findings in
mind, a tentative diagnosis of GENERAL PARESIS was made, and this was
substantiated by the laboratory tests, which gave positive W. R.’s in
blood and spinal fluid, globulin, excessive albumin, slight pleocytosis,
and a “paretic” gold sol reaction.
When the patient’s mental condition was somewhat better, he gave a
history of syphilitic infection 15 years before, for which he had had
almost continuous treatment. As a matter of fact, treatment had been
pretty strenuous because he had recurring skin lesions and iritis. It
was practically impossible to get the skin lesions to heal with mercury,
and it was not until salvarsan was introduced that a good result was
obtained in this respect. After one or two injections of this drug, the
skin lesion disappeared and has never returned. However, at least once a
year, he has had attacks of iritis, and for this reason was still being
treated for syphilis at the outbreak of his psychosis.
He was at once placed on more strenuous antisyphilitic treatment in the
form of diarsenol, semi-weekly, aided by mercury injections. After a few
months of this treatment, his mental condition improved so much that he
seemed to be entirely normal. Treatment was continued, however, without
any abatement, and it was of great interest to note at the end of five
months of such treatment that, although mentally he seemed entirely
well, he had an attack of iritis, which was considered as a sign of
active syphilis. This would appear to indicate the great difficulty of
getting results in certain cases of syphilis with any drugs at our
command at present, as in the iritis we are dealing with a condition
which as a rule reacts fairly readily to antisyphilitic remedies.
1. Are there different strains of spirochetes showing various
degrees of malignancy? This question has been discussed at length
in the literature but there is no satisfactory answer at the
present time. We must always consider the reaction of the organism
and the host; and it is true in syphilis, as in every other
disease, that in some individuals it is more difficult to get any
therapeutic results than in others.
2. Was the failure to obtain results by long years of treatment due
to “drug fastness” of the spirochetes? It has been held that the
organism of syphilis will develop an immunity after a time to
mercury and arsenic preparations. This led Fournier to recommend
intermittent treatment as more efficient than continuous
treatment. Noguchi has shown that in test tube experiments, the
spirochetes develop a tolerance to increasing doses of arsenic. It
must be emphasized, however, that this finding has not been
established for the conditions _in vivo_. Another explanation of
the failure of treatment in certain instances has been offered by
McDonagh, who describes a life cycle of the organism of syphilis
under the name of _cytorrhyctes luis_, of which he believes the
spirochete to be merely one form, the other forms not being
affected by arsenic or mercury.
=Some results of systematic intravenous salvarsan therapy in PARETIC
NEUROSYPHILIS (“general paresis”) are partial in the sense that with
clinical recovery the laboratory tests remain partially or less
strongly positive.=
=Case 117.= Annie Martin was a charwoman, 37 years of age. She had
applied for relief at a general hospital, to which she was admitted on
the suspicion of nephritis; but upon admission she became markedly
excited and noisy, and spoke of seeing angels and hearing God speak to
her. As the attendants were unable to quiet her, she was promptly
transferred to the Psychopathic Hospital. She maintained that she had
been sent to the Psychopathic Hospital through the spite of the general
hospital doctors, and she claimed that other people were also attempting
to work her harm for the purpose of taking her children from her. Visual
and auditory hallucinations were marked, as was the patient’s loquacity,
irritability, and flight of ideas. However, she seemed entirely oriented
and her memory appeared to be intact. She was able to explain somewhat
clearly her supposed condition. The voices told her that somebody was
after her and that her soul belonged to the devil; that she was to be
married but that her soul was to be damned. These voices probably
belonged to priests. She was under the impression that she was going to
be sent to an electric chair and said, “I think I am coming to the end
and I want a pair of rosary beads before the end comes.”
This patient’s pupils were markedly unequal and entirely stiff to light
and accommodation. =Neurologically=, however, there were no other
symptoms. There was a slight trace of albumin in the urine and there
were no casts.
The psychiatric =diagnosis= in this case would off-hand undoubtedly be
dementia praecox. Yet the stiff pupils are almost proof positive of
neurosyphilis. If further proof were necessary, it is found in the
laboratory tests, which showed a positive W. R. of the serum and fluid,
with a “paretic” gold sol reaction; there were 22 cells per cmm., there
was excess albumin, and a positive globulin reaction.
Under intensive antisyphilitic treatment, there was a slow improvement.
After several months, the patient was entirely free from mental
symptoms; the spinal fluid tests became entirely negative except that
the gold sol reaction has remained strongly positive.
1. Should treatment be continued in the case of Annie Martin in
spite of the clinical recovery and the negative tests except the
gold sol? We would again emphasize that it is unreasonable to
suppose that a long-standing case of syphilis can be cured in a
period of a few months of treatment and while the tests may become
negative, it would seem foolhardy to stop treatment on this
account. We do know that in many cases a Wassermann reaction
remaining negative for many months may again become positive,
indicating that the negative reaction did not mean cure but rather
the absence of the Wassermann bodies in the circulation at the
time the test was made.
2. What is the significance of the paretic gold sol reaction when
the other tests have become negative? As previously stated, the
gold reducing substance in the spinal fluid seems to be different
from the substances which give the other pathological reactions.
We should feel in this case that the process which was producing
these gold reducing bodies had not been stopped, in other words,
cure was not complete.
3. Should one make a diagnosis on the “paretic” gold sol reaction
alone? The so-called paretic gold sol curve is not always
indicative of general paresis or even of syphilis but may occur in
non-syphilitic conditions as brain tumor, multiple sclerosis, etc.
In our experience we have seen no case of _untreated_
neurosyphilis in which the gold sol alone was positive, that is,
in cases in which therapy has not changed the findings in the
spinal fluid. In our experience the gold sol reaction has been
fortified by one or several of the other tests as the W. R.,
globulin test, pleocytosis.
