rotation, limb-contractions and clonic movements.
1. Should this soldier recover for disability obtained in service?
Marie was inclined to think military service in part responsible
for the development of the paresis. Laignel-Lavastine thought so
also, but that the amount assigned should be 5%–10% of the maximum
assignable.
2. What is the duty of the military authorities relative to so
called traumatic paresis? Medicolegally speaking, Froissart,
quoted by Rayneau, states that a victim of traumatic paresis _may
or may not_ have presented mental disorders before the accident,
that is, that the paretic symptoms may develop out of a clear sky
as a result of the accident. The accident itself must be of a
serious nature. The accident must be followed by phenomena
pointing to brain injury of traumatic nature. These phenomena need
not be characteristic symptoms of general paresis at the outset.
The period elapsing between the trauma and the supervening
condition of paresis must be occupied without notable
interruption, at first by phenomena of a purely traumatic nature,
later by signs indicating the onset and evolution of general
paresis.
The French invaliding process called _Réforme No. 1_ with pension
is granted according to the governmental instructions only to
officers, subalterns, and soldiers whose disease is due to trauma.
In view of this governmental regulation, the military surgeon must
write out certificates describing every cranial trauma, however
slight, which might have a bearing on the development of paresis.
However, he should not too readily admit trauma as a cause of
paresis. If a long period of quietude, a period in which the
trauma itself seems to have undergone a complete recovery,
supervenes, then general paresis should not be reported by the
surgeon.
Lépine has recently noted the following features as desirable in
board reports concerning paretics: nature of trauma, length of
service, fatigue endured, insomnia, date of infection, treatment,
W. R.
=Can “gassing” light up a paresis? Example from de Massary of
Issy-les-Moulineaux.=
=Case H.= A soldier, 35, was sent to the _Centre Neurologique_ with a
hospital ticket reading:
“Neurasthenia, general weakness following intoxication by gas.”
The soldier was thought at first to be a neurasthenic. But he soon
showed signs of more pronounced mental trouble. The voice was
suspicious. There was a slight irregularity of pupils.
An epileptiform attack occurred, followed by aggravation of symptoms.
Lumbar puncture showed pleocytosis. The W. R. of the serum proved
positive.
Yet the evident =neurosyphilis=, possibly =paretic= (de Massary’s
diagnosis), was preceded by a neurasthenia and the neurasthenia was
preceded by “gassing.”
De Massary believes the patient _and his family_ would perhaps be
justified in believing the condition produced by the injury. De Massary
is not clear as to the financial deserts of the patient. It is not a
manifest case of aggravation of antebellum symptoms, even if it be
neuropathologically an instance of acquired loss of resistance to
pre-existent spirochetes in body or brain.
1. What adjuvant factors have been recognized in military paresis?
Aside from syphilis, Rayneau finds that alcoholism, malaria,
sunstroke and various intoxications serve as causes for paresis.
Rayneau points out that the apparent integrity of the mind in
general paresis may be such that they last in the army some time
and have their oddities ascribed to misconduct or breaches of
discipline. In fact the Legrande du Saulle called this early
period in general paresis the _medicolegal period_, showing, as it
so often does, thefts, outrages against decency, frauds, assaults,
exhibitionism and the like. To be sure these acts are absurd and
infantile and not difficult to recognize as of psychotic origin.
=Syphilis may bring out epilepsy in a subject having taint. Case
from Bonhoeffer, 1915.=
=Case I.=[28] A man of 35 in the _Landwehr_ acquired syphilis some time
in the summer of 1914. He was a good soldier, passed through several
clashes, and was promoted to _Unteroffizier_.
To understand what followed it must be stated that he had been a
bed-wetter to 11, had been practically a teetotaler (Bonhoeffer’s point
is perhaps that otherwise epilepsy might have developed sooner?), and,
when he did drink, vomited almost at once, and had amnesia for the
period of drunkenness. His father had been somewhat of a drinker. His
sister had suffered from convulsions as a child.
February, 1915, the _Unteroffizier_ lost appetite, got headaches, and
went to hospital for a time. Upon getting better, he was sent on service
to Berlin. In a Berlin hotel he had his first convulsions and
unconsciousness, biting his tongue. He was confused for several days,
and, when he had become clear, had a pronounced retrograde amnesia
together with a tendency to fabricate a filling for the lost period.
This retrograde amnesia is uncommon in epilepsy and suggests organic
disease. No sign of organic disease was found on neurological
examination. The patient had no signs of the epileptic make-up. The
serum W. R. was negative. On the whole, Bonhoeffer regards the epilepsy
as “reactive” to the syphilis, as a syphilogenic epilepsy.
As to the amnesia, it is of interest that alcohol should long before
have been able to cause amnesia in this man in the same way as does now
the syphilitic epilepsy.
1. In view of the fact that this _Landwehr_ man appears to have
acquired syphilis while on campaign, what is the responsibility of
the government for treatment? The Canadian authorities, as stated
under Case E, are in doubt whether contraction of venereal disease
constitutes negligence on the part of the soldier. It would appear
to us that where a government does not take suitable steps to
prevent the acquisition of syphilis by the soldiers, the
government must assume a measure of responsibility for the
syphilis incurred. The government’s responsibility would be still
greater in equity, it would appear, if commercial opportunities
for the acquisition of syphilis are maintained under more or less
close government supervision or (even as has been claimed for
certain encampments on our own Mexican border) if shelter for
illicit sex relations is afforded within the limits of a military
camp. In a certain community, “E,” for example, it is claimed by
Exner,[29] the district for prostitutes was “situated within the
lines of military camps and protected and ‘regulated’ by the
military authorities.”
But even if the government has no legal responsibility in this
regard, it would be well to consider the ultimate results of the
syphilis that will probably be acquired by great numbers of
soldiers under campaign conditions. Aside from the ravages of
syphilis outside the nervous system, it is well known, as Weygandt
intimates for German conditions, that the aftermath of war will be
a high proportion of cases of neurosyphilis.
Weygandt remarks in his review of the influence of the war upon
psychiatry, that the opportunity for syphilitic infection in the
campaign is considerable. In the war of 1870, the conditions in
this regard were extremely unfavorable, and writing in 1915,
Weygandt remarks that at present there should be a prophylaxis
against syphilitic infection by the soldiers, which prophylaxis
should be the most energetic possible. Continence on the part of
the soldiers and the isolation of infected women, with examination
by specialists, have been advocated by Neisser and by Mendel. In
the ’80’s a great number of cases of locomotor ataxia developed in
Germany, which were due to syphilis acquired by the soldiers and
officers in the war of 1870.
=Syphilis in a psychopathic subject. Convulsions 5 days after
Dixmude. Case from Bonhoeffer, 1915.=
=Case J.=[30] A soldier in the reserves, 23, was, subsequently to his
being brought to hospital, described by his wife as a rather
over-sensitive fellow, who could hardly look at blood and was meticulous
about the household. He had always been subject to headaches, especially
after hard work. However, he had passed through his military training
well in 1910, not even having been _bestraft_.
He began service in October and fought at Dixmude on the 19th. On the
24th in the trench and while being carried back, he had several spells
of pallor, falling stiff, and then having convulsions. Brought finally
to the Charité in Berlin, he had more spells of sudden pallor, collapse
with brief convulsions, tossings in bed, and absences, post-convulsive
headaches, and mild bad humor.
There were numerous attacks several days apart in the first seven weeks.
The patient was not of an “epileptic” disposition, though he was rather
readily dissatisfied. Headaches also occurred without relation to
convulsions.
The serum W. R. was positive. Treatment by mercurial inunctions. No
further convulsions. Prognosis as to the possibility of a constitutional
epilepsy unknown.
=SYPHILITIC ROOT-SCIATICA (lumbosacral radiculitis) in a fireworks
man with a French artillery regiment. Case presented from Dejerine’s
clinic by Long.=
=Case K.= No direct relation of this example of root-sciatica to the war
is claimed nor was there a question of financial reparation.
There was no prior injury. At the end of March, 1915, the workman was
taken with acute pains in lumbar region and thighs, and with urgent but
retarded micturition.
Unfit for work, he remained, however, five months with the regiment, and
was then retired for two months to a hospital behind the lines. He
reached the Salpétrière October 12, 1915, with “double sciatica,
intractable.”
There was no demonstrable paralysis but the legs seemed to have “melted
away,” _fondu_, as the patient said. Pains were spontaneously felt in
the lumbar plexus and sciatic nerve regions, not passing, however,
beyond the thighs. These pains were more intense with movements of legs;
but coughing did not intensify the pains. Neuralgic points could be
demonstrated by the finger in lumbar and gluteal regions and above and
below the iliac crests (corresponding with rami of first lumbar nerves).
