Chapter XIV: Introduction (6)

The brain weighed 1450 grams. The vessels at the base showed a slight degree of sclerosis. There was a slight opacity of the frontal, parietal, and temporal pia overlying slightly atrophied convolutions, whose surfaces showed in a few places slight cuppings. The ependyma over the thalami and the floor of the fourth ventricle was finely roughened. The spinal cord showed a typical TABES DORSALIS.

Although we probably cannot regard either Case 42 or Case 43 as a case of paretic neurosyphilis, and although it must remain doubtful whether they are cases of any form whatever of neurosyphilis (in the absence of the modern tests), yet it seems clear that both these cases may very well have been cases of neurosyphilis on account of the existence of a definite tabetic process in each. The symptoms of these cases, like those of Cases 38 to 41, suggest how difficult it must be _to make a clinical diagnosis of general paresis safely without employing available laboratory tests_. Yet how frequently in the past have neurologists brought data concerning various phenomena in long series of so-called paretics in which the error of diagnosis was certainly between 5 and 15% and frequently still greater. The entire question of the symptomatology of paretic and non-paretic neurosyphilis, therefore, needs re-opening and revision.

=CEREBRAL GLIOSIS (probably non-syphilitic) producing the clinical
picture of paretic neurosyphilis (“general paresis”). Autopsy.=

=Case 44.= John Hughes was a hostler, and later assistant with a wholesale drug company, with which he remained for 32 years. He had been moderately but constantly alcoholic all his adult life up to 50 years of age, and at 45 had had an attack of so-called nervous prostration, in which his head had troubled him and he had been seclusive. At 49, he had a serious attack of otitis media, associated with delirium, swelling of the feet, and what was called rheumatism. After this attack of otitis media, Hughes appears to have been not altogether right.

At 53, after a quarrel with his employer, Hughes quit work, began to trade a little in hens and pigs, became forgetful, especially of recent events, and did “a variety of peculiar things.” He was a married man but he had no children. There had been miscarriages but of unknown origin; venereal disease was denied. At 55, a week before admission, Hughes had a spell of unconsciousness for several hours, after which his speech was thick, and restlessness, insomnia, and a wandering tendency set in. Visual hallucinations, fabulation, tremors, “excited-looking” eyes, are described. He would sweep things from the dining-room table, pulled a hot stove into the middle of the floor, attempted to sweep paint off the floor, and cut up a carpet with a knife.

The patient on commitment November 5, 1904, was well developed and nourished. The mucous membranes were rather pale. Bruises and excoriations of limbs. Harsh breathing at the base of each lung. Enlargement of heart; sounds irregular. Accentuation of aortic second sound; tension fair, rate 80. Slight brachial arteriosclerosis. Abdomen slightly distended. The urine contained a faint trace of albumin and many hyaline casts.

Moderate tremor of extended hands. Slight tongue tremor. Romberg’s sign absent (slight swaying). Considerable ataxia of extremities (inability to stand with foot on opposite knee). Vision poor. Hearing could not be tested accurately. Prompt pupil reactions with direct light. Slight consensual reaction in left pupil, absent in right. Deep reflexes equal and lively.

Quiet and orderly at first. Later restless and noisy. Questions were answered at times relevantly, more often irrelevantly. Patient irritable, intractable. Required repeated urging to take nourishment. Consciousness clouded. Orientation imperfect. Attendants are possibly “officers.” Date September, 1995. Slight errors in repeating alphabet. Mistakes in Lord’s Prayer with rhyming tendency. Simple arithmetical tests answered automatically with many mistakes. More complex combinations incorrect. Handwriting tremulous (noted as “typical of general paresis”). Auditory hallucinations (answering invisible persons), “All right, I’m coming.” Amnesia and confabulation. Q. “Have you had breakfast?” A. “No,” (later) “Yes, I had a very light breakfast.” Q. “What did you have?” A. “Anything that came along. A few green peas and beans that were left, bread and butter and pie. I had a good breakfast. Guess feed is very high.” Q. “Give names of your sisters and brothers.” A. “There are three or four I never see. I will have to think them up.” (Later)—“Lillie, Abbie, Julia, George.” On repetition of question, “Elizabeth, Julia, Annie and Lizzie.”

Delusions somewhat doubtful. At no time euphoria.

The patient remained only nine days in the hospital, developing diarrhea a week after admission.

=Post Mortem Findings.= The =cause of death= was bilateral bronchopneumonia of hypostatic distribution, accompanied by bronchitis and acute splenitis. The intestinal tract was normal (despite the diarrhea). No cultures. The heart showed acute myocarditis.

The vessels in general showed no sclerosis, except that the aorta showed a few patches with calcification near bifurcation. There was a moderate degree of mitral sclerosis. The kidneys showed a moderate degree of chronic interstitial nephritis. The heart weighed 530 grams and there was moderate dilatation of all the valves.

There were some evidences of chronic disease outside the nervous system, namely, an obliterative pleuritis on the right side, chronic perisplenitis, and chronic external adhesive pachymeningitis.

The =nervous system= showed a pia mater thin and transparent, with a moderate congestion of larger and smaller vessels. No noteworthy change of the brain substance or of the ventricles was found, except that the cerebral substance was of unusual firmness (autopsy twelve hours after death).

It is clear that the brain was not wholly normal, exhibiting a general induration due in part to subpial gliosis and in part doubtless to perivascular gliosis. =Microscopically= the tissues showed features of great interest, especially multiple focal neuroglia cell proliferations of a perivascular distribution, considerable subpial fibrillar gliosis of an unusually focal type, and a rather general subpial cellular =gliosis=. Histologically, it seemed that this chronic progressive process had started, not so much in relation with dying nerve cells, as in relation with blood vessels. The =perivascular= deposits of neuroglia cells were confined almost exclusively to the infragranular cortex layers. It seems plain that the diagnosis of general paresis was not justified. It is probable that the diagnosis of neurosyphilis is not justified. The explanation may be that now and then cases of cerebral sclerosis may clinically imitate the neurosyphilitic process. It must be borne in mind that the diagnosis in this case was made, like the other cases at head of Part III, without the advantage of modern systematic methods. Clinically speaking, of course, there was no definite Argyll-Robertson pupil, although the consensual reaction, slight on the left side, was absent in the right pupil. The general picture appeared to be one of the so-called demented form of paretic neurosyphilis.