=Some effects of systematic intravenous salvarsan therapy in PARETIC
NEUROSYPHILIS (“general paresis”) are limited to the laboratory
findings without clinical improvement.=
Two examples of such limitation are offered: William Roberts (118) and
John Silver (119).
=Case 118.= A bank teller, William Roberts, 39, was sent to the
Psychopathic Hospital for a depression so marked that he had become
entirely unable to work or care for himself. The story was that some
money had been left him by his uncle, that Roberts could not prove his
right to the money, and that depression, insomnia, and occasional
periods of confusion had followed during a period of about five months.
On admission, Roberts appeared wholly disoriented and unable even to
give his correct age. Attention could not be held, and the patient would
slide off into statements like: “Oh, I made a mistake, I fooled a lot of
people, I have a terrible disease, they are going to get it, they are
going to get me,” etc., etc. There was great difficulty in thinking, and
a marked reaction of fear. This cluster of phenomena certainly suggested
very strongly the diagnosis of manic-depressive psychosis.
=Neurologically=, Roberts proved quite negative except that the tendon
reflexes were very active and the pupils reacted somewhat sluggishly to
light. The blood serum W. R. was negative. No history of syphilis could
be obtained; nevertheless, Roberts kept dropping remarks about the
terrible disease from which he was suffering. It seemed best to proceed
to lumbar puncture, and the spinal fluid disclosed a positive W. R.,
globulin, increased albumin, pleocytosis, and “paretic” gold sol
reaction.
The =diagnosis= of GENERAL PARESIS was accordingly made. During the next
year and a half, no improvement was made; a slight speech defect was
developed, and tremors of the hand and tongue appeared.
The effect of treatment is particularly instructive. Only after 18
months in the hospital was intensive antisyphilitic treatment
instituted; but after a few months of this treatment the W. R. of the
spinal fluid had become negative, the cells normal in number, globulin
absent, albumin present only in normal amount. Only the gold sol
reaction remained positive. It is still of a paretic type. Treatment,
however, did not succeed in altering the patient’s mental condition in
the slightest. At the end of many months of treatment, we still confront
a man showing marked psychic symptoms and a “paretic” gold sol reaction
without other laboratory signs.
1. What is the significance of the practically negative tests in
this case without clinical improvement? One must believe that the
tests became negative as the result of treatment, and that this
change in the tests was due to the clearing up of some
inflammatory reactions which were present. This may mean that the
syphilis had been reduced to inactivity or latency if not cured,
or at least that there was no activity sufficient to cause a
positive W. R. in the blood serum, whereas whatever activity was
present in the brain was in such a region that it did not cause
any reacting substances to be cast into the spinal fluid. This
would not mean that there would necessarily be any return of
function already lost, because this may be considered as a
permanent loss which cannot be compensated for. As to these tests,
we now feel that the case should remain stationary; that is, that
no new symptoms will be added. However, we believe that it is
somewhat premature with our present knowledge to make this claim
very forcibly, and would rather suggest that this case be
considered as demonstrating an interesting fact, the meaning of
which can be learned only after a period of years.
2. Why does the gold sol reaction remain strongly positive when all
the other tests become negative? As already pointed out, above
(Case Martin (117)) there is no known rule about the disappearance
of one or other of the abnormal findings in spinal fluid under
treatment, and we can at present offer no explanation of this
phenomenon. It does, however, illustrate how careful we must be in
drawing any conclusions from tests in cases that are being
treated.
=Diminution in the spinal fluid tests may occur in treated cases of
neurosyphilis without clinical improvement.=
=Case 119.= John Silver, a man 29 years of age, presented classical
symptoms of GENERAL PARESIS: He had a convulsion shortly before his
admission to the Psychopathic Hospital, his memory was poor, he was only
partially oriented, he was very euphoric and expansive—thought he had
millions, that he was the Czar of Russia, and so on. His tendon reflexes
were very much increased and there was a marked speech defect. The W. R.
of both blood and spinal fluid were strongly positive; the spinal fluid
showed globulin, increased albumin, pleocytosis, and a “paretic” gold
sol reaction. There was, therefore, no question about the diagnosis, and
the patient was at once put under antisyphilitic treatment. This was
continued for five months; slowly the intensity of the reactions in the
spinal fluid diminished. At the end of the five months, there was the
very slightest possible trace of globulin, with a doubtful increase in
albumin, one cell per cmm., and a mild syphilitic gold sol reaction. The
W. R.’s in the blood and spinal fluid, however, remained strongly
positive. There was no mental improvement coincident with the weakening
of the spinal fluid tests, and at the end of the five months, the
patient had a series of convulsions in which he died.
This case is given as a contrast to Case Henry (114) in which clinical
improvement occurred without diminution in laboratory tests; in the case
of John Silver, marked diminution in the intensity of these tests had no
prognostic significance. This was in keeping with the condition as shown
in Case Roberts (118) where, while the gold sol was the only test to
remain positive, the patient did not improve mentally.
1. What is the explanation of the lessening of the pathological
elements in the spinal fluid under treatment? We have seen that
the various findings may occur independently of one another, and
we must admit that we do not know definitely what it signifies, or
why one may be present or absent. It has been held by Head and
Fearnsides that the findings in the spinal fluid represent
conditions in the spinal cord and spinal meninges, or at the base
of the brain only, and not conditions elsewhere. This is in
keeping with our finding that the gold sol reaction in the spinal
fluid post mortem very often differs from that in the ventricular
fluids or cerebral, subdural, and subpial fluids. And further, we
have found that during life the findings in paresis in the spinal
fluid may differ markedly from those in the third ventricle, and
that the change in the fluid in these two areas under treatment
may not occur simultaneously.