The inguinal region was involved and the painful zone reached the
sciatic notch and the upper part of the posterior surface of the thigh.
The sensory disorder had another distribution objectively tested. The
sacral and perineal regions were free. Anesthesia of inner surfaces of
thighs, hypesthesia of the anterior surfaces of thighs and lower legs.
The anesthesia grew more and more marked lower down and was maximal in
the feet, which were practically insensible to all tests, including
those for bone sensation. There was a longitudinal strip of skin of
lower leg which retained sensation.
Position sense of toes, except great toes, was poor. There was a slight
ataxia attributable to the sensory disorder—reflexes of upper
extremities, abdominal, and cremasteric preserved, knee-jerks, Achilles
and plantar reactions absent.
The vesical sphincter shortly regained its function, though its disorder
had been an initial symptom.
Pupils normal.
The “sciatica” here affects the lumbosacral plexus. Signs of disorder at
one time or other affected the first lumbar distribution of the third
lumbar and first and second sacral nerves.
As to the syphilitic nature of this affection, there had been at
eighteen (22 years before) a colorless small induration of the penis,
lasting about three weeks. There was now evident a small oval pigmented
scar. The patient had married at 20 and has had three healthy children.
The lumbar puncture fluid yielded pleocytosis (120 per cmm.). Mercurial
treatment was instituted.
The treatment has not reduced the pains. Long thinks it was undertaken
too long (six months) after onset. The warning for early diagnosis is
manifest. There was somehow a delay under the medical conditions of the
army.
=Can the “lighting up” of NEUROSYPHILIS IN CIVIL LIFE be induced by
the domestic stress of war? A possible example from Dr. R. Percy
Smith, London.=
=Case L.= A German Jew in London passed into the PARETIC form of
NEUROSYPHILIS shortly after the outbreak of war under conditions
suggesting that the stress of emotions directly or indirectly lighted up
the neural process.
The man was a bank-officer, 52 years old, and married. He had lived many
years in England and was in fact a naturalized citizen. He had been
under treatment for syphilis by Sir Jonathan Hutchinson, 29 years
before, namely, at the age of 23. Subsequently, Sir John had given him
permission to marry.
It proved that for years the man had had fixed pupils, absent
knee-jerks, and a perforated ulcer of the foot. However, there had been
no other mental or nervous symptoms preventing bank-officer’s work.
At the outbreak of war the man was discharged from the bank. He grew
worried and sleepless. He began to charge himself with sex irregularity.
He went down to the city and burned trust documents belonging to others.
From worry and self-accusation he passed into depression and agitation.
He developed a belief that not only he but also his German wife were to
be executed. He thought he was a criminal and was to be hanged.
The depression then altered to a condition of hilarity and loquacity.
In addition to the fixed pupils and absent knee-jerks, a speech disorder
shortly developed.
The patient was placed under care, but quickly (a few months?) passed
into an advanced stage of paretic neurosyphilis and died.
=SHELL-SHOCK PSEUDOPARESIS (non-syphilitic). Recovery. Case from
Pitres and Marchand of Bordeaux.=
=Case M.= June 19, 1915, a shell exploded some distance from Lieutenant
R. He remembers the gaseous smell, the bursting of several shells nearby
and a sensation of being lifted into the air. When he recovered
consciousness, he was in hospital at Paris-Plage, covered with bruises
and scratches. They told him he had been delirious and had vomited and
spat blood.
June 24, his wife came to see him, but this visit he could not remember.
Nor could his wife at first recognize him, he was so thin. He roused a
few moments and recognized his wife, but relapsed into torpor again.
Speech was difficult and ideas confused.
A few days later he was able to rise; but his mental status grew worse,
especially as to speech and writing; the latter quite illegible. There
was insomnia, or, if he slept, war dreams.
August 7, he began a period of five months’ convalescence passed with
his family, depressed, given to spells of weeping, confined to bed or
couch, unable to “find words,” conscious of his state and troubled about
it, speaking of nothing but the war, and afraid to go out for fear of
ambuscade. There was at first a slight lameness of the right leg.
Although he could walk, he felt pain in the knee on flexing the right
leg on the thigh. He walked holding this leg in extension.
On going back to the colors, he was immediately evacuated to the _Centre
Neurologique_ at Bordeaux, January 20, 1916.
Examination found a bored, impatient, irritated man, vexed that a man
who was not sick should be sent up “_comme fou_.”
Omitting negative details, =neurological examination= showed slight
lameness as above, body stiff and movements jerky; difficult, unsteady
gait. The lieutenant could stand for some time on either leg, tongue and
face tremulous during speech. Limbs moderately tremulous, especially in
the performance of test movements.
Knee-jerks and Achilles jerks absent. Other reflexes, including
pupillary, normal. Segmentary hypalgesia of right leg, especially about
knee. Tremulous speech and writing. Patient would stop short in speaking
for lack of words.
Malnutrition. Appetite good, but a bursting feeling after meals.
Skin dry, scaly on legs, fissured on fingers.
Serum W. R. negative. Fluid not examined.
=Mental examination.= Conscious and complaining of his troubles,
Lieutenant R. claimed persistently that he was not sick. Memory for
recent events was in general poor. Errands easily forgotten. Lost in the
street. Complaint of corpse odors round him. Everybody is looking at him
and making fun of him. He was apt to insult bystanders. He was afraid of
German spies. Things in shops angered him as they seemed to him to be of
German manufacture.
There were frequent periods of depression, with pallor and no
spontaneous speech for some hours to a half-day. Headaches coming on and
stopping suddenly.
As to diagnosis, the first impression, say Pitres and Marchand, was that
of general paresis. The progress of symptoms after the shock was
consistent with this diagnosis. The mental state and the physical
findings seemed consistent, although the pupils were normal. His partial
insight into his symptoms was not inconsistent with the diagnosis. He
had a characteristic self-confidence. There had been four stillbirths
(two twins) two children are alive, 11 and 13. Typhoid fever at 30.
Syphilis denied. No mental disease in the family.
The patient had never done military duty, having been invalided for
“right apex.” But he had volunteered and been accepted in September,
1914.
1. Was this diagnosis, general paresis, at any time justified? The
spinal fluid should of course have been examined. The peculiar
lameness of the right leg was certainly not characteristic of
general paresis, and was perhaps hysterical. (There was no
limitation of visual fields or any other definite sign of
hysteria.) Presumably some quality of speech defect, the amnesia,
and the euphoria, together with absent knee-jerks, led to the
diagnosis general paresis. By the 20th of March, 1916, the
knee-jerks had become lively; the Achilles jerks normal. At this
time the patient had gained in weight, could walk though stiffly,
had headache (especially right frontal) and a feeling of lead in
head, less tremor, lack of desire to undertake anything. He still
wanted to go back into service. He still saw spies about. Dreams
terrible; devoured by spiders, leggins instruments of torture.
Skin still atrophic. June 4 there was no more tremor of speech or
face. Symptoms largely disappeared except a few ideas of
persecution. Recovery October, 1916.
2. How was Lieutenant R. cured? Apparently by rest in the _Centre
Neurologique_. Pitres and Marchand do not speak of the subtle
relation between mental state and the idea of non-return to
military service. This motive might still work even if Lieutenant
R. kept protesting quite sincerely that he wanted to go back into
military service.
=SHELL-SHOCK PSEUDOTABES (non-syphilitic, serum W. R. positive).
Improvement. Case from Pitres and Marchand of Bordeaux.=
=Case N.= Innkeeper B., 36, a shell-shock and burial victim June 20,
1915, was looked on by a number of physicians as a case of genuine
tabes.
Even eight months after the episode, he still showed (when observed by
Pitres and Marchand, February 3, 1916) absence of knee-jerks and
Achilles jerks, a slight swaying in the Romberg position, pupils
sluggish to light, incoordination, delayed sensations. There was also a
history of pains in the legs, compared by the patient to those of
sciatica. These pains came in crises, the longest of which had lasted 30
hours.
It seems that this soldier’s troubles began the day after his shock with
a feeling of swollen feet and of cotton wool under them. He stayed on
service, however, walking with increasing difficulty.
At the time of his evacuation, July 10, he could walk with great
difficulty. “Strips of lead were between his legs.” He could hardly
control movements in the dark, or descend stairs. Often his legs would
bend under him. Vesical function sluggish.
After a few months the patient could walk better. On February, 1916, he
walked thrusting his legs forward trembling, and dragging toes a little.
He could not support himself on either leg. Jerkiness and incoordination
in extension or flexion of leg on thigh.
The muscular weakness was decidedly against tabes or at all events a
pure tabes. The incoordination proved to be due, not to loss of position
sense (which was intact) but to unsteady muscular contractions. Deep
sensibility was intact.