=Differential diagnosis between NEUROSYPHILIS and NEURASTHENIA.=

=Case 45.= Albert Robinson, a man of 28 years, was shipwrecked on one of the Great Lakes. The ship was on the rocks for eight days, and Robinson was under a great strain. Ever after the wreck, Robinson had felt severe pain in the head, neck, and back, and a feeling of great weakness whenever he exerted himself physically or mentally, and seven months after the wreck, he had several attacks of fainting.

For a number of weeks he had worried a good deal about his inability to make money, especially as money was badly needed on account of his wife’s approaching confinement. A few days before entrance, Robinson had become very forgetful, and was unable to recall, the night before entrance, where he had been during the day. On the whole, however, on mental examination no actual evidence of memory defect could be shown to exist.

=Physically=, Robinson was entirely negative, except for some hard glands in each groin. =Mentally=, there was little to show except depression, worry over his financial condition, and his inability to work. The serum W. R. proved negative.

=Diagnosis=: On the whole, the diagnosis of psychoneurosis (see case Harrison (9)) due to the shock at the time of the shipwreck seemed to be proper. To be sure, the patient gave a history of a chancre at 25, treated for two years, after which he was declared cured.

However, following up the clue of admitted syphilis, rigorous questioning elicited the fact that a few months before there had been diplopia, lasting part of a day.

=Lumbar puncture= seemed desirable. The fluid was clear but contained 125 cells per cmm. with appropriately increased amounts of albumin and globulin. The spinal fluid W. R. was positive. The diagnosis of CEREBROSPINAL SYPHILIS seemed established.

The lesson of this case appears to be that perhaps we should never exclude syphilis until we have made an examination of the cerebrospinal fluid. The W. R. of the blood in meningovascular (non-paretic syphilis) is negative in many cases (the figure is sometimes set as high as 40%).

=Treatment=: After a half dozen injections of salvarsan, all symptoms disappeared and Robinson went back to work, claiming to be in a better condition than for some time past.

1. How shall we explain such a symptom as the transient diplopia?
This diplopia is probably an example of a neurorecidive, but it
will be observed that it occurred without salvarsan therapy. See
discussion above under the case of Bennett (34), where the general
result of the neurorecidive inquiry launched by Ehrlich early in
the history of salvarsan therapy showed that precisely similar
phenomena had always occurred in neurosyphilis, whether under
treatment or not. The anatomical and histopathological explanation
of such phenomena is, of course, doubtful, but a review of the
findings in the case of Alice Morton (1) will show how many
apparently serious symptoms in neurosyphilitics are actually
irritative or at least due to lesions which are entirely
recoverable. We may suppose, first, a local proliferation of
spirochetes; second, a local over-formation of toxic substances,
directly or indirectly the product of spirochetosis; thirdly, a
local exudation; fourthly, a local proliferation; fifthly, a
combination of these phenomena, any or all of which may be
regarded as but transient. We have sometimes found at autopsy very
little exudate except in small areas; sometimes not more than a
few mm. or cm. in superficial extent. Note, for example, the small
areas of lymphocytosis demonstrable in but two foci in the case of
Alice Morton, the paradigm placed at the beginning of this book.

=NEUROSYPHILIS(?) in the SECONDARY STAGE of syphilis. HYSTERICAL
symptoms. Diagnosis?=

=Case 46.= Alice Caperson was a colored girl of 18 years. She had acquired syphilis five months before admission to the hospital, and the secondary symptoms of this syphilis had just disappeared before admission.

Very shortly after acquiring syphilis, the young negress began to act peculiarly. She describes herself as having a sort of nightmare, both when asleep and also when awake. For instance, she saw her dead grandmother. It appeared at first like a seraph; then it came nearer to her and seemed to fill out; and then was dressed precisely as her grandmother had been. This seraph appeared as though trying to tell her something, but she could not make out what the something was. The vision had appeared on two or three occasions.

Our examination detected little beyond instability and irritability of mood with some depression. The patient readily fell to weeping. She soon made friends in the wards, however, and got on well. =Physical examination= was entirely negative but the W. R. of the blood serum was positive. The W. R. of the spinal fluid was negative, as was the gold sol reaction; there was an excess of albumin and a positive globulin test; there were seven cells per cmm.

The psychiatric diagnosis of a case like that of Alice Caperson would waver between hysteria and dementia praecox. However, as for dementia praecox there are hardly any typical symptoms. There is insight into the hallucinations, which are hypnagogic. There are, however, no hysterical stigmata.

The spinal fluid reaction is typical of the secondary stage of syphilis. It is commonly said that in every case of syphilis the nervous system is involved at some period, if only to the degree shown in the present case. However, such involvement tends to disappear both with and without antisyphilitic treatment, just as do the secondary skin symptoms. So far as syphilis is concerned, the prognosis under radical treatment is as good as usual. We are inclined to regard the case as one of the HYSTERICAL or PSYCHOPATHIC group and inasmuch as cases occurring in the developmental stage of a patient’s life are of fairly good general prognosis, we are inclined to regard the prognosis in this particular case as good under proper therapy and hygiene.

1. What is the relation of neuroses to syphilis? Neurasthenia,
chorea, hysteria, and epilepsy are often grouped (for example, by
Nonne) as neuroses bearing at times important relations to
neurosyphilis. (For the relations of neurasthenia, chorea, and
epilepsy, see cases of Greeley Harrison (9), Margaret Green (72),
and David Borofski (49), respectively.) As for the hysteria shown
in Caperson, Charcot enumerated syphilis among _agents
provocateurs_ of hysteria along with alcohol, lead, arsenic, and
the like. Fournier has also considered the problem. It is clearly
necessary to show that before infection there were no hysterical
symptoms, and that the hysteria developed during the operation of
the syphilitic process, and it is probably necessary to show that
the symptoms will clear up under antisyphilitic treatment, if we
are to concede the existence of a syphilitic hysteria.