=Systematic intensive treatment of PARETIC NEUROSYPHILIS (“general
paresis”), including intraventricular injections of salvarsan, may
entirely fail.=
=Case 120.= James McGinnis, aged 39, came to the hospital on a
stretcher, semi-conscious, moaning, unable to reply to questions; there
were signs of a right hemiplegia.
The next day, McGinnis cleared a little and became able to utter a few
words. His wife said that he had been entirely well up to four years
ago. At that time he was struck in the eye by the head of a hammer that
flew off the handle. Diplopia had developed, but disappeared.
Only two years later did a marked change appear. McGinnis became
careless as to personal appearance. Seemed absent-minded, apathetic and
drowsy; he would fall asleep in his chair or while at work. He lost his
position and became apprehensive, making not very strenuous efforts to
find work, and finally consulted a physician. The physician told him
that he had a sluggish liver and gave him calomel.
Six months later, McGinnis was restored to his position as foreman, and
his work remained satisfactory for some six months. Then (about six
months before coming to hospital), his speech became slow and somewhat
unintelligible. He quit work, saying that his speech was going from him
and that he might be considered to be drunk. His memory grew rapidly
worse. There was improvement after a vacation and he returned to work,
but continued to be ataxic, complained of vertigo, and fell down several
times, though without loss of consciousness. On the very day of his
admission to the hospital, in attempting to get out of bed, he fell, and
psychotic symptoms at once appeared. There was slight improvement again
with entire disappearance of all paralysis after a few days, a slow
clearing up of the speech disturbance, and a certain return of memory.
=Physically=, there was little to note. =Neurologically=, the left pupil
failed to react to light. The tendon reflexes were all very active, and
more active on the left side. Other abnormal reflexes were absent.
Improvement continued for a number of weeks, but the patient never
recovered from his speech defect, and his memory remained impaired.
Irritable at times, McGinnis was for the most part very happy and sure
he would get well. The W. R. of the blood serum was negative, but the
spinal fluid reaction was strongly positive, even down to 0.1 cc. The
globulin and albumin amounts were excessive. There was a “paretic” gold
sol reaction. There were 7 cells per cmm. The diagnosis of GENERAL
PARESIS was made.
Intravenous injections of salvarsan, arsenobenzol or diarsenol were
made, and intramuscular injections of mercury, and potassium iodid by
mouth were given. No real improvement occurred after a certain initial
betterment; the spinal fluid yielded no changes. Diarsenolized serum
according to the Swift-Ellis technique was then injected into the third
ventricle. Under this treatment also there was no change for the better
over a period of several months. The patient died suddenly after a
series of convulsions, apparently from paralysis of respiration.
1. What are the causes of hemiplegia and confusion or
unconsciousness? We must consider epilepsy, brain tumor, cerebral
thrombosis, cerebral hemorrhage, multiple sclerosis, cerebral
spinal syphilis, and general paresis.
=MILD TREATMENT, often thought “adequate,” MAY FAIL, WHEN INTENSIVE
TREATMENT PROVES SUCCESSFUL.=
=Case 121.= Arthur Bright, a printer, had acquired syphilis in his 49th
year, some six months before examination. He had been treated during
these six months by three injections of salvarsan, injections of
mercury, and mercury by mouth. He had been apparently cured until about
a month before admission. He had fallen without warning from his chair
in a convulsion accompanied by unconsciousness, which lasted about two
hours. The patient had since been feeling rather peculiar. For instance,
time seemed to flow too rapidly. Sometimes the patient had had
difficulty in talking.
=Physically=, nothing abnormal could be found either in general
condition or =neurologically=. The patient was, however, incontinent.
=Mentally=, he was apathetic and unalert, even paying no attention to
his outside physician when he came to visit him.
The =diagnosis= of cerebrospinal syphilis already suggested by his
history was confirmed by the laboratory tests, which showed a positive
serum and spinal fluid W. R., paretic gold sol reaction, 41 cells per
cmm., an excess of albumin, and a positive globulin test.
1. What is the prognosis in cerebrospinal syphilis in the early
secondary stage? The prognosis appears very good provided that
intensive treatment be given and provided that no vascular insult
or other focal destructive lesion occurs before treatment has had
time to do its work.
2. Why did not the “effective” (?) treatment for the syphilis,
dating from the primary lesion, succeed in staving off the
cerebrospinal syphilis? It remains a question whether the
treatment by three injections of salvarsan was efficient in this
particular case. Of course, it may prove true that no treatment
whatever in the present stage of knowledge will stave off
cerebrospinal symptoms in certain cases.
=Treatment=: Bright was given intravenous injections of diarsenol twice
a week, with occasional injections of mercury salicylate. After two
weeks, the patient seemed markedly improved, and continued to improve
rapidly. He was symptomatically well at six weeks. The spinal fluid had
then become negative, although the serum W. R. had remained positive.
After discharge from the hospital, Bright returned to his work, but
continued to take the diarsenol treatment weekly, and two months later
the serum W. R. became negative.
Small injections of diarsenol at intervals of a month were continued,
and Bright remained perfectly well for four months, when a peculiar
seizure developed and lasted for several hours. This seizure consisted
in a sort of somnambulism in which Bright stood up at a table, making
marks on paper, and could not be persuaded to desist. After this
seizure, Bright re-entered the hospital, again showed no mental or
physical symptoms and no abnormalities of blood or spinal fluid.
3. What is the explanation of this seizure? It is possibly due to a
small vascular insult, for which potassium iodid may be suggested
with precautions as to hygiene and continued observation. He has
since remained entirely well.
=Another example where MILD MEASURES (though conceived to be
“adequate”) SEEMED TO BE LEADING TO FAILURE; INTENSIVE THERAPY
SUCCESSFUL.=
=Case 122.= Levi Morovitz, a waiter, 39 years of age, came to the
hospital with evidences of an old left hemiplegia, including the left
side of the face (there was a left-sided Babinski, Gordon, and
Oppenheim, and all the reflexes were fairly active; sluggish pupil
reactions, Rombergism, and speech defect). Morovitz was much depressed,
very slow in thinking processes, had a marked memory disturbance in
general and apparently much deterioration mentally.