There were no mental symptoms. There was a slight hesitation in speech
and doubling of syllables, but nothing demonstrable with test phrases.
The serum W. R. was positive. Syphilis denied.
1. What is the cause of these phenomena? Pitres and Marchand lean to
the hypothesis of slight internal traumatism. They believe that
there is either (a) slight internal hemorrhage in the nervous
system, or possibly (b) what they call “nerve cell contusion,” or
perhaps (c) caisson-disease-like phenomena from aerial
decompression. Some authors incriminate (d) the gases. It has been
reported by certain French authors that shortly after shell-shock
injury or burial there is a pleocytosis in the spinal fluid as
well as evidence of hemorrhage. The pleocytosis is said to last
only a short time; hence when patient arrives at a base hospital
lumbar puncture usually discloses nothing.
Baalim and Ashtaroth
Paradise Lost, Book I, line 422.
VII. SUMMARY AND KEY
No more important human problem now exists than syphilis. Syphilis of
the nervous system or, briefly, neurosyphilis is a highly important
fraction of the total problem. The few outstanding dates and items which
we present on the following page give but a faint idea of the amount of
observation and thinking which the medical aspects of neurosyphilis
alone have required. The present work deals with but a small fraction of
the results of this work, nor can we more than glance at the scientific
history of syphilis and neurosyphilis—a history that would form an epoch
in itself.
It is only in the most recent years that syphilology and the narrower
science of neurosyphilology have threatened to become separate
disciplines boasting full time specialized workers. Up to recent years
the contributions to the theory of syphilis have been largely
by-products of work in larger sciences and arts. Thus, the cellular
pathology of syphilis as worked out by Virchow and the more special
vascular features as worked out by Heubner were incidental in the
progress of pathological anatomy and histology. The bold procedure of
Quincke in proposing lumbar puncture also had its more general ground in
the extension of clinical medicine,—an interpretation likewise true of
the French achievements in the cyto-diagnosis and chemical diagnosis of
the lumbar puncture fluids. The careful histological definitions of the
Nissl-Alzheimer group were incidental to the application of approved and
classical pathological methods to neurological and psychiatric material.
Again, the work of Schaudinn, as well as that of Metchnikoff and Roux,
was ingenious work with the methods of parasitology and experimental
pathology. The great work of Schaudinn in establishing the constancy of
the spirocheta pallida in syphilis may be said to have started
syphilology as something approaching a special discipline. The ideas of
one of the greatest of immunologists, Bordet, were almost immediately
applied to the serum diagnosis of syphilis by Wassermann and the further
application of this method to the problems of neurosyphilis was almost
immediate, with the spirocheta pallida as an object of attack. The
commanding intelligence of Ehrlich could at once seek application of
long incubated ideas of chemotherapy with the startling outcome,
salvarsan.
=DATES, NEUROSYPHILIS=
VIRCHOW PATHOLOGY 1858
HEUBNER ENDARTERITIS 1874
QUINCKE LUMBAR PUNCTURE 1891
RAVAUT, SICARD, NAGEOTTI, WIDAL CYTODIAGNOSIS, C.S.F. 1901
WIDAL, SICARD, RAVAUT ALBUMIN, C.S.F. 1903
METCHNIKOFF AND ROUX TRANSMISSION TO APES 1903
ALZHEIMER HISTOPATHOLOGY, BRAIN SYPHILIS 1904
SCHAUDINN AND HOFFMANN SPIROCHETA PALLIDA 1905
WASSERMANN, NEISSER AND BRUCK SERUM DIAGNOSIS 1906
PLAUT WASSERMANN REACTION, C.S.F. 1908
EHRLICH SALVARSAN 1909
SWIFT AND ELLIS SALVARSANIZED SERUM 1912
NOGUCHI AND MOORE SPIROCHETES, BRAIN TISSUE, 1913
PARESIS
LANGE GOLD SOL TEST 1913
CHART 28
The history of syphilis and neurosyphilis was now to be thickly sown
with ideas and results growing from the achievements of Schaudinn and
Ehrlich. The positive reactions in the blood and spinal fluid in the
most striking of mental diseases, general paresis, led to the impression
that general paresis itself might at last be proved to be what Mœbius
had suspected, namely, 100% syphilitic. We know how difficult is the
technical proof of spirochetosis in the brains of general paretics both
post mortem and ante mortem, but no one doubts the certainty of the
syphilitic hypothesis concerning the origin of general paresis.
The data of the gold sol reaction ultimately obtained from the ideas of
Thomas Graham concerning colloids, as developed by Szigmondi and
effectively applied by Lange, have broadened and solidified the whole
plane of attack.
The ingenious suggestions of Swift and Ellis (salvarsanized serum) and
the notable work of Noguchi and Moore (spirochetosis in paretic brains)
indicate to us as Americans what the establishment of scientific
institutes may do to permit the rapid application of new ideas to
branches of inquiry that are opened out. Scientific institutes do not
manufacture a Virchow, a Metchnikoff, a Schaudinn, a Bordet or an
Ehrlich but they directly permit such men to work and indirectly
stimulate the development of more.
The series of 137 cases here at least presented does not touch
systematically the problems of the neuropathology of syphilis, which
would themselves require a textbook of respectable size. We have,
however, presented in Part I, cases 1 to 8, some indication of the
protean nature of the material and from time to time in the remainder of
the book somewhat fuller accounts of the pathological anatomy and
histology have been presented than are strictly necessary in the
demonstration of the principles of modern systematic diagnosis and
treatment.
Our work may be said to represent psychopathic hospital practice as
available to us in our official capacities at the Psychopathic
Department of the Boston State Hospital. A word is necessary concerning
the nature of this practice. The dispensary and ward practice of a
modern state psychopathic hospital, such as the Boston institution
(founded in 1912) and the Ann Arbor institution (founded in 1906), is to
be sharply distinguished from asylum practice. Those who have not
followed the evolution of the modern psychopathic hospital with the
lowering of bars to the admission of patients and the extension of its
benefits to a group of sick persons far removed from the medicolegal
concept “insanity” may not soon grasp the general nature of psychopathic
hospital material. Psychopathic hospital practice stands, in fact,
almost midway between asylum practice in the classical sense and private
practice. This has come about through the great extension of the
so-called voluntary relation under which hundreds of patients now resort
to the beds and out-patient rooms of a psychopathic hospital, who would
formerly have remained untreated or inadequately treated. Moreover, the
broadening of the concept of mental diseases as a whole has permitted in
some parts of the world the establishment of laws under which
psychopathic and psychotic patients may be brought to psychopathic
hospitals and even to asylums under the easiest possible conditions and
restrictions, omitting court procedure altogether. The operation of the
voluntary and temporary care provisions of law has accordingly yielded
us, in the Boston institution, a great group of cases formerly not at
all accessible to hospital diagnosis and treatment. Needless to say, as
always under such conditions, we have been able to show not merely that
hospital diagnosis or treatment is of importance to a new group of
cases, but also that home treatment, especially home treatment under
supervision, is possible and even ideal for a large group of cases about
which utter darkness or profound misgivings ruled in the not very
distant past.
Accordingly, we are fain to insist that our material is of importance in
new programs of community organization for the stamping out of disease.
The work in psychopathic hospitals upon neurosyphilis in particular is
essentially a part of the public health program, although our special
work will not soon be taken over by the public health officers, so
complicated are the ramifications of medical and social diagnosis and
treatment in the neurosyphilis group.
We have tried in Part IV (medicolegal and social cases) to give a few
examples to illustrate the part played by neurosyphilis in society; but
we regard this part of our work as the least satisfactory and the least
representative in the total work. Our colleagues in social service, in
mental hygiene, in psychopathology and in criminology will easily in the
next few years provide a far more adequate basis for a full account of
the public and social aspects of neurosyphilis. One point we should
emphasize here. The psychopathic hospital worker, whether physician or
social worker, must shortly decide upon and consolidate a program with
relation to the families of neurosyphilitics.
The syphilographers of the dermatological and special syphilis clinics
have their identical problems with the families of syphilitics; but the
dispensaries for mental cases and in particular the psychopathic
hospital and asylum out-patient departments tap another reservoir of
syphilitic families at a stage when the memory of the initial horrors of
syphilitic infection is dimmed or erased. Any program for the diagnosis
and treatment of syphilis of the innocent must take into account not
only the skin, syphilis, and internal medicine clinics but also the
clinics for mental and nervous diseases wherein neurosyphilitics are not
infrequent. Whether the ultimate percentage will stand at 10, 15 or 20%
for the neurosyphilitics in mental clinics, is of no importance to the
principle. There are enough neurosyphilitics having economical
importance and humanly precious families to warrant definite steps.