2. What are the evidences of neurosyphilis in the secondary and
primary stages of syphilis? As above stated, the findings in
Caperson are typical enough. Wile and Stokes at first stated that
60 to 70% of the secondary syphilitics show changes in the spinal
fluid; in a further article they maintain that probably every case
shows such changes and that clinical symptoms of neurosyphilis of
the secondary period can probably be determined. They claim that
it is probable also that the same holds for primary syphilis
itself. The importance of these claims lodges partly in the
relation of these early signs of neurosyphilis to the whole
question of latency and to the question of _paresis sine paresi_.
For a discussion of _paresis sine paresi_ see cases Lawlor (25),
Vogel (52).

=Differential diagnosis between NEUROSYPHILIS and MANIC-DEPRESSIVE
PSYCHOSIS.[9]=

=Case 47.= As in other instances (compare Martha Bartlett (21) and Annie Monks (85)) so also in the case of Ethel Hunter, a woman 61 years of age, there was no initial suspicion of neurosyphilis. Mrs. Hunter was brought to the hospital stuporous as a result of an overdose of paraldehyd. The paraldehyd had been administered by a physician to combat insomnia and agitation. As soon as Mrs. H. had recovered from the drug stupor, this agitation appeared once more, and it was clear that she was suffering from marked depression. There was tremendous worry over the sickness of a woman with whom the patient lived. The patient was very self-accusatory, blaming herself for many things that had happened in the household. Besides her agitation, depression, self-accusations, and insomnia, the patient showed a good deal of the symptom frequently termed “retardation”—a kind of lagging of all mental processes found, according to Kraepelin, in manic-depressive psychosis.

Accordingly, the diagnosis of manic-depressive psychosis might well have been rendered. The fact that the psychosis so far as known began in the involution period was not against the diagnosis since the so-called involution-melancholia of this period is at least in a certain fraction of cases nothing more or less than a form of manic-depressive psychosis. However, the =physical examination= made the diagnosis of manic-depressive psychosis a little doubtful. There was a superficial thickening of the arteries (blood pressure: systolic, 170; diastolic, 104), which thickening would not in itself be against the diagnosis of manic-depressive psychosis. (In point of fact, arteriosclerosis is rather common late in this disease and previous attacks could not be excluded on the basis of available history.) The contracted pupils were irregular and both reacted sluggishly to light, although better to accommodation; the right pupil was larger than the left. The arm reflexes were pretty active. The left knee-jerk could not be obtained, nor was the right knee-jerk more than very sluggish. The Achilles reflexes could not be obtained. Although there was not a positive Romberg sign, there was a considerable swaying in Romberg position. There was no speech defect. The other reflexes showed nothing abnormal. On the whole, we had to conclude that, although Mrs. Hunter might be an instance of manic-depressive psychosis, still there was much of neurological interest in the case.

This conclusion was emphasized when the W. R. of the blood serum was found to be positive. The spinal fluid W. R. was also positive, and the gold sol index was of the “paretic” type. There were 74 cells to the cmm. Globulin stood at ++++, and albumin at ++++.

This case, therefore, again illustrates, as well the protean nature of GENERAL PARESIS (the diagnosis rendered), as the doubtful value of making a psychiatric diagnosis without due consideration of the physical examination and laboratory findings. How easy might it have been, at least some years ago, to consider that this patient of 61 years had suffered a slight shock at some previous time (left knee-jerk absent), but was as a matter of fact a case of manic-depressive psychosis with a vascular complication!

Note: We must again duly insist that the merely sluggish light reactions of the pupils in such a case as this do not especially point to general paresis. The literature seems to establish that sluggishness of light reaction precedes the classical Argyll-Robertson pupil. Yet it does not do to say that, if the Argyll-Robertson pupil pretty conclusively points to neurosyphilis (for exceptions see cases Falvey (55), Murphy (60)), then a sluggish pupillary reaction to light looks in the same direction. Sluggishness may precede stiffness in many, or perhaps all, cases, but sluggishness of pupils is a frequent phenomenon outside the syphilitic group of cases.

1. What part is played by emotional shock and psychic causes in the
starting up of general paresis? The answer to this question cannot
be definite. That a paretic process can be started up after trauma
is admitted on all sides; but we here suppose actual physical or
chemical brain disturbance permitting increased spirochetosis or
inflammatory reaction. In the case of psychic shock, or what might
be called _psychogenic general paresis_, our best resort will be
to the indirect effects of hormone action, or of vasomotor and
other autonomic disturbances produced directly or indirectly by
emotion. We are clearly here dealing with material too speculative
to be of practical service at this time.

2. Was the depressive drug therapy in the case of Hunter
justifiable? The paraldehyd had been administered by a physician
apparently on purely symptomatic grounds to combat the insomnia
and agitation of this woman of 61 years. With all due
acknowledgment of the difficulties of private practice, we must
insist that when ordinary measures in the relief of insomnia and
agitation are insufficient to curb these conditions, then a
positive danger ensues with the larger doses. As a rule, with
these larger doses and with the withdrawal of sensory stimulation,
the patients relapse into a stupor of grave moment. We need only
recall the situation in delirium tremens where adequately
depressive drugs often tend to kill the patient.

=Case for diagnosis. Errors in the diagnosis of NEUROSYPHILIS are
possible even when abundant clinical and laboratory data are
available.=

=Case 48.= The first error chosen for demonstration is that in the case of the machinist, Milton Safsky.