A history was obtained to the effect that Morovitz had acquired syphilis
at about 33, but that he had received practically continuous treatment
ever since at a dispensary. He had, in fact, received four injections of
salvarsan a year before coming to the hospital. Of late, Morovitz had
become much more cheerful and talkative, imagining he could do great
things if he had money. He had begun to eat very rapidly and to be very
nervous. His feet had begun to drag; a distinct speech defect developed,
but from this he had recovered. About six weeks before entrance,
Morovitz had a shock, which left him with the left hemiplegia above
mentioned and with considerable headache.
Even while the preliminary examination was being performed, Morovitz
developed a minor seizure without loss of consciousness. First came
severe pain over the frontal region, which grew in severity so that the
patient held his head in his hands. A bit later, twitching movements
began in the thumb and in the fingers of the left hand, and the small
muscles of the extensor group of the thumb and third finger showed
contractions. These contractions grew more general and the excursions of
the fingers greater, until finally every finger of the left hand became
involved, whereupon movements of the same sort, though of smaller
amplitude, began in the other hand. Finally the left arm began to jerk
with alternate contractions of the biceps and triceps. The whole seizure
lasted more than five minutes. During the seizure there was dizziness
and pain in the head, chiefly on the right side.
=Diagnosis=: The attention is at once arrested by the data of the
seizures described. It appeared that we had to assume an irritation of
the right side of the brain, possibly due to vascular disease, or to
brain tumor, or perhaps to syphilis. The shock with residual hemiplegia
would be consistent enough with any of these diagnoses. However, the
history seemed somewhat long for brain tumor. Nor were there any
definite symptoms of intracranial pressure. “Adequate” treatment
unfortunately does not rule out syphilis. The comparatively early age
(39) of the patient makes it difficult to explain the vascular disease
except on the basis of syphilis. Add to the hemiplegia the euphoria and
grandiose ideas of a year’s duration, and we arrive at a diagnosis of
neurosyphilis, probably PARETIC NEUROSYPHILIS.
The laboratory tests showed the W. R. of the serum and spinal fluid
positive, 80 cells per cmm. in the fluid, large amounts of globulin and
albumin, and a “paretic” type of gold sol reaction.
To be sure the Jacksonian seizure is not especially characteristic of
paretic neurosyphilis, and even suggests a local irritation in the motor
area, such as a localized meningitis, possibly of a diffuse gummatous
nature.
This patient was put on intensive antisyphilitic treatment, namely,
salvarsan twice a week and injections of mercury. He recovered rapidly.
After a few months he left the hospital, and after treatment had
continued for a year, he resumed his work by which time both blood and
spinal fluid had become negative.
It must be recalled that this patient had from the time of his infection
what has been considered good antisyphilitic therapy, in spite of which
he developed after a period of years, the symptoms and signs of
neurosyphilis in its most dangerous form. The conclusion must be drawn
that however good such treatment is for the majority of cases, it was
insufficient for Morovitz. That the early failure to cure was not due to
any “drug fastness” of the spirochete or to any peculiarity of strain is
proved by the result of more vigorous antisyphilitic treatment which
caused an apparent if not a real cure. With our modern methods of
treatment checked by Wassermann reactions and spinal fluid examinations,
treatment is given according to the _needs of the individual patient_
rather than according to general preconceptions. We have reason to
believe that under these conditions there will be fewer cases developing
late symptoms on account of insufficient treatment given even to
patients who are willing to co-operate to the last degree.
The fact that Morovitz had no apparent symptoms for several years led to
rather desultory treatment chiefly in the form of mercury by mouth.
Previous to the time when the W. R. and lumbar puncture were available,
the physician had no exact means of determining cure except the
non-appearance of symptoms. But a period of years of quiescence before
the outbreak of symptoms referable to the involvement of the nervous
system is characteristic of syphilis. With this knowledge in mind it is
evident that today the care of a syphilitic patient must be guided, in
part at least, by examinations of the spinal fluid and W. R.
=Salvarsan treatment may even occasionally be of value in simple
FEEBLEMINDEDNESS due to congenital syphilis.=
=Case 123.= The somewhat unattractive Robert Matthews was brought, at 5
years of age, to the hospital for backwardness of mind. It appears that
the patient was born at term, with instruments, that he began to talk at
a year, and to walk at 13 months, but that in point of fact, he had not
talked intelligibly to date. Robert had never played with other children
and is regarded by his parents as backward. In fact, Robert’s sister—a
year his junior—is much brighter. Robert had had scarlet fever but
without sequelae.
Examination by the Binet scale showed that, although he is actually 5½
years, he graded by the Binet scale at 4 and was regarded as
feebleminded.
The =physical examination= showed a general adenopathy and prominent
frontal bosses. In the study of the family history in the search for an
etiology for the evident feeblemindedness, little or none could be
found. There were no miscarriages or stillbirths; the parents were
living and well. There was only the one sister above-mentioned, who is
brighter than Robert.
The advantage of a routine W. R. is here well shown, for the W. R. in
the serum was positive.
1. What is the prognosis of cases of syphilitic feeblemindedness? It
would appear that every case is an individual problem.
2. What is the effect of treatment? Robert Matthews was given
mercury protoiodid ⅛ gr., three times a day, by mouth, for three
months. The protoiodid was followed by ten injections of
salvarsan, average: 0.15 gram, during six months. At the end of
this period, the W. R. in the blood had become negative. A
re-examination by the Binet scale, when Robert was 6–5⁄12 years of
age, showed him to grade at 5⅖, so that one might conclude that
Robert had shown more mental progress in a year than he had
previously.