The Massachusetts Commission for Mental Diseases has in the last few
years employed the services of two medical workers whose time has been
largely devoted to the applications of our recent knowledge in
neurosyphilis and has gone so far as to establish a neurosyphilis ward
in one of the district state institutions (Summer Street, Worcester,
under the Grafton Hospital Board). Special social workers in the field
of neurosyphilis have also been available from time to time. These
social workers are enabled with the support of the medical profession to
do a great deal of good, for example, with the slogan THE CHILD OF A
PARETIC IS THE CHILD OF A SYPHILITIC.
The nature of the intake of patients into psychopathic hospital wards
and out-patient clinics is such that great numbers of non-mental
syphilitics arrive for diagnosis and possible treatment. Moreover, the
existence of syphilis in non-suspects is a fact picked up by the way in
routine Wassermann serum diagnosis.
The mental clinic in the modern sense with the medicolegal bars lowered
or well nigh removed, turns rapidly into a clinic for neurological cases
as well. The German models for mental and nerve clinics are rapidly
being imitated. The result of this administrative novelty in our
hospital procedure has incidentally yielded us many representative cases
of entirely non-psychotic and even non-psychopathic neurosyphilis. Our
impression grows and deepens that _the neurosyphilitic is seldom merely
a spinal syphilitic_. The neurosyphilitic is nearly always the victim
not merely of spinal disease but also of intracranial disease. Per
contra, the victim of intracranial neurosyphilis is almost always more
or less importantly affected by spinal neurosyphilis.
The net result of the modern work on neurosyphilis has been to bring the
neurologist and the psychiatrist together upon one platform in diagnosis
and more and more upon one platform in treatment. But aside from the
clinical evidence that the neurosyphilitic is apt to be a victim of both
brain syphilis and cord syphilis, the autopsy evidence is stronger
still. Even the victim of tabetic neurosyphilis (“tabes dorsalis”)
himself is rarely found at autopsy without more or less evidence of
significant encephalic disease of a chronic inflammatory or degenerative
nature. Aside from tabes dorsalis and Erb’s paraplegia, the rule is
almost universal that neurosyphilis is a matter of the entire nervous
system.
In view of the generalization of neurosyphilitic process, one might
question the advantage of any topical grouping of neurosyphilitic
disease. Practically speaking, however, as we have shown in Chart 5, it
seems advisable to separate the neurosyphilitic diseases into six
roughly distinguishable groups. First, there is the great group that we
have chosen to term =diffuse neurosyphilis=, including many of the cases
of so-called cerebral or cerebrospinal syphilis of the neurological
clinics and the group of cases that have been treated in private
practice by internists and neurologists without recourse to
institutions. These cases have lived at home and have not been socially
hard to manage until the late phases of their disease when the victims,
if poor, are sent to almshouses and infirmaries under municipal or state
care. These are the cases which have been in the past regarded as most
amenable to the classical iodid and mercurial treatment. Indeed there is
record of numerous therapeutic successes in the group.
Whereas the lesions in diffuse neurosyphilis are chiefly chronic
inflammatory and degenerative changes of a diffuse nature (with vascular
changes incidental or subordinate to the inflammation and the
degeneration), there is an important and large group of cases that we
have termed =vascular neurosyphilis= in which the factors of
inflammation and degeneration are subordinate to vascular insults. These
are cases of syphilitic arteriosclerosis and the best examples are
victims of cerebral thrombosis. The clinical symptoms of the immediate
attacks (of apoplectiform, epileptiform or other acute nature) are not
in themselves distinguishable from the immediate effects of
non-syphilitic vascular disease; nevertheless the establishment of their
syphilitic etiology is of the utmost importance on account of the
possibilities of treatment of the underlying syphilis. For, as the
neuropathologist must always insist, the immediate effects of vascular
insults whether syphilitic or non-syphilitic are much more extensive
than the ultimate paralytic or residual irritative effects; and by
consequence a greater optimism is justifiable in the confronting of
these cases than the nihilistic observer is likely to entertain.
Physicians dealing with chronic disease in general are apt to be
somewhat nihilistic, but this nihilism is increased a hundred fold in
disease of the nervous system. How important then is any work which
shall demonstrate partial or even complete recovery from serious looking
apoplectic and other seizures, besides all of which the point of
syphilitic treatment naturally lies in the prevention of future insults
of the same sort. Therapeutic experience in this vascular group has
almost as good a toll of successes as in the diffuse neurosyphilis group
above mentioned, that is to say, the modern systematic treatment and
even the old pre-salvarsan treatments have succeeded fairly well in
removing the products of inflammation from the membranes of the nervous
system and in abolishing vascular disease.
The old principle that the dead neurone in the central nervous system
cannot be regenerated remains a perfectly firm principle; but there are
any number of neurones and even neurone systems that are not essential
to life or to the pursuit of happiness. We accordingly have just as good
a theoretical therapeutic outlook in many instances of chronic
neurosyphilis as we have in chronic diseases of many other organs. Add
to this the fact that a great number of the most sharply-defined and
grave symptoms are probably not due to destruction of neurones but to
irritation and functional disability of neurones, and the conclusion is
compelled that, as hinted above, an entirely unjustifiable pessimism and
nihilism have prevailed in some quarters. Of course, the recoil from
such pessimism with the onset of salvarsan treatment led various
enthusiasts to an undue optimism.
Another great group distinguished by the existence of spinal cord
disease is the group we have termed =tabetic neurosyphilis=, which group
contains the classical tabes dorsalis or locomotor ataxia and its
congeners.
The question of therapeutic optimism comes up most forcibly in the field
of tabes. It is hard, however, at this time to give a proper and
scientifically founded estimate of the therapeutic outcome in tabetic
neurosyphilis with modern methods. So much can be said: namely, that the
alleviation of pain and the palliation of other symptoms can be
successfully claimed as a result of the renewed interest in the
treatment of this affection. What was said above concerning the finality
of the death process in a dead neurone is very strikingly true, of
course, of some of the neurones of the posterior columns in tabes
dorsalis. Still only portions of these neurones (namely, those which run
an intradural course) are strikingly altered in a great many cases. Now
and again one is greatly astonished to observe the restoration of the
lost knee-jerk in cases of neurosyphilis (see for instance the case of
Alice Morton (1), with discussion). In short, the relation of several
tabetic symptoms to irritative conditions and functional disability of
neurones may be considered established. Naturally, moreover, if therapy
can stop the upward course of the affection as it passes from lower to
higher nerve roots (according to reasonably well-established ideas of
the genesis and progress of this affection), we are entitled to a
further degree of optimism.
The question of therapeutic optimism _versus_ pessimism is forced upon
attention in the fourth great group of neurosyphilitic diseases which we
have chosen to distinguish, namely, the group of =paretic neurosyphilis=
including the disease formerly known as general paresis, paralytic
dementia, softening of the brain and the like.
Of course, no one can gainsay there is a group of cases having in the
natural course of events a prognosis of fatality within a term of years,
say three to five years, and we have cases in our series which go to
show that even with the modern intensive treatment the characteristic
down-grade symptomatic progress and ultimate fatality occur. Still, we
have other cases diagnostically on all fours with the fatal cases that
have seemed to get either entirely well with the laboratory tests
returning to normal and without further mental symptoms, or else lose
mental symptoms on the one hand or laboratory signs on the other. We
should strongly object to any account of paretic neurosyphilis which
should insist that its necessary outcome is fatality within a term of
years. Of course, viewing our knowledge of the affection in the past, we
should be compelled to object to the generalization “_paresis fatal_” on
the evidences of the universally recognized remissions. If nature can
stop a paretic process, why cannot man do as much? Can it be alleged
that our own apparent therapeutic successes and those of others are
merely curious examples of coincidences, namely, that remissions have
chosen to occur precisely when therapy was systematically applied? The
percentage of therapeutic successes with modern intensive treatment,
wherever it may ultimately stand, is already too high for this
hypothesis of fortuitous remissions.[31]
Moreover, we believe that the details of the clinical progress of some
of the reported cases are convincing on this point. What, however, is
the distinguishing feature of paretic neurosyphilis? It is in one sense
a particular kind of diffuse neurosyphilis. The tissues are apt to show
not only encephalic but also spinal changes. There is apt to be a more
or less well-defined meningitis, but the characteristic feature, without
which the diagnosis of paretic neurosyphilis would hardly be rendered,
is the existence of disease of the cerebral cortex. This disease is
parenchymatous in the sense of showing nerve cell destruction. There is
also an interstitial reaction in the shape of a neuroglia overgrowth,
but the striking and pathognomonic feature is the infiltration of the
sheaths of the small vessels in the cortex, giving evidence of an
inflammation very intimately affecting the cellular mechanisms of the
nervous system. It is striking how often a smaller or larger share of
the cells found in the vessel sheaths are plasma cells. It does not
appear, however, that the diagnosis of paretic neurosyphilis as against
diffuse non-paretic neurosyphilis can be made in the stained sections
with complete safety on the basis of plasmocytosis in the former and
lymphocytosis in the latter. Whatever the results of careful
histological differentiation by future neuropathologists may yield, it
is at all events true that we cannot yet make an important
differentiation clinically on the basis of the differential count of
plasma cells and lymphocytes in the puncture fluids. However this may
be, there is an important distinction between diffuse neurosyphilis of
the non-paretic type and paretic neurosyphilis in that paretic
neurosyphilis rarely if ever fails to show important degrees of
intracortical perivascular inflammation with larger or smaller numbers
of plasma cells.