Safsky, about 8 months before his entrance to the hospital in the 42d year of his life, had begun to lose strength, to grow thin and pale, and to suffer from an extreme and continuous thirst. He was said to have drunk as much as 6½ gal. in a day, and passed appropriately large quantities of urine. After a time, his management at a general hospital became difficult, as Safsky became confused, cried “hysterically,” and was at times very noisy. He sustained a marked memory loss, seemed to show visual hallucinations, and complained of headache, both frontal and occipital, and of pain about the eyes. Sometimes the patient was very euphoric and expressed what seemed to be delusions of grandeur, saying he was wealthy and owned many machine shops.

Some symptoms, e.g., polydipsia and polyuria amounting to a diabetes insipidus, associated with headache and arrested attention, suggested possibly a new growth in the pituitary region. The mental symptoms might naturally be supposed to be due to some infiltration or pressure effect of intracranial growth. After admission to the Psychopathic Hospital, the patient was found difficult to arouse, although he could eventually be aroused. His orientation proved to be as poor as his memory. From time to time, the patient became a bit more intelligent and able to execute requests.

The =physical examination= was in general almost entirely negative. =Neurologically=, the pupils were markedly contracted and reacted slowly to light, though they were otherwise normal. The deep reflexes were all somewhat lively, though equal. The umbilical and cremasteric reflexes in particular were present. Systematic examination revealed no other reflex disorder, nor any disturbance of sensation. There was a coarse tremor of the extended hands. There were no phenomena of importance in the visual fields.

As against the diagnosis of growth, pituitary or extrapituitary (diabetes insipidus and headache), a hypothesis of neurosyphilis had to be considered. Not only were the contracted, slowly-reacting pupils and the active deep reflexes suggestive, but the euphoria with grandiose ideas looked entirely consistent. As for the polyuria, one had to think of the so-called syphilitic polyuria of the textbooks, which is regarded as a more or less characteristic result of syphilitic involvement of the _basis cerebri_. Moreover, the W. R. in the spinal fluid proved to be slightly positive; 146 cells per cmm. were found therein; there was a large quantity of globulin, and a very marked increase in albumin. These observations seemed to be exceedingly suggestive of a cerebral syphilis.

However, as the case progressed, the diagnostic situation changed. The W. R. upon a second puncture fluid proved negative. After some weeks, characteristic symptoms of intracranial pressure developed; the diagnosis of BRAIN TUMOR had to be taken as established, and there is no doubt of its correctness.

1. What is the explanation of the weakly positive W. R. in Safsky’s
spinal fluid? An explanation is not easy to find. Possibly we may
regard the reaction as an example of error in technique. It is
even possible that it may have been produced by exudative products
in the spinal fluid.

2. What precautions may be taken against an error in diagnosis such
as was first made through the positive spinal fluid Wassermann in
the case of Safsky? First, repetition of the W. R.; secondly, it
is very unusual to find a weakly positive W. R. in a case with
such marked excess of albumin and such very marked increase of
globulin as was shown by this case.

3. How can we explain the inflammatory products in the puncture
fluid? Superficial brain tumors are frequently associated with a
so-called _meningitis sympathica_. The products of such meningitis
are exhibited: _viz._, globulin, albumin, and pleocytosis, exactly
as shown in Safsky.

=Can PARETIC NEUROSYPHILIS (“general paresis”) appear clinically
EARLY (e.g., two years) after the initial syphilitic infection?=

=Case 49.= David Borofski, a street car conductor, 27 years of age, suddenly had a convulsion while at work in his car. For four months Borofski continued to have rather numerous convulsions, was finally compelled to discontinue work, and resorted to the Psychopathic Hospital. It appears from his own story that, about two years before, he had had a chancre, for which he had been treated at a general hospital syphilis clinic, and of which he was told he was cured. With a progressive loss of memory and with convulsions, Borofski became much concerned about himself, and was finally persuaded by his fellow-workers to come to the Psychopathic Hospital.

The convulsions were described as follows: The patient gives a short cry, has convulsive movements for about ten minutes, remains unconscious for perhaps half an hour, and wakes with headache, dizziness, and a feverish appearance. Sometimes the attacks were more severe, with frothing at the mouth, biting of lips, and loss of sphincter control. There were also slight attacks, occurring almost every day, without loss of consciousness; these latter attacks consisted of dizziness, inability to speak for a few seconds, and some arm twitching.

=Physically=, Borofski was well developed and nourished, with a blood pressure of 160. The only abnormal phenomena =neurologically= were absent knee-jerks and ankle-jerks, sluggish pupillary reactions, and slight tremor of the hands.

=Mentally=, despite suggestive complaint of amnesia, the memory was found to be fairly good but knowledge of current events and school knowledge was poor. The simplest problems in arithmetic Borofski gave up.

The first diagnosis in such a case would naturally be epilepsy. However, when an epileptic or epileptiform attack occurs for the first time in adult life, the chances are probably against an idiopathic epilepsy. (This is not a universal rule but will serve.) Borofski himself, moreover, gave a history of syphilis. And the very nature of the attacks, with arm twitching and without loss of consciousness, would not readily fit into the frame of the idiopathic group. The absence of certain reflexes and the sluggish pupils are naturally also suggestive of syphilis, although not convincing.

The W. R. of the serum proved positive, as did that of the spinal fluid. The gold sol reaction was characteristically “paretic”; there was an excess of albumin and a positive globulin, and there were 15 cells per cmm. There could be little or no doubt of the diagnosis of some form of neurosyphilis. The laboratory picture was consistent either with general paresis or with cerebrospinal syphilis. So far as we are aware in the present stage of knowledge, the two conditions can hardly be differentiated unless we choose to rely on therapeutics. However, it is exceedingly rare for general paresis to occur only two years after the original infection. If we can trust this statistical fact, we shall perhaps be wiser to term the case of Borofski one of DIFFUSE CEREBROSPINAL SYPHILIS, and not one of paresis.

=Treatment=: Borofski was put on antisyphilitic treatment consisting of 0.6 gram of salvarsan twice a week and potassium iodid, together with intramuscular injections of mercury salicylate. The convulsions then ceased. After four months Borofski returned to work, and he has remained at work for a year. He has never regained his former health.