Note: The patient’s sister, 4 years of age, is attractive and bright,
measuring beyond her actual age according to the intelligence tests.
However, the girl was found to have a positive W. R. It may be that
Robert and his sister illustrate the hypothesis of Mott: that the
syphilitic virus becomes less potent as the years go on, and that the
younger children in the family are less affected than the older.
However, in our series, there are a number of instances in which this
hypothesis is not substantiated.
3. What is the share of syphilis in the production of
feeblemindedness? The percentage of syphilitic cases found in
institutions is not high. A variety of cases have been proved to
be congenitally syphilitic in the absence of a positive serum W.
R.
Fernald[19] has charted a comparison of cases diagnosticated “moron”
(that is, feeblemindedness proper, in the narrower English sense) and
“imbecile.” Fernald says that the morons have, as a group, many more bad
family histories than have the imbeciles, to quote—“Only 70% of the
[imbecile] group have bad family histories. This at first seems
surprising, but when we consider that more of our syphilitic, traumatic,
and sporadic cases tend toward the lower end of the feebleminded group,
and when we remember that with such cases there is often a seemingly
normal family tree, the drop in the curve appears logical.”
The situation with the idiots, of whom only 38 came into Fernald’s
study, was similar; 12 out of 38, or 32%, of idiots, had good family
histories. On these figures, how unfortunate it would be to dub
feeblemindedness hereditary! It is true, however, that 68–70% of the
idiots and imbeciles, judging by W. E. Fernald’s intensive study, do
have bad family histories.
Goddard[20] states that of all the causes of feeblemindedness, there is
perhaps none for which there is less evidence than syphilis. Goddard
found syphilis in 27 of his intensively charted cases of
feeblemindedness, that is, in 9% of all his charts. He finds the
majority of the syphilis cases occurring in relatives of the
feebleminded to be in the hereditary group; for example, of 164 charts
in the hereditary group, 17, or 10%, showed syphilis. In 34 charts in a
group termed “probably hereditary” 3, or 9%, showed syphilis. Of 37
charts in the group termed “neuropathic” 4, or 11%, showed syphilis,
whereas in 57 “accident” and 8 “no cause” groups, there were but 2 (4%),
and one, or 13%, showing syphilis. However, Goddard concedes that much
more careful studies are necessary if we are to give an exact evaluation
of syphilogenic feeblemindedness.
The first ten of the Waverley Anatomical Series are shortly to be
described in a forthcoming publication.[21] Of these ten cases, four
showed some slight evidence of chronic inflammatory changes, indicating
the possibility of a syphilitic or similar infectious condition. These
cases, be it remembered, were not cases of juvenile paresis, but cases
of what, for the lack of a better name, may be called “ordinary”
feeblemindedness.
If all or any of these processes are syphilitic, the syphilis is
virtually extinct. The cases had not been treated for syphilis and were
not regarded as syphilitic, though several of them showed a few stigmata
somewhat suggestive of syphilis. The anatomical conclusion at this time
is still doubtful.
As in the text case, the hypothesis of syphilis as a direct cause for
simple feeblemindedness must be entertained for a few cases. In any
event, it would not seem logical to let any institution for the
feebleminded run without a Wassermann analysis of the population. In
addition to the Wassermann data from the blood serum, osteological data
from the X-ray have proved of occasional value for syphilis diagnosis in
this as in other groups.
“Within the gates of Hell sat Sin and Death.”
Paradise Lost, Book X, Line 230.
VI. NEUROSYPHILIS AND THE WAR
Although the American toll of war syphilis has not yet begun and
although the crop of neurosyphilis due to war infections may not arrive
until the mid or late twenties of the century (witness German experience
in the eighties of the last century), it seems proper here to give a
number of abstracts _re_ neurosyphilis as it has developed in the war.
Available reports from English, French, and German sources have been
levied upon for the years 1914–16.
It is clear that all the armies have had their share of
neurosyphilitics, some clearly diseased before enlistment, some
developing symptoms as a result of training, stress, or shock, others
hastened or made worse by war conditions.
There are important questions of pension, retirement, and compensation
for neurosyphilitics. No previous war has had the benefit of the
Wassermann reaction and other exact tests bearing upon the nature,
progress, and curability of neurosyphilis.
That we shall have our fill of pension and other problems can already be
seen from continental reports. Thibierge,[22] for example, states that
syphilis has become a real epidemic among the French soldiers and
mobilized munition workers.
Hecht[23] of Austria claims that no less than an equivalent of 60 army
divisions have been temporarily withdrawn from fighting on the Teutonic
side for venereal diseases. He commends Neisser’s idea that salvarsan
and mercury should be given in the trenches. While hundreds or thousands
of Austrians are sick with syphilis, sound and healthy men are being
shot down in their stead. The diagnosis of syphilis, according to Hecht,
ought to be a signal for sending the men to the front. He makes even the
somewhat bizarre suggestion that special companies of syphilitics should
be formed, for convenience of treatment, on the firing line.
Not only is the syphilis problem in the army of importance to the
military authorities, but also to the civil population, and perhaps to
them a greater problem. With the great increase of venereal disease that
is the result of the conditions of army life in war time, there will be
a considerable percentage of cases developing neurosyphilis a number of
years after discharge from the army, but caused by the infection
acquired during service. In addition many men will bring the disease
back to America in an infectious stage and spread it. We would advocate
that the names of all soldiers who had acquired syphilis and were not
considered cured at time of discharge should be given to health
organizations in their home states that they may be given further care.
These practical and several theoretical questions are raised by the
following fourteen cases which we have condensed from their sources.