What has the therapeutist to face in this matter? The answer, as
elsewhere, depends somewhat upon what the future may decide as to the
habitat and toxic or antitoxic activities of the spirocheta pallida. The
early claims that the spirocheta pallida was extravascular and lay for
the most part in the parenchyma and not in the vessel sheaths were
perhaps overbold, since other workers have found the spirochete in the
vessel sheaths also (Mott).
Aside from the spirochete and its accessibility to spirochetocidal
drugs, there seems to be no reason for supposing that the perivascular
sheaths cannot be cleansed of their inflammatory contents. There is,
again, no reason why the phagocytic cells should not continue to perform
their scavenger function until such time as the degenerative process in
the parenchyma (a process not necessarily progressive in the absence of
the spirochete or its products) ceases. There is every reason to suppose
that a great many of the clinical phenomena are not necessarily due to
permanent destruction of neurones and neuronic organs (dendrites,
axis-cylinders, nets and the like) but are due to various microphysical
conditions of pressure, intoxication and the like.
The inflammatory conditions in the spinal cord of poliomyelitis, which
conditions are precisely as striking as those of the paretic cortex, are
beyond a question cleared away in the progress of the affection.
Reference to the paradigm case (1) will show the type of our argument.
There is no manner of doubt that in this paradigm case almost every
portion of the nervous system had been sometime swept by spirochetosis
and many of its small vessel sheaths stuffed with chronic inflammatory
products. As for paretic neurosyphilis itself, a great many of its most
striking clinical phenomena, such as loss of memory and disorientation,
as well as great degrees of apparent dementia, are found virtually as
often in cases with very slight anatomical changes as in cases with
marked cortical devastation. The inference is plain, that these
phenomena are to a degree functional rather than structural.
In brief, we conclude not only from therapeutic experience but also on
_a priori_ grounds that the histological conditions in paretic
neurosyphilis are not entirely hopeless, and certainly not more hopeless
than conditions in many chronic diseases outside the nervous system.
Accordingly, we plead for a temperate optimism as to therapeutic results
in general paresis.
A fifth group of neurosyphilitic cases bulking rather largely in
textbooks of pathology is the group of the =gummata=. For a variety of
reasons (therapeutic and otherwise) the actual number of gummata of the
nervous system available for clinical or even for anatomical study is
much smaller than the books might lead one to infer.
The sixth and last of the main groups of neurosyphilitic diseases is
that of the =juvenile forms=, among which we find not only diffuse forms
without a special and well-defined course, but also characteristic
examples of paretic and tabetic neurosyphilis. The distinction of a
juvenile or congenital group of neurosyphilitics is, on theoretical
grounds, perhaps hardly defensible. On practical grounds, however, the
juvenile neurosyphilitics do form a group having special relations to
feeblemindedness, epilepsy and the like.
We must be clearly understood as to the rough, six-unit classification
just given. It is practical merely. For comparison we have given in
other charts more expanded lists of the diagnostic entities in
neurosyphilis among which that of Head and Fearnsides is of special
interest, see Chart 2, page 21.
We shall now proceed to a brief analysis of the findings in our chosen
series of 137 cases. We shall not reproduce the case headings of these
cases, but expand their statements where necessary and tie them together
so far as possible into a reasonable and systematic statement of the
situation in neurosyphilis. The footnotes will contain references to
other cases in which identical points are illustrated as in the leading
cases. The leading cases will in all instances be placed first in the
footnotes.
The paradigm[32] shows meningeal, vascular and parenchymatous lesions
and thus illustrates our definition of the term DIFFUSE which means
precisely meningeal, vascular and parenchymatous. The meningeal lesions
gave rise to two prominent sets of lesions, first, the marked tabetic
lesions of the spinal cord (due to the spinal root neuritis incidental
to the spinal meningeal inflammation), secondly, the characteristic
asymmetrical and focal atrophy of cranial nerves incidental to a now
largely extinct meningeal process at the base of the brain. The vascular
lesions are responsible for another important and characteristic factor
in the case, namely, the bilateral pyramidal tract sclerosis; the
bilateral cysts of softening of the corpora striata are characteristic
effects of old syphilitic cerebral thromboses. The parenchymatous
disease in our paradigm is everywhere obvious, less so perhaps in the
cortex itself than elsewhere, although here also evident in the shape of
lesions suggesting an early phase of tissue atrophy.
The paradigm is of interest in demonstrating what in broad lines must be
taken as an ascending disease proceeding not only from spinal cord to
encephalon but also traceable as proceeding from lower parts of the
spinal cord to upper parts thereof and from the lower encephalon to the
higher structures of the cerebral cortex itself.
The paradigm insistently calls attention to the advantage of persistent
therapy not only in its display of remarkable successive recoveries from
permanent looking symptoms but also histologically from the remnants of
inflammatory process to be found in an otherwise almost wholly
dismantled nervous system with extinct lesions.
TABETIC NEUROSYPHILIS[33] (“tabes dorsalis”), of course, often proceeds
to death without special complications of syphilitic nature. We have
chosen a case, however, to demonstrate a terminal complication with
vascular insult. Incidentally the case shows another complication
inasmuch as the cause of death was rupture of aortic aneurysm. It is
important to bear in mind these complications in tabes dorsalis which go
to prove that the spirochetosis of tabetic neurosyphilis is not limited
to the region of the spinal roots or to the spinal region in general.
Tabetic neurosyphilis is apt to be only a part of a total picture of
neurosyphilis just as neurosyphilis itself is only a part of the general
syphilitic process.
Our case of PARETIC NEUROSYPHILIS[34] (“general paresis”) is a
characteristic one in duration (three years and three months). The
aortic sclerosis almost constantly found in neurosyphilis and especially
in paretic neurosyphilis is here also shown. The spinal cord showed
lesions which are also almost always found in paretic neurosyphilis. The
characteristic frontal emphasis of the atrophic and indurative lesions
is shown. There is also a display of gross changes in the pia mater. The
characteristic so-called granular ependymitis or sanding of the
ventricular surface is shown. The case is distinguishable from the
paradigm in not showing the effects of vascular insults in the shape of
cysts of softening. The cerebellar sclerosis of the case is fairly
characteristic of paretic cases. There is even a suggestion of atrophy
in the temporal region suggesting the so-called Lissauer’s paresis.
Clinically the case belongs in the classical grandiose group of paretics
(“O. K. No. 1 superfine”).
VASCULAR NEUROSYPHILIS[35] is illustrated in a fourth autopsied case. It
may be noted that the pia mater in this case is practically normal. The
tissues outside the area of softening due to the syphilitic thrombosis
of nutrient vessels are practically normal. The case was one of almost
complete sensory aphasia with word-deafness. The clinical picture is
accordingly quite distinct from those of the paradigm (1) and of the
case of general paresis (3) just discussed.
JUVENILE PARESIS[36] is illustrated by a case with exceedingly extensive
lesions, largely meningeal and parenchymatous. The cerebral lesions are
atypical since in places they suggest the tuberous sclerosis of
Bourneville. The brain atrophy is extreme (965 grams) and it is possible
that this apparent brain atrophy was in part hypoplasia, since the
spirochetosis of this case was doubtless congenital. However, clinically
the patient was fairly normal up to the age of 18.
A case of so-called SYPHILITIC EXTRAOCULAR PALSY[37] demonstrates a
characteristic meningeal process more extensive than the clinical
symptoms would have indicated. In fact, focal clinical nerve palsies are
as a rule, if not constantly, partial phenomena of a far more extensive
process of neurosyphilis. They are far more limited clinically than
anatomically and histologically. It seems at first sight improper to
term them cases of diffuse neurosyphilis in view of their clinical
focality, yet they are best described as partial cases of diffuse
neurosyphilis.