Fifteen months after beginning of treatment the laboratory tests were again made (there had been more than 60 injections of salvarsan), and the cell count and gold sol reactions were found to be negative. Globulin and albumin were also in smaller amounts than in the original examination. However, the W. R. of the serum and the spinal fluid remained positive.

Head and Fearnsides state that cases of cerebrospinal syphilis should return negative spinal fluid tests after six months of treatment. Upon this criterion of Head and Fearnsides, Borofski would not be a case of cerebrospinal syphilis; but it is probably impossible to separate various forms of neurosyphilis into categories on any such grounds.

1. Shall case David Borofski be regarded as one of paretic
neurosyphilis (“general paresis”)? He has returned to work and has
remained at work, though without regaining his former health. In
any event, however, he does not offer the typical picture of
inevitable decline and death presented by the typical case of
Pietro Martiro (15) presented in our discussion of systematic
diagnosis. However, we could not upon laboratory grounds, or even
upon the ground of clinical observation, distinguish Borofski from
Martiro; Borofski has greatly improved; Martiro is dead. Borofski
developed his obvious neurosyphilis only two years after the
original infection. The conservative syphilographer might,
accordingly, reply that David Borofski is not a typical case of
paretic neurosyphilis (“general paresis”) either in the length of
the incubation period for his neurosyphilitic symptoms, or in his
outcome.

2. What is the cause of such convulsions as those developed by David
Borofski? Evidence from clear cases of general paresis with
convulsions leads to the hypothesis that such convulsions as those
developed by Borofski are not necessarily based upon frank
destructive lesions such as would be produced by the plugging of
terminal arteries. They may well be produced through the
activities of minor lesions, only demonstrable by microscopic
methods, either through properly disposed cell losses or by the
pressure of exudate, or even by endotoxins or other substances
derived from the bodies of dead or living spirochetes.

3. Aside from the well-known syphilitic epilepsy due to meningitis,
is there a non-meningitic epilepsy (such a disease as Fournier
formerly described under the term parasyphilitic epilepsy)? We
dismiss from discussion the so-called symptomatic epilepsies which
are the result of a gross organic disease of the brain substance
or its membranes, and which do not differ so far as we are aware
from organic epilepsy produced by other gross lesions of an
identical size and structure. These symptomatic epilepsies may be
partial, or even may present the appearance of generalized
epilepsy. We may also leave out of account those epileptic
pictures which are produced in general paresis itself, and which
may be viewed as nothing but partial phenomena of general paresis.
The kind of so-called “parasyphilitic” epilepsy that Fournier
described is a kind of epilepsy that cannot be distinguished from
genuine epilepsy, in which the sole disease-phenomenon throughout
a long period of time consists of epileptic convulsions. It
appears that these “parasyphilitic” imitations of genuine epilepsy
occur in individuals with a very long post-infective “incubation
period,” but that there are some cases in which the epilepsy
appears, on the contrary, in the very earliest stages of syphilis.
The attacks are a little less common than those of idiopathic
epilepsy; they have the same apparently causeless beginning; are
associated with complete amnesia; and are followed by
characteristic dazed states. The patient’s intelligence, however,
suffers little. Now and then a case reacts well to antisyphilitic
treatment energetically pushed. (Spontaneous long remissions in
non-syphilitic epilepsy must be remembered.) Petit mal attacks
occur sometimes between the more severe attacks. In short, it
would appear that there is a group of syphilitic epilepsies in
which the brain shows no gross structural lesions, which
accordingly do not exhibit any Jacksonian appearances, and which
last a comparatively long time without changing their character,
and often without being especially altered for the better by any
form of antisyphilitic treatment. This condition is sometimes
known as a post-syphilitic epileptic neurosis. Nonne had been able
to collect up to 1902 some 12 cases from his own service.

4. Would it be proper to call Borofski a case of taboparesis? Absent
knee-jerks in a victim of paretic neurosyphilis should not be used
to suggest a diagnosis of taboparesis. This question of
terminology has been discussed above, under Sullivan (16).

5. What is the mechanism by which the amnesia of a case like
Borofski is produced? The answer runs in the same terms as the
answer to the questions concerning the cause of convulsions. The
amnesia in general paresis has surprising functionality. A study
of autopsied cases of general paresis has shown that amnesia is
practically as common in cases without marked destruction of brain
tissue as in cases with atrophy of classical extent and depth. The
clinical recovery in this case was practically complete in respect
to memory. We must regard the amnesia as not due to the
destruction of storage cells bearing the so-called neurograms
(Morton Prince).

6. What is the explanation of the persistently positive W. R.’s of
the serum and spinal fluid associated with diminished globulin and
albumin tests, a negative gold sol reaction, and normal cell
count? See discussion under Case Martha Bartlett (21).

7. How atypical is the early development of paretic symptoms in
David Borofski? C. B. Craig has collected, in 100 cases of brain
syphilis (a list including both paretic and non-paretic cases),
some data on this point. The shortest period reported by Craig was
in a case in which the neurosyphilitic symptoms appeared one month
after infection. Craig found three cases where symptoms appeared
in six months, and six cases within a year. The longest
post-infective period of Craig’s list was thirty years. Our case
of Chatterton (73) developed symptoms 33 years after infection and
Washington (66), forty years after infection. Nonne casts some
doubt on statements to the effect that tabetic symptoms may occur
three to four months after infection. It seems to be admitted that
pupillary anomalies and reflex changes may occur in the early
secondaries and may recover under antisyphilitic treatment.
Nonne’s case of longest post-infective interval, like that of
Craig, was one of 30 years.

Myerson has reported a 20–year old patient who acquired chancre
April 1, 1911 (spirochetes demonstrated); salvarsan was
administered April 20th. There were no secondary symptoms, but in
May, headache, visual disturbance, vertigo, and other symptoms
developed (neurorecidive). Upon June 20th, that is, 11 weeks after
development of the chancre, aphasia and astasia developed, with
numbness of the left side. At this time, the pupils were slightly
irregular and unequal but reacted normally. The signs in the fluid
were positive. Upon this question see our cases of Bright (121)
and Bennett (34).