=A tabetic lieutenant “shell-shocked” into paresis? Case from Donath
of Vienna.=
=Case A.=[24] An apparently competent German professor in an
intermediate school, a lieutenant of infantry reserves, 33 years old, on
the 17th August, 1914, was stunned for a while by the shock of a
cannon-firing 25 feet away. Urination became difficult. Headaches and
limb pains ensued, with paralysis of fingers, gastric troubles,
forgetfulness especially for names, insomnia, and general scattering of
mental faculties.
=Neurologically=, the pupils were irregular, left larger than right;
Argyll-Robertson reaction. Right knee-jerk livelier than left. Achilles
reactions absent. Slow and dissociated pain reactions in feet, lower
thighs and lower quarter of upper thighs, with hypalgesia or analgesia.
Station good; gait steady. Mentally depressed, slow of thought. Speech
poor and of indistinct construction (mild dementia). Calculation ability
poor. No pleasure in work.
Wassermann reaction of serum weakly positive.
It seems that for a year the patient had been subject to spells of
anger. He was irritated by his wife who had been nervous since an
earthquake.
_On the occasion of the earthquake_, 1911, the patient himself had had a
spell of _difficulty with urination_. The spell had lasted two or three
months. The patient had had a chancre in 1902, “cured” in four or five
weeks with xeroform. In 1908, when about to marry, he had had six
mercurial inunctions.
1. Is this a case of traumatic paresis? From the somewhat meagre
account it would appear that Donath’s lieutenant should rather be
termed “shell-shock paresis,” in the sense of a paretic
neurosyphilis liberated by shell-shock (using shell-shock in the
sense of a shock _without_ direct brain injury).
2. What compensation is due such a man as Donath’s lieutenant? The
ordinary principles applicable to traumatic paresis are not here
in point, since no symptoms pointing to trauma of brain ever
supervened. See discussion under Case G.
3. How frequent is paresis in armies? R. L. Richards in White and
Jelliffe’s Treatment of Nervous and Mental Diseases writes as
follows (of course concerning peace times):
“The French estimate that paresis cases are 7 per cent of all
their military cases. The German estimate is 6.6 per cent. In our
own army at the Government Hospital for the Insane, of 490 cases
of mental diseases among officers and enlisted men, 37, or 7 per
cent, were paresis. During the Russo-Japanese War, in the Russian
Psychiatric Hospital at Harbin, the percentage of paresis was 5.6
per cent among the cases developing at the front.”
=A French soldier “shell-shocked” (also burial) into incipient tabes
dorsalis? Case from Duco and Blum of Paris.=
=Case B.=[25] A French soldier was buried by effects of shell explosion
September 8th, 1914. He sustained no wound or fracture.
Incontinence of urine developed. Anesthesia of penis and scrotum.
Reflexes absent; pupils sluggish. Wassermann reactions suspicious.
The diagnosis =tabes dorsalis incipiens= was made (hematomyelia of conus
terminalis eliminated).
The patient was estimated to be “40% incapacitated,” according to the
French “_échelle de gravité_” of conditions. A full pension would not be
justified in the opinion of the French authors.
1. Is there evidence of an increase or exacerbation of tabes
dorsalis in the war? Birnbaum,[26] reviewing German war neurology,
quotes Weygandt as believing that the war has probably had to do
with the production of both tabes and paresis in many instances.
Other cases, however, have merely been made worse by the war
stress. Thirdly, there are cases in which the war stress has done
no harm whatever. Westphal has seen both tabes and paresis develop
in men who had never before shown any mental or physical symptoms
whatever, and accordingly, Westphal must be counted among those
who regard war stress as a liberating factor for these diseases.
Redlich and Donath are cited in the same connection. (The case of
Donath is the case presented above as Case A.)
A very interesting claim was made by Cimbal to the effect that he
found many examples of paresis developing in the early period of
the war, particularly in November and December, 1914. Later,
according to Cimbal, cerebrospinal syphilis and tabes became more
prevalent.
=Neurosyphilis in a German recruit, possibly AGGRAVATED ON military
SERVICE. Pension not allowable. Case from Weygandt.=
=Case C.=[27] A German, long alcoholic and thought to be weakminded,
volunteered, but shortly had to be released from service. He began to be
forgetful and obstinate, cried, and even appeared to be subject to
hallucinations. The pupils were unequal and sluggish. The uvula hung to
the right. The left knee-jerk was lively, right weak. Fine tremors of
hands. Hypalgesia of backs of hands. Stumbling speech. Attention poor.
It appeared that he had been infected with syphilis in 1881 and in 1903
had had an ulcer of the left leg.
The military commission denied that his service had brought about the
disease. In the phrase of the Canadian Pension Board the German
commission would probably have rendered a report “aggravated on
service,” not “by service.” (See Canadian cases D, E, and F.)
1. Has paresis increased in the war? Both French and German figures
controvert the claim. Marie, for example, found not a single
paretic amongst the skull injury cases at the Salpétrière. Most
authors are found demonstrating cases which they clearly regard as
in some way produced or unfavorably influenced by the war. There
seems, therefore, to be a little inconsistency between the general
statement that paresis has not increased in the war and the
somewhat frequent cases described as occurring in and modified by
the war. However, Bonhoeffer, on the basis of nine months’ war
experience, also holds it to be probable that paresis is no more
frequent in the field than in the home population.
2. Is the old syphilitic especially liable to break down under war
conditions? According to Richards, Shaikewicz says that in the
Russo-Japanese war paresis was noted especially among the officers
and non-commissioned officers, and that it was undoubtedly
hastened in its development by war conditions. Steida says that
while ordinarily we find paresis developing twelve to twenty years
after the primary sore of syphilis, in these cases it developed in
five to ten years after the primary sore. Some of the cases
progressed with unusual rapidity. It was also noticed that among
soldiers from the front, under treatment, evidences of syphilis
were present in 20%, while among the other soldiers under
treatment, evidences of syphilis were present in 1.6%. Undoubtedly
the old syphilitic is especially liable to break down under war
conditions.