A case of GUMMA[38] of the left HEMISPHERE is presented which appears to
have led to death in about four years from onset. This case, like many
others, is not an example of purely focalized syphilitic process
inasmuch as cysts of softening indicating slight vascular insults are
present elsewhere (pons). There is also a degree of leptomeningitis,
particularly basal.
Our discussion of the nature and forms of neurosyphilis is completed by
a rare case probably belonging in the so-called _cervical hypertrophic
meningitis of Charcot_ but actually due to a GUMMA OF THE SPINAL
MENINGES.[39] The importance of therapeutic optimism is emphasized in
this case as in the paradigm. Theoretically the meningeal inflammation
of neurosyphilis ought to be almost entirely if not entirely removed by
therapy, and these two cases, like several others in the series, seem to
illustrate this possibility.
Neurosyphilis sometimes receives the clinical diagnosis neurasthenia
simply through omission to apply proved diagnostic methods. An instance
is given in which the PARETIC form of NEUROSYPHILIS (“general paresis”)
received the diagnosis _neurasthenia_[40] for a period of five years, at
any time during which period it would doubtless have been possible to
render the correct diagnosis and apply treatment.
Neurosyphilis may imitate not only the psychoneuroses but also the
psychoses themselves. We present a case of an architect, which looked
almost precisely like _manic-depressive psychosis_[41] and had a history
of attacks, but in which the positive serum W. R. led (in accordance
with hospital rules) to an examination of the spinal fluid. The spinal
fluid tests proved the case to be one of PARETIC NEUROSYPHILIS.
However, a positive serum W. R., even when associated with mental
symptoms, and when those mental symptoms include grandiosity, does not
prove the existence of neurosyphilis either in its paretic or
non-paretic form. Our instance seems to be one of MANIC-DEPRESSIVE
PSYCHOSIS.[42] The spinal fluid tests were entirely negative. The course
of the disease was also that of manic-depressive psychosis. In the
absence of positive spinal fluid tests, the diagnosis neurosyphilis was
excluded.
Neurosyphilis and even PARETIC NEUROSYPHILIS may result in symptoms that
would ordinarily lead to the diagnosis _dementia praecox_.[43]
It is important not to rule out neurosyphilis on the ground of a
_negative serum_ W. R. The fluid W. R. may turn out positive. We present
a case (of a salesman)[44] in which the serum W. R. was repeatedly
negative (even salvarsan did not act provocatively) yet the spinal fluid
W. R. proved positive. The case was clinically one of classical PARETIC
NEUROSYPHILIS (“general paresis”). It is a good rule to proceed to
lumbar puncture, even when the serum W. R. is negative, if there are
suspicious symptoms (e.g., speech defect and memory impairment,
grandiosity) or signs (e.g., marked reflex disorder, especially
pupillary disorder).
DIFFUSE NEUROSYPHILIS was above defined as
“meningovasculoparenchymatous.” This disease is typically associated
with six positive tests (positive serum W. R., positive fluid W. R.,
pleocytosis, gold sol reaction, positive globulin reaction and excess
albumin). One or more and frequently several of these six tests are
likely to run mild in diffuse neurosyphilis; that is to say, these tests
are apt to run milder than the identical tests in paretic neurosyphilis
(“general paresis”). The clinical course of the diffuse, and especially
the meningovascular cases, is likely to be protracted. The prognosis as
to life is good, barring fatal vascular insults. The illustrative
case[45] was a case with slow course. There was a series of attacks
followed by a paralytic stroke, a finding highly typical of the diffuse
form of neurosyphilis. The spinal fluid reactions were mild, suitable to
the general principle above stated.
These tests are likely to run stronger, as above stated, in paretic
neurosyphilis (“general paresis”), than in the diffuse form. In
particular, the gold sol reaction is likely to be shown in what is
termed “paretic” form rather than in what is termed “syphilitic” form.
The clinical course of PARETIC NEUROSYPHILIS is likely to be brief. A
characteristic case[46] with very heavy globulin and albumin tests is
presented.
TABOPARETIC NEUROSYPHILIS[47] (“taboparesis”) is clinically a
combination of the symptoms of tabetic (“tabes dorsalis”) and those of
paretic neurosyphilis (“general paresis”). First comes the tabes
dorsalis lasting often for many years. Afterward follows a
characteristic general paresis. The ultimate paretic picture is likely
to retain, however, various characteristics of tabes. The laboratory
tests in the paretic phase of taboparesis are characteristic of general
paresis and not of tabes dorsalis. The prognosis after the paretic phase
has arrived is that of general paresis.
The diagnosis of the neurosyphilitic forms would be easy if these
principles were always carried out to the letter. The important fact is
as follows: diffuse (that is, meningovasculoparenchymatous
neurosyphilis) may look like paretic neurosyphilis (“general
paresis”)[48] at certain periods of the clinical and laboratory
examination. This fact is of obvious importance. The general prognosis
of diffuse neurosyphilis is regarded as good _quoad vitam_. The general
prognosis of paresis is bad. If, however, the differential diagnosis
cannot be rendered at particular phases of a given case, then no safe
prognosis can be offered in the individual case. In particular no
prognosis affecting the administration or non-administration of modern
systematic treatment can or should be offered in these doubtful phases.
It is not always safe to exclude neurosyphilis even when the _fluid_ W.
R. is _negative_.[49] Particularly in vascular neurosyphilis the fluid
W. R. and even all the other laboratory signs in the spinal fluid may
sometimes be negative. A positive serum W. R. yields the correct pointer
to diagnosis. Of course, also in many cases of vascular neurosyphilis
one or more of the laboratory signs may be suggestive even when the
fluid W. R. is negative. Theoretically there may be cases in which all
the six tests are negative and yet the diagnosis neurosyphilis be the
correct one.
A clinically important sign in neurosyphilis is the so-called
_seizures_. These occur both in DIFFUSE NON-PARETIC NEUROSYPHILIS[50]
and in PARETIC NEUROSYPHILIS.[51]
_Aphasia_ is likewise a symptom in both these forms of neurosyphilis,
namely, in the DIFFUSE non-paretic[52] and in the PARETIC form.[53]
The literature contains reference not only to seizures and aphasia as
characteristically paretic but also to _remissions_. Remissions like
seizures and aphasia are found in both the PARETIC[54] and NON-PARETIC
forms of neurosyphilis.[55] They have important bearings on prognosis in
all forms of neurosyphilis and are of especial significance in the
evaluation of treatment. (Remissions coincident with apparent cure.)
So far we have been dealing with cases of neurosyphilis in which there
was no doubt of the existence of mental symptoms. There are cases,
however, in which although the laboratory signs of neurosyphilis exist,
proving beyond doubt the existence of a chronic inflammatory reaction
and allied pathological conditions in the cerebrospinal axis, there are
no mental symptoms of neurosyphilis. We have called some of these cases
PARESIS SINE PARESI[56] and present examples.
To illustrate complications we give a case of PARETIC NEUROSYPHILIS with
autopsy in which there were ante mortem signs of HERPES ZOSTER[57] or,
at all events, of a skin eruption limited to the area of a thoracic
nerve.
A case of GUMMA of the brain[58] in which decompression was warranted
and performed is presented. The fluid W. R., as in many such cases, was
negative; serum positive.
A case of CRANIAL NEUROSYPHILIS (extraocular palsy[59] without mental
symptoms) showed a positive Wassermann serum test and a negative spinal
fluid.
The laboratory reactions in TABETIC NEUROSYPHILIS[60] (“tabes dorsalis”)
run somewhat like those of diffuse non-paretic neurosyphilis and are
accordingly milder than those of paretic neurosyphilis. The fluid W. R.
and the gold sol reaction in particular are apt to run mild. The
clinical course of tabes dorsalis is well known to be protracted and the
prognosis _quoad vitam_ is good except that we must always bear in mind
the possibility of vascular insults and complications of a syphilitic
origin in the rest of the body.
It is important to remember that TABETIC NEUROSYPHILIS is often quite
atypical[61] clinically and may even show no single symptom warranting
the old clinical name locomotor ataxia.
There are even cases in which the name tabes dorsalis is not warranted
in view of the fact that the lesions are not low in the cord but are
higher up (TABES CERVICALIS[62]).
A rare form of neurosyphilis is ERB’S SYPHILITIC SPASTIC PARAPLEGIA[63]
against which one needs to consider a number of non-syphilitic spinal
cord diseases. Our case showed a weakly positive serum W. R., a negative
fluid W. R., and the other tests of the spinal fluid were moderately
positive.
SYPHILITIC MUSCULAR ATROPHY[64] is classified by Head and Fearnsides
both in their meningovascular group and in their group of the so-called
syphilis centralis. Our case affecting in large part the small muscles
of the hands in a teamster, may be due either to spinal parenchymal
lesions or to root neuritis or to both.