=Hemitremor following hemiplegia in PARETIC NEUROSYPHILIS (“general
paresis”). Autopsy.=

=Case 50.= Achilles Akropovlos, 39 years, had symptoms six months before commitment to Danvers Hospital. There were attacks of confusion, difficulty in walking, and speech defect, resulting in an entire incapacity to work and eventual commitment. Rather unusual and striking was a very marked tremor, apparently limited to the right side of the body. =Physically=, Akropovlos was normal, but =neurologically= he showed, in addition to the marked right-sided tremor, a marked speech defect, and a degree of ataxia. The tendon reflexes were very active, but there were no abnormal reflexes, and the pupils reacted normally. According to the history, the difficulty had followed a slight attack of apoplexy. =Mentally=, there was a marked confusion. The blood serum and the spinal fluid were both positive to the W. R.; globulin was present, and albumin was increased; there were 43 cells per cmm. There was hardly any diagnosis to make except general paresis.

Death followed 18 months later, or two years after onset of symptoms. Increasing weakness, emaciation, and dementia preceded death. Autopsy confirmed the diagnosis of PARETIC NEUROSYPHILIS.

1. What is the usual cause of death in general paresis? Intercurrent
disease very frequently occurs in general paresis, and such
intercurrent disease is then given as the cause of death. As a
matter of fact, however, one feels that in many of these cases the
intercurrent pneumonia or infection—frequently of the
bladder,—bedsores, sepsis, and the like, are merely accidental
incidents in a condition that is leading to death, and which has
caused a lowered resistance to infection. In certain instances
where nursing is exceptionally good and where no such infection
occurs, the patient continues to grow weaker and weaker, paralyses
of all the muscles follow and finally paralysis of deglutition or
respiration may lead to death. The emaciation and paralyses may be
of such a grade that the patient is entirely devoid of fat and
unable to move at all. Not infrequently vascular crises occur, and
one of these may be responsible for death.

2. What was the cause of the hemitremor? The hemitremor suggested an
irritative or destructive lesion in the motor path. Delving into
the history it was learned that the patient had had a shock
followed by a right hemiparesis. This had cleared up leaving the
tremor as a residuum. The autopsy disclosed a reddish-brown
pigmentation and fibrous thickening of the pia over the left motor
area, confirming the idea of a previous hemorrhage. As a rule the
shock phenomena occurring in paresis clear up more completely and
no gross lesion is visible post mortem. However, cerebral
hemorrhage must be expected in any person suffering from syphilis,
and is no rarity in paretic neurosyphilis.

=PARETIC NEUROSYPHILIS (“general paresis”) with NORMALLY REACTING
PUPILS. History of trauma. Autopsy.=

=Case 51.= Daniel Wheelwright, a barber of English extraction, 57 years of age, had had a sunstroke at 15. At 42, there had been pneumonia, after which an attack of rheumatism was said to have kept the patient from work for a year. There was trauma of head (falling wrench) at 44. This blow on the head was the assigned cause of the mental disease, symptoms of which, however, did not develop until about the first of September, 1905, about three months before entrance, January 9, 1906, and about six months before death, March 20, 1906.

It seems that the patient had begun to change in manner; he had become despondent and apathetic, silent, and somnolent. Two weeks later, he stopped working, began to read the papers once more, and became somewhat more cheerful.

About Thanksgiving, Wheelwright got up at midnight, and remained up, lighting all the fires and talking continuously. During the next two weeks, he talked much to himself, laughing out at times. About two weeks before Christmas he went out and started to make a sidewalk of old boards, working in his shirtsleeves, without a hat. He would work until midnight making screens for windows. During the day, he would go out and give money to passing children; would offer to pay the grocer twice as much as articles were worth.

On the day before Christmas, he put out all the fires and lights in the house, sent all the family to bed, and opened all the doors. Christmas morning, he rose early and got the washtubs ready. He helped his compliant wife to do the washing, then put out all the fires and opened the windows. After Christmas, he began to tell how rich he was going to be through starting a garden and by making butter. He bought six or seven quarts of milk daily, and procured carrots and oranges, grinding them up to color the milk. January 9th he was committed to Danvers Hospital.

=Physically=, there were few symptoms. =Neurologically=, there was a tremor of tongue, fingers, and face. The knee-jerks were lively. The pupils reacted normally; the patient was restless, pacing up and down. There was a speech defect demonstrable with test phrases. Orientation was imperfect for time and for place. Handwriting was poor, memory impairment was marked, but the patient was given to fabrication as to past events. A characteristic sample of statements:

“Do you know that this is an insane hospital?” “Yes; there are two or three men here out of their heads. I could cure them with my hands but they won’t let me. I could get all the sick men on their feet just by rubbing them. I can do anything with my hands. I can build a house by just sitting down and thinking about it. I can whip all the men in this place. I have better sense now than I ever had in my life.”

Again, “How long have you been here?” “Over three months; they have put me in heaven three times since I have been here. They killed me, crushed my heart, and turned my blood to water. I am all right now. I let the sun shine on my heart and it brought it together. I can whip every man in here as fast as they come up.”

Again, “I will make a million dollars on my garden when I get it. I can make a million dollars on half an acre. I can do anything. I can move this house by just thinking of it.”

During a special examination, the patient told how he had fastened wings on his hands and feet, and how he had gone to heaven; he told how he had soared high above the earth, and how differently the stars look when up near heaven than they do from the earth. He spoke of seeing angels and of the beauties of heaven.

The diagnosis of PARETIC NEUROSYPHILIS was confirmed at autopsy.