But, on the whole, the German authors in this war find no evidence
favoring Steida’s claim of the hastened post-infective outbreak.
3. How did it come about that the efficient German system
permitted this alcoholic and weakminded syphilitic to enter the
army? As will be seen, he was a volunteer. In general, the German
system has been supplied with army surgeons who have been trained,
not by brief and “brush-up” courses, but by longer periods,
sometimes two years in duration.
=Syphilis contracted before enlistment, “AGGRAVATED BY SERVICE.”
Canadian case, courtesy of Dr. J. L. Todd, Canadian Board of Pension
Commissioners.=
=Case D.= A laboring man, 42, who always strenuously denied syphilitic
infection, proceeded to France eight months after enlistment. He had not
been in France three weeks when he dropped unconscious. He regained
consciousness, but remained stupid, dull in expression, and with memory
impaired. His speech was also impaired. There was dizziness and a
right-sided hemiplegia.
He was confined to bed four months and was then “boarded” for discharge.
=Physically=, his heart was slightly enlarged both right and left;
sounds irregular; extra systoles; aortic systolic murmur transmitted to
neck; blood pressure 140:40. Precordial pain, dyspnea.
=Neurologically=, there was a partial spastic paralysis of the right
thigh which could be abducted, could be flexed to 120°, and showed some
power in the quadriceps. There was also a spastic paralysis of the right
arm, but the shoulder girdle movements were not impaired. There was a
slight weakness on the right side of the face. There was no anesthesia
anywhere.
The deep reflexes were increased on the right side, Babinski on right,
flexor contractures of right hand, extensor contractures of right leg,
abdominal and epigastric reflexes absent, pupils active, tongue
protruded in straight line.
Fluid: slight increase in protein. W. R.+++
The Board of Pension Commissioners ruled that the condition had been
aggravated _by_ service. (See Case E, “aggravated _on_ service.”)
1. In view of the fact that the majority of the cases here
abstracted happen to be in common soldiers, is there any evidence
bearing on relative incidence in officers and men? Quoting R. L.
Richards:
“The percentage of paresis cases among officers alone is variously
estimated from 50 per cent in the German army (Stier) to 58.9 per
cent in the Austrian army (Drastich). Since paresis is a disease
of more advanced life, it is but natural that the percentage of
paresis among officers, non-commissioned officers, and older
soldiers should be higher than among the whole military body,
where the average age is, as we have seen, well below thirty
years. Hence the above figures do not mean a greater prevalence of
syphilis among those classes, but that we have no means of knowing
how many of the others develop paresis. If anything it shows that
these ‘soldiers by calling,’ have a more stable mental make-up,
since they succumb chiefly to an exogenous toxin.”
Rayneau at the 19th Congress of French Alienists and Neurologists
at Nantes in 1909, discussing the insane of the army from a
medicolegal point of view, states that the most frequent mental
disease amongst officers and soldiers is general paresis. At
least, this disease is the most frequent basis of invaliding,
retirement, or placing in the inactive list. He states that French
and foreign statistics are at one upon this matter, quoting
Christian as finding 32% among the soldiers interned at Charenton;
Gamier at Dijon, 59%; Meilhon at Quimper, 42% and Talon at
Marseilles, 33.8%. Grilli found 31 of 40 officers interned in
Florence, Sienna and Milan victims of general paresis. Stier’s
German statistics indicate about 50%. Rayneau himself found 16 of
20 officers paretic and 17 out of 27 subalterns and _gendarmes_.
The Neurological Society of Paris held a conference December 15,
1916, with the chiefs of the neurological and psychiatric military
centres of France, and discussed a variety of questions concerning
invaliding, incapacity, and compensation in neuroses and psychoses
of war. Dupré dealt especially with the psychoses of war as caused
by trauma, strain, infection, and intoxication. General paresis is
regarded by Dupré as the most important of the dementias found in
the army. The medicolegal point of view is, of course, that
general paresis is necessarily related to an old syphilis, but its
late development leads to misinterpretations as to its probable
cause, both by the family and friends and even by magistrates. The
war acts in the French nomenclature as an _agent revélateur_ or as
an _agent accélérateur_. Although its cause is prior and exterior
to the war, general paresis in a majority of cases is brought out
(_revélé_) by the lack of adaptability of the general paretic to
the novelty and difficulties of his surroundings and duties in
war. Trauma, strain, and alcohol in a certain number of cases
accelerate the progress of a general paresis. The aggravation of
paresis is produced by these same factors, but especially by
violent cerebral trauma. According to Dupré, the Val-de-Grace
statistics show that the number of paretics has not been increased
by the war. Medicolegally, the victim of general paresis, like the
victim of traumatic or infectious chronic mental disorder, may be
assigned an incapacity of from 50 to 100%, and these patients are
invalided under _Réforme No. 1_,—a permanent invaliding.
Lépine of Lyons also discusses the compensation question in
general paresis. Lépine thinks that, although syphilis is
indispensable in paresis, yet the truth is that syphilis plus
something else unknown to us is responsible for general paresis.
This something else is neither a special kind of virus nor is it a
particular kind of prepared soil alone. Trauma, physical,
intellectual, and moral strain, and insomnia are the factors to
which he calls special attention as adjuncts in the production of
general paresis. As to the responsibility of the State for the
production of general paresis, according to Lépine, the maximal
responsibility should be 40% on account of the very considerable
predisposition to paresis created by pre-existent syphilis.
Marie remarked that, although there had been thousands of head
cases at the Salpétrière, there had not been a single case of
general paresis. Dupré agreed with Marie that trauma was not a
frequent etiological factor; strain and alcohol were more
important. The Society agreed that in exceptional cases, where an
encephalic trauma could be regarded as accelerating or aggravating
the disease, the degree of incapacity might be set at from 10 to
30 per cent.