It is a little extraordinary and very important that the _laboratory
signs_ are apt to be positive even in the SECONDARY period of SYPHILIS.
Perhaps a third of all cases of syphilis in the secondaries would, if
tested, yield positives precisely like those of full-blown paretic or
diffuse neurosyphilis. Strangely enough, these signs may occur without
clinical symptoms. The illustrative case,[65] a mechanic, yielded
various mental symptoms. The cases of secondary syphilis with laboratory
signs of neurosyphilis but without clinical symptoms are of the greatest
theoretical importance in relation to the problem above mentioned of
_paresis sine paresi_. It may well be inquired whether in some instances
the neurosyphilis of the secondaries does not persist until the
exhibition of mental or physical symptoms of neurosyphilis years later.
It must be remembered that this conception is hardly more than a
hypothesis at the present time. That such signs of chronic inflammation
could exist without symptoms is not so surprising when one thinks of the
startling immediate improvement seen after treatment or even in
remissions without treatment. One is reminded of the crisis in pneumonia
wherein clinical improvement takes place entirely independent of the
mechanical conditions in the lung which just after the crisis remain as
suppurative as before.
The diagnosis of JUVENILE NEUROSYPHILIS is made upon the same lines as
that of neurosyphilis in the adult. We present two cases, one with optic
atrophy[66] and the other with signs of congenital syphilis antedating
the symptoms of paresis.[67]
Congenital syphilis is also apparently capable of producing a simple
form of FEEBLEMINDEDNESS,[68] that is to say, a form of disease
non-paretic, non-tabetic, and without special tendency to vascular
insults.
We present a case of JUVENILE TABETIC NEUROSYPHILIS (“juvenile
tabes”).[69] The tests were all positive.
The line of separation between typical and atypical cases of
neurosyphilis is vague and indistinct and some of the cases classified
by us amongst puzzles perhaps belong under systematic diagnosis and vice
versa. The section on PUZZLES AND ERRORS in the diagnosis of
neurosyphilis is introduced by six cases of error in the diagnosis of
the paretic form of neurosyphilis.[70] These errors were made known by
autopsy. Aside from the sixth case, whose etiology must remain in doubt
and which was a unique case of PERIVASCULAR GLIOSIS, there is ground for
the belief that the other five cases in this Danvers Hospital study of
diagnostic errors were perhaps actually syphilitic though not of the
paretic form of neurosyphilis. At all events, the brain tissues in these
cases failed to show the plasma cell deposits which are characteristic
in the sheaths of the intracortical vessels in paretic neurosyphilis.
A case illustrates the complication of TABES by _arteriosclerotic
symptoms_, in which case the arteriosclerosis may naturally have been of
syphilitic origin. Two cases especially illustrate the possibility of
confusing the ataxia of general paresis with CEREBELLAR ATAXIA. These
cases showed lesions of the cerebellar structures, notably of the
dentate nucleus. No one can read these cases or any of the autopsied
cases in our series, without perceiving how fundamental and even
critical is the demand for autopsies in fatal cases of neurosyphilis.
The practitioner who can secure an autopsy in a fatal case of
neurosyphilis and have the tissues worked up by approved
neuropathological methods is almost bound to add his bit to neurological
theory. Even cases of classical tabes dorsalis are often signally
important to the theorist on account of the relations of the neural to
the non-neural complications.
We then proceed to a group of cases without special order in which a
variety of diagnostic questions arose.
A case of questionable neurosyphilis in the secondary stage of syphilis
brings up the problems of syphilitic _neurasthenia_.[71]
Syphilis may act as _agent provocateur_ of HYSTERIA as Charcot
insisted.[72]
A case illustrative of difficulties in diagnosis between neurosyphilis
and manic-depressive psychosis follows.[73]
A case for diagnosis is given which shows that errors in the diagnosis
of neurosyphilis are entirely possible even when abundant clinical and
laboratory data are available. A case with a weakly positive Wassermann
reaction in the spinal fluid finally turned out to be one of BRAIN
TUMOR.[74]
Some questions as to the diagnosis of NEUROSYPHILIS _versus Idiopathic
Epilepsy_ are brought up by a case in which phenomena of paresis seemed
to have occurred very early, about two years after the initial
syphilitic infection.[75]
A case of PARETIC NEUROSYPHILIS is offered in which _hemiplegia_ and
_hemitremor_ strongly suggested _vascular_ lesions; but the autopsy
showed no coarse lesions and merely confirmed the diagnosis paresis
microscopically.[76]
An autopsied case of PARETIC NEUROSYPHILIS is given, in which the pupils
persisted in reacting normally. Herpes zoster-like lesions in life
yielded no special signs at autopsy (all root-ganglia looked alike above
and below zone of “shingles.”)[77]
An example of NEUROSYPHILIS, probably PARETIC, yielded symptoms highly
suggestive of _manic-depressive psychosis_.[78] An interesting feature
in this case was the birth of a healthy child nine months after the
onset of the psychotic attack.
An example of _exophthalmic goitre_[79] following the acquisition of
SYPHILIS showed at autopsy a heavy scarring of the optic thalamus and
unilaterally atrophic process in the cerebral cortex.
We come to some questions concerning the _Argyll-Robertson pupil_. It is
agreed on all hands that the Argyll-Robertson pupil is characteristic of
the paretic and tabetic forms, but the sign occurs also in other
neurosyphilitic conditions;[80] in fact the sign does not necessarily
indicate neurosyphilis as an instance of PINEAL TUMOR demonstrates.[81]
The question raised above as to the possibility that neurosyphilis may
exist in the absence of positive findings in the spinal fluid is
illustrated in a man, a mechanic, who claimed syphilitic infection and
showed an _Argyll-Robertson pupil_ on one side.[82] The serum W. R. was
positive; the _fluid tests_ were _negative_.
An extraordinary case is given in some detail in which NEUROSYPHILIS in
the form termed DISSEMINATED ENCEPHALITIS[83] proved fatal within seven
months of the initial infection.
We have frequently mentioned the classical assumption that paretic
neurosyphilis (“general paresis”) is a fatal disease. Some have
suggested that there is another form clinically almost identical with
general paresis except that it pursues a long course and the suggestion
has been made that these cases be termed _pseudoparesis_.[84] We are of
the opinion that this term should be dropped and advocate the use of the
word pseudoparesis only for non-syphilitic disease looking like paresis,
such as alcoholic pseudoparesis and the like.
The question whether there is a form of mental disease SYPHILITIC
PARANOIA[85] is raised by a case with auditory hallucinations, ideas of
persecution and attacks of excitement. The diagnosis of alcoholic
hallucinosis was actually made although there is no proof that the
patient ever drank alcohol.
Alcohol may cause symptoms identical with those of paretic
neurosyphilis, including seizures, Argyll-Robertson pupils, speech
defect and mental symptoms. The differentiation is readily made by the
negative laboratory findings. An illustration is given in our case of
the alcoholic teamster. Cases such as this bear the name ALCOHOLIC
PSEUDOPARESIS.[86]
However, when the clinical picture is the same as in the case of our
teamster, the alcohol may only be a complicating factor in
neurosyphilis, as shown by our next case of the _alcoholic_ steamfitter
who in fact was shown to have NEUROSYPHILIS.[87]
Sometimes cases of apparently frank _alcoholism_, even with apparently
characteristic delirium tremens and neuritis, prove to be essentially
neurosyphilitic.[88] On the other hand, true combinations of ALCOHOLISM
and NEUROSYPHILIS occur which it would be proper to classify under
either heading and in which therapy must take serious account of both
conditions.[89]
As above stated, we elect to use the term pseudoparesis only for
non-syphilitic cases. There are other forms of pseudoparesis than
alcoholic pseudoparesis. The question of _Diabetic Pseudoparesis_ is
raised by an exceedingly complicated case of which our best
interpretation is that the patient, a proved syphilitic (with syphilitic
osteomyelitis (?)), a huge doorkeeper, was perhaps suffering from an old
SYPHILITIC scarring of the PITUITARY body.[90] Neither this case nor a
second case, one of PARETIC NEUROSYPHILIS with _glycosuria_ is actually
entitled to the diagnosis diabetic pseudoparesis. The second case of
paretic neurosyphilis with glycosuria brings up some unanswerable
questions as to the pancreatic or basal meningitic or other origin for
the glycosuria.[91]
_Isolated symptoms_ are often presented by neurosyphilitics (e.g.,
hemianopsia);[92] but we tend to regard these cases as due to focal
lesions that are merely part and parcel of DIFFUSE LESIONS.