1. What is the significance of the normally reacting pupils? While
it is usual to find pupillary anomalies in neurosyphilis, these
changes are not an essential part and it is not rare to find
normal pupils in all forms of neurosyphilis. It is less frequent
to find a normal pupil in tabetic than in diffuse or paretic
neurosyphilis. In paretic neurosyphilis it is the rule to find
pupillary changes during some stage of the disease, but not
necessarily early. At times the pupillary sign may be one of the
earliest signs of neurosyphilis—again it may occur only as a late
symptom, if at all. One of the most important of the pupillary
signs is irregularity of contour. While this does not always mean
neurosyphilis it is highly suggestive and certainly indicates
careful examination even though the W. R. in the blood be
negative.

2. What was the relation of trauma to the development of the
neurosyphilitic symptoms? It is, of course, the rule in all forms
of mental disease to have some factor offered by the patient or
relatives as the cause of the psychosis. Often these assigned
causes are minor events thought of only after the later appearance
of symptoms. In this case it was not thought that the trauma had
any causal effect. For a discussion of trauma and neurosyphilis
see cases Joseph O’Hearn (90), Levi Sussman (91), and Joseph
Larkin (92).

=NEUROSYPHILIS, probably PARETIC, with symptoms highly suggestive of
MANIC-DEPRESSIVE PSYCHOSIS.=

=Case 52.= Bessie Vogel[10] was admitted to the Psychopathic hospital New Year’s day, 1915, in a very much excited condition. The family history is very meagre, and all that is of significance is that mother has always been very “nervous.” The records in part:

=Past History.= Very healthy as a child, and except for occasional throat trouble and headache had no physical ailments until eight years ago, when she had an operation for appendicitis, and two and one-half years ago was operated upon for hernia and adhesions. Following this she began to show a lack of energy, neglected her housework, was much depressed, wept frequently, complained constantly of pain in various places, and was ill-tempered. In about five months she improved, and then after a couple of weeks at the shore seemed entirely well.

=Present Illness.= In November, 1914, that is, about seventeen months after the recovery from the previous depression, she again began to show practically the same symptoms. She was depressed, could not sleep, and would get up in the night and sew; was self-centered and hypersensitive, then became restless and nervous; wanted to go shopping and out for dinner; went to New York and then to New Bedford. Symptoms became more marked; she became very ill-tempered, threatened her husband when angry over trifles, threatened suicide, then began to get active and spent money extravagantly. At the end of two months, that is, Jan. 1, 1915, she was admitted to the hospital.

=Physical Examination.= A small, thin woman, appearing to be about 45 years old (actual age 37). Aside from the absence of teeth and the operation scars, the general examination is negative. =Neuromuscular system=: The pupils are round, regular, equal, and react to light and accommodation, but do not hold very well. Extraocular movements well performed, no palsies of facial muscles, tongue protruded medially without tremor. Uvula is raised symmetrically. Biceps and triceps and supinator reflexes are present and brisk. Patellar and Achilles reflexes are equal on the two sides and brisk. Abdominal skin reflexes not obtained. Plantar reflex active and flexor in type. No Babinski, Gordon, or Oppenheim. No tremors.

Wassermann reaction serum positive. Examination of spinal fluid: clear, globulin ++++, albumin ++++; cells, 130 per cmm.; small lymphocytes, 79.9%; large lymphocytes, 14.1%; polymorphonuclear leucocytes, 4.6%; plasma cells, 0.7%; endothelial cell, 0.7%. W. R. positive. Gold sol reaction, 55555522 +-.

=Mental Examination.= On admission patient showed great psychomotor activity, was very playful, marked flight of ideas, was expansive, very emotional, very erotic. She slept very little, appetite was poor, and she lost weight rapidly. Orientation and memory intact. No hallucinations elicited. In about three weeks improvement began, and at the end of eight weeks she appeared practically recovered. On April 9, 1915,—that is, 13 weeks after admission,—she was allowed home on visit. On leaving, she appeared normal in every way. There was no evidence of psychotic symptoms, she had good insight, and physically there was absolutely nothing of a neurological nature that was abnormal.

This case, with the history of a previous depression and its clinical picture during the acute stage, and its recovery, is certainly in every respect typical of manic-depressive insanity, and only the positive result of the six tests causes us to put it in the group of GENERAL PARESIS. Only the further course will shed any light as to the correct significance of these findings, and even then we shall not be too sure that we had not been dealing with a manic-depressive psychosis in a latent neurosyphilitic. We would strongly emphasize the point that at the present time this patient presents no mental or physical signs of cerebrospinal syphilis or general paresis; but the six tests are still positive. This case differs from the ordinary general paresis remission in that there is not a single physical sign of paresis present.

There are many transitional cases between this case which shows no symptoms or signs of neurosyphilis except the laboratory tests, and the typical case of general paresis. Thus we have cases with slight character change and no physical signs except rare “seizures.” On the other hand, in many cases the presence of abnormal neurological phenomena without definite mental signs is first noted. Certain remitted cases show only some slight pupillary or reflex abnormality. We believe we have here added the last link in the chain between the primary and quaternary symptoms.

This case is illustrative of several which we have published elsewhere under the name of _paresis sine paresi_ or latent neurosyphilis to illustrate how all the laboratory signs of neurosyphilis may be present in a patient without any physical or mental symptoms that may be correlated with these findings.

We summarize our discussion of this as follows:

1. There is a group of cases showing the laboratory signs characteristic of central nervous system syphilis: (_a_) positive W. R. in the serum, (_b_) positive W. R. in the spinal fluid, (_c_) pleocytosis, (_d_) excess of albumin, and (_e_) of globulin in the spinal fluid, (_f_) gold sol reaction of central nervous system syphilis, and which show no sign or symptom of neurosyphilis.

2. We believe these cases represent a form of chronic cerebrospinal syphilis, probably paretic in type.

3. They have the greatest theoretical and practical significance in the consideration of the life history of neural syphilis, in the concept of _Allergie_, in regard to results of treatment, and finally as to the evaluation of the laboratory tests.