=Syphilis contracted before enlistment, “AGGRAVATED ON SERVICE.”
Canadian case, courtesy of Dr. J. L. Todd, Canadian Board of Pension
Commissioners.=
=Case E.= A laboring man, 44, acquired syphilis at a time unknown. Ten
months after enlistment this man developed symptoms on the firing line.
He was inattentive, irrational, incoherent. The diagnosis was then
“mania.”
There were, however, scars at angle of mouth and on lower lip. Occipital
glands were palpable, fine tremor of hands. The W. R. was +++.
Later the patient became violent, destructive, untidy, disoriented.
Auditory hallucinations are recorded.
He was “boarded” for discharge five months after the first symptoms. The
board agreed that these symptoms would have appeared in civil life. In
view of a difference of opinion as to the part played by stress of
service, his condition was set down as “_aggravated on service_” (not,
it will be noted, _by_ service, see Case D).
1. Under what conditions should pensions be awarded for disability
resulting from venereal diseases? According to a personal
communication from Dr. J. L. Todd, Chairman of the Board of
Pension Commissioners for Canada, pensions are awarded for all
disabilities appearing _during_ service, unless they can be shown
certainly to be due to the men’s own fault and negligence. It
would appear that _during_ service covers both aggravations _by_
and _on_ service. There remains some doubt as to whether
contraction of venereal disease constitutes negligence.
2. What have been conditions in the small inactive American army of
the past? Richards has made a study of statistics at the
Government Hospital for the Insane, Washington.
“The leading features of this mental disease were well exemplified
in our cases the past year. They formed 7.5 per cent of the total
number. They averaged forty years of age, and Ziehen says 80 per
cent of all cases are in the fourth or fifth decade of life. They
averaged ten and a half years’ service, which would indicate that
the military life was their calling. Only one had any serious
hereditary defect. Stigmata of degeneration were infrequent,
averaging only two for each case. 66 per cent had good schooling,
considering their opportunities. Physical signs were frequent in
each case. Only one showed normal light reaction. Ziehen says the
light reaction is retained in only 20 per cent of the cases.
Patellar reflex was absent in one case and normal or exaggerated
in five. The speech defect was slight in four cases. Other
physical signs were present in the usual proportions. Memory
defects existed in all the cases. In four the onset was with
excitement. One began with a character change as the most marked
feature. In only two were the transfer diagnoses correct. One,
beginning as a quiet dementia, was diagnosticated paralysis
agitans, because of a marked tremor. One was excited and euphoric
and was called a manic-depressive psychosis. One with an obscure
onset was diagnosticated as a neurasthenic. The other one was
first observed in this hospital. The physical signs should have
led to a correct diagnosis in each of these cases.”
=Duration of neurosyphilitic process important _re_ compensation.
Canadian case, courtesy of Dr. C. B. Farrar, Psychiatrist, Military
Hospitals Commission.=
=Case F.= A Canadian of 36 enlisted in 1915, served in England, and was
returned to Canada in February, 1917, clearly suffering from some form
of neurosyphilis (W. R. positive in serum and fluid, globulin,
pleocytosis 108).
There is no record of any disability or symptom of nervous or mental
disease at enlistment. The first symptoms were noted by the patient in
May, 1916, six months or more after enlistment. The case was reviewed at
a Canadian Special Hospital, October 11, 1916, by a board of examiners.
This board reported that:
“The condition could only come from syphilitic infection of three years’
standing” (a decision bearing on compensation); but the general
diagnosis remained:
“Cerebrospinal lues, =aggravated by service=.”
The picture which the medical board regarded as of at least three years’
standing was as follows:
History of incontinence, shooting pains, attacks of syncope, general
weakness, facial tremor, exaggerated knee-jerks, pupils react with small
excursion. Speech and writing disorder, perception dull, lapses of
attention, memory defect, defective insight into nature of disorder,
emotional apathy.
1. Was the conclusion “aggravated by service” sound? On humanitarian
grounds the victim is naturally conceded the benefit of the doubt.
But it is questionable how scientifically sound the conclusion
really was.
2. Could the condition come only from syphilitic infection of at
least three years’ standing? Hardly any single symptom in this
case need be of so long a standing; yet the combination of
symptoms seems by very weight of numbers to justify the conclusion
of the medical board.
=Can PARETIC NEUROSYPHILIS (“general paresis”) be lighted up by the
stress of military service without injury or disease? A possible
example from P. Marie, Chatelin and Patrikios of Paris.=
=Case G.= In apparently good health a French soldier repaired to the
colors, in August, 1914, being then 23 years old.
Two years later, August, 1916, symptoms appeared: speech disorder with
stammering, change of character (had become easily excitable), stumbling
gait. He became more and more preoccupied with his own affairs, grew
worse, and was sent to hospital in October, 1916.
He was then foolish and overhappy, especially when interviewed. There
was marked rapid tremor of face and tongue. Speech hesitant, monotonous,
and stammering to the point of unintelligibility. His memory, at first
preserved, became impaired so that half of a test phrase was forgotten.
Simple addition was impossible and fantastic sums would be given instead
of right answers; handwriting tremulous, letters often missed, others
irregular, unequal, and misshapen.
Excitable from onset, the patient now became at times suddenly violent,
striking his wife without provocation. After visit at home, he would
forget to return to hospital. Often he would leave hospital without
permission (of course the more surprising in a disciplined soldier).
No delusions were found.
The serum and fluid W. R. were positive, albumin in fluid,
lymphocytosis.
=Neurological examination.= Unequal pupils, slight right-side mydriasis,
pupils stiff to light, weakly responsive in accommodation, reflexes
lively, fingers tremulous on extension of arms.