4. Here is perhaps offered the last link to form a complete chain between the symptoms of the primary stage of syphilis and its final termination of life as the result of the diseases cerebrospinal syphilis or general paresis.

=SYPHILIS (?); EXOPHTHALMIC GOITRE; neurosyphilitic old lesion of
optic thalamus; unilateral induration and atrophy of left cerebral
cortex. Autopsy.=

=Case 53.= Carrie Pearson, a housewife 25 years of age, died at Danvers Hospital less than a week after admission, and it was at first stated that her symptoms had lasted but two weeks before admission. In point of fact, a further investigation showed an important succession of symptoms, lasting some four years.

Carrie had been considered a healthy child, going to school at the usual age, and progressing well with her studies. She however, left school in the ninth grammar grade, at the age of 15, and went to work in a milltown. She married a worthless person at the age of 18, and lived with her husband for three years. There was one child born a year after marriage. Two years later, however, a tremendous goitre had developed such that her neck was described as “out square with the face,” and at the same time the patient’s eyes had become prominent.

About two weeks before admission, she had gone to a neighboring town to take care of a sick woman, but during her endeavor to be a nurse, she had broken out into a mania, tearing up furniture and bedding, and talking irrelevantly for a period of four days. She also showed insomnia and continually tore off her clothing from her body.

Upon =examination=, the marked enlargement of the thyroid gland together with the prominent eyeballs, husky voice, and pulse rate of 150 per minute, were entirely consistent with the diagnosis of exophthalmic goitre. The patient described herself as “Carrie Nation.” Asked to write her name, she took the pen and tried to spatter ink, wrote hurriedly and carelessly her maiden name and several words without apparent meaning. Asked to write, “God save the Commonwealth of Massachusetts,” she wrote: “God save the common pal U S Spe Manor Gen, or til pat. Since Lord, or no prime in Hear to the God Tel. Ho. n and or Mabel, or gal.” After this, she took paper and wrote meaningless scrawls, saying that it was Japanese writing. There was much motor restlessness with distractibility, pointing and grimacing, mimicking the actions of those about her.

Death occurred from exhaustion, and the case might not have been regarded as unusual except for the autopsy, which showed a peculiar brain lesion, described below. The point of greatest interest in the case was the fact that syphilis is, although not proved to exist by laboratory tests, beyond question a factor in the case. Although the woman had given birth to a normal child, who is still alive, yet in the period of a few years her breasts had atrophied, her hair had disappeared from the axilla and from the pubes; varicose veins had developed in both legs. Whereas there was little or no fat over the chest or back, the omentum and mesentery were very plentifully supplied with fat. It is probable, then, that we are dealing with a case of exophthalmic goitre somehow of syphilitic origin. The brain lesion is consistent with this hypothesis.

=Autopsy=, March 3, 1907. Four hours post mortem.

Body length, 165 cm. Body of a well developed and well nourished
young woman. Lividity in dependent parts. Purplish discoloration of
left thigh to knees. Skin rough and scaly. Petechial eruption over
chest. Neck thick, protrudes anteriorly. Varicose veins over upper
parts of calves on both legs. Eyes protruding, not covered entirely
by lids. Pupils equal, dilated. Subcutaneous fat very deep over
lower part of body. Very little fat over chest and back. Breasts are
very small, apparently atrophied. Normal amount of hair on head,
slight amount over pubes. Axillary hair absent. Fat on section of a
light yellow color. Omentum extends to pubes, plentifully supplied
with fat. Large amount of mesenteric fat. Appendix normal.
Intestines smooth and glistening. Slightly injected. No fluid in
peritoneum. Uterus small, retroverted.

Cortical hemiatrophy—A, relatively normal right precentral (“motor”)
cortex; B, atrophic left precentral.

Note in B:

1. Absence of giant pyramids of Betz (corticospinal, upper motor
neurones).

2. Superficial (subpial) condensation of tissues with sclerosis
(gliosis). The tissues in all areas examined _on the left side_
yielded this effect.
]

HEAD: HAIR in good quantity. SCALP normal. CALVARIUM shows diploë.
DURA MATER over left cerebral hemisphere inseparably adherent to
calvarium, over right hemisphere normal. Arachnoidal VILLI
moderately developed. PIA MATER shows injected veins, notably in the
sulci of the right hemisphere. Pia mater everywhere thin and clear.
VESSELS at base of normal appearance.

BRAIN weight 1180 grams. Spread on a board, the right hemisphere
tends to flatten so that it measures 1.5 cm. more from side to side
than its fellow. Besides more marked venous injection, the right
hemisphere shows also flatter and slightly more plastic
convolutions. The posterior poles of the hemispheres are a little
firmer than the parts anterior. The orbital and hippocampal gyri on
the right side are a little firmer than the surrounding parts. On
section the gray and white matter shows no lesions, excepting the
slight plasticity of the tissues at large on the right side and a
well marked induration, with retraction under the knife, of the
occipital and hippocampal white matter. The basal ganglia of the
left side are normal. On the right side a sagittal section
demonstrates a rounded area of induration, with ill-defined borders,
measuring perhaps 1.5 cm. from above downwards by 2 × 2 cm.,
situated largely in the lenticular nucleus and involving the greater
portion of the globus pallidus, a small segment of the putamen below
and behind and the regionary part of the anterior commissure with
surrounding tissues. The most striking feature of this lesion is the
occurrence in the middle of a cluster of vacuoles or cystic clefts,
with smooth pale interiors, ranging from pinhead to 0.25 cm. or even
0.5 cm. in greatest diameters. There are six to eight clefts to a
surface of section. The color of the lesion differs little from that
of the globus pallidus itself, but the tissue is a trifle
translucent. It is impossible to demarcate the lesion with the eye.
Induration is demonstrable several mm. beyond the visible part of
the lesion. The consistence of the lesion slightly surpasses the
usual consistence of the olivary bodies.

CEREBELLUM, PONS and BULB weight 165 grams. Cerebellar tissue a
trifle more plastic than usual. The right olive is not so prominent
as usual.