A neurosyphilitic case (a steward) with the rather unusual complication
(for our northern region) of severe MALARIA producing cerebral
thrombosis is reported.[93]
The diagnosis _Dementia Praecox_[94] was actually made in the case of a
young school-teacher in whom the laboratory findings proved conclusively
that the condition was one of NEUROSYPHILIS. The gold sol reaction in
this case was mild. The chief lesion at autopsy was a fresh looking,
gelatinous pial exudate over the spinal cord which turned out to contain
an almost pure display of very numerous plasma cells.
The question of LUES MALIGNA[95] is brought up in a rectifier of spirits
in whom the characteristic tremendous destruction of tissue, toxemia and
failure to react to antisyphilitic treatment were illustrated. Moreover,
this case had a trauma (cautery) to the tonsil, as in other cases of
lues maligna.
A case somewhat suggestive of _brain tumor_, of _neurosyphilis_ and of
_multiple sclerosis_[96] turned out to be MULTIPLE SCLEROSIS (the fluid
showed a pleocytosis and a moderate amount of globulin with a paretic
type of gold sol reaction).
As a foil to this case that we regard as multiple sclerosis, we present
a second case with nystagmus, optic atrophy and spasticity in which the
suspicion of _multiple sclerosis_ might well be raised but which the
tests demonstrated to be NEUROSYPHILITIC.[97]
An even stranger imitation of well-defined non-syphilitic entities was
presented by a case apparently of _Huntington’s chorea_[98] (except for
absence of the hereditary taint) which case, however, proved to the
surprise of all diagnosticians to be one of NEUROSYPHILIS.
Frequent errors of diagnosis must occur in the field of the senile
psychoses. We present a case that would at first blush warrant the
diagnosis of _senile arteriosclerotic psychosis_[99] in a sea captain of
75 years (wife dead 15 years before of general paresis) who turned out
to be a characteristic case from the laboratory standpoint of
NEUROSYPHILIS.
The Protean nature of the symptomatology of neurosyphilis is
sufficiently established. Still, a case that might fit into textbooks
concerning DISSOCIATION OF PERSONALITY[100] is certainly a clinical
oddity, as illustrated by a fugacious musician.
A case with strong suspicions of _neurosyphilis_ of _tabetic_ type
turned out to be more probably one of neural complications in PERNICIOUS
ANEMIA.[101]
NEUROSYPHILIS IN JUVENILES presents puzzling conditions.
One case was marked clinically by _attacks of excitement_.[102] It is
impossible to place this case among the main groups of juvenile
neurosyphilis.
Another case of FEEBLEMINDEDNESS,[103] also NEUROSYPHILITIC in origin,
presented physical symptoms and laboratory signs of paretic
neurosyphilis; yet this case had been considered one of _simple
feeblemindedness_.
A case apparently of JUVENILE PARETIC NEUROSYPHILIS in a 15 year old boy
presented the rather unusual complication of shocks with
quadriplegia,[104] a _vascular complication_ not usually expected in the
paretic type of neurosyphilis in adults.
Epileptic phenomena[105] are rare as the effect of JUVENILE
NEUROSYPHILIS, but occur as demonstrated in a case which slipshod
methods of diagnosis might well have regarded as one of _idiopathic
epilepsy_.
A case of JUVENILE PARETIC NEUROSYPHILIS with the complication of
ADDISON’S DISEASE[106] is given (autopsy confirmation).
The puzzle in diagnosis offered by syphilis in the secondary stage[107]
is illustrated by a case which showed the characteristic NEUROSYPHILITIC
complications of the SECONDARY STAGE of syphilis. This patient may well
have been a moron at the outset and exhibited some reactions (refusal to
talk) explicable on the basis of feeblemindedness. She was a
neurosyphilitic only in the sense of the neural complication that we
find in the secondary stage of syphilis. As stated above, we do not yet
know what the fate of these neural complications of secondary syphilis
is to be. The frequency of this finding in secondary syphilis is
probably too great to warrant the hypothesis that it must always go on
to a chronic neurosyphilis; but we certainly are warranted in regarding
these cases as potential chronic neurosyphilitics.
A case of TABOPARETIC NEUROSYPHILIS in which the heavy exudate
characteristic of paresis became a soil for a growth of the typhoid
bacillus is presented with autopsy.[108] This fatality with TYPHOID
MENINGITIS is merely a concrete example of the many complications which
syphilitics and especially neurosyphilitics have to sustain.
The case series then goes on to illustrate, though quite inadequately, a
variety of MEDICOLEGAL AND SOCIAL complications of neurosyphilis. It is
well known that many social complications with grave moral, economic and
even political difficulties occur.
Our series starts with a “public character”[109] whose eloquence and
reformatory efforts led to a considerable notoriety. The autopsy in this
case showed singularly few lesions despite the fact that the case was
microscopically one of wholly characteristic PARETIC NEUROSYPHILIS. The
question might arise how far we are entitled to correlate the
reformatory efforts of this always eccentric character with syphilis.
The man himself a physician, was aware of the doubt which his
Argyll-Robertson pupils threw upon his medical situation. He explained
them on the basis of an old smallpox! We are inclined to think that the
whole of this man’s life, from his giving up of medical practice to live
as a kind of literary and political hack, was due to subtle changes of
neurosyphilitic origin. The fact that there was a certain delinquent
streak in the man is not inconsistent with this idea. Interestingly
enough, a fall on the ice in the man’s 61st year actually started up the
fatal process, a condition of affairs amply illustrated in cases of
neurosyphilis, brought out by trauma that come to the attention of the
Industrial Accident Board in connection with claims for compensation.
A case of sudden _grandiosity_[110] illustrates an episode of
NEUROSYPHILITIC origin. Such a person might well be regarded by the lay
newspaper reader as a crank or a grafter but the neurosyphilitic
possibility should always be entertained in cases of this order.
As against the social difficulties that look in the direction of the
classical paretic grandeur, we present a case of apparent _suicidal
attempt_ by gas, which attempt was followed by a period of amnesia that,
taking into account the laboratory findings, was probably
NEUROSYPHILITIC.[111]
Vistas of extraordinary interest are opened out by studies of the
relation of neurosyphilis to _delinquency_. The case of the psychopathic
reformer (Case 83) above mentioned was one in which the delinquency may
possibly have been related to acquired syphilis. We present also a case
of juvenile neurosyphilis, a young man of reform school type[112] in
which JUVENILE PARETIC NEUROSYPHILIS was established. This patient, in
fact, deteriorated very rapidly to a condition of considerable dementia
a few months after the diagnosis was established.
A striking case of so-called DEFECTIVE DELINQUENCY is presented, an
alcoholic prostitute of the reformatory group.[113] The NEUROSYPHILIS in
this case was a complication rather than an original factor in the
delinquency.
One case of PARESIS SINE PARESI was that of an habitual criminal[114]
and forger who, without showing mental or physical symptoms of
neurosyphilis, yielded the laboratory signs of paretic neurosyphilis.
Again, as in the case of the prostitute just mentioned, the
CRIMINALITY[115] seems to have antedated the neurosyphilis and even to
have been hereditary.
By way of introducing the next group of Industrial Accident Board cases,
we present a case of JUVENILE PARESIS with initial TRAUM.
The Industrial Board group is of note in that the signs of the traumatic
form[116] of paretic neurosyphilis do not occur immediately upon the
accident. Some time elapses in which the physical, chemical or
parasitological changes have time to work themselves out in the injured
tissues. Many hypotheses may be raised as to the reason why a trauma
lights up a syphilitic process. Of course, =false claims=[117] =may be
made for compensation by neurosyphilitics= in whom the symptoms were
already in existence before the accident and in whom they may not even
be markedly exacerbated by the accident. The false claimants can
probably not readily frame a story which the expert psychiatrist cannot
discredit if he is allowed to perform laboratory tests and give the
patient the benefit of thorough examination. However, some cases of
established PARETIC NEUROSYPHILIS are perhaps truly subject to
_exacerbations_[118] of the clinical process and it may well be held
that such exacerbations warrant partial compensation.
The fact that a trauma may light up a syphilitic process is illustrated
in a case that came to the Psychopathic Hospital, in which a SYPHILITIC
LESION developed in the skull AT THE SITE OF SKULL INJURY.[119]
A case of OCCUPATION-NEUROSIS[120] that might be interpreted as a
_syphilitic neuritis_ is presented. The case is still in doubt as to its
scientific evaluation.
The workmen’s compensation group of syphilitic cases is of extraordinary
general interest since it indicates that employers may well be on the
lookout not to employ known syphilitics unless fortified by special
insurance arrangements. Whether in future employers may desire =to
employ only W. R. negative workmen= is one of the highly complicated
questions _re_ workmen’s compensation and health insurance.
But the problems of neurosyphilis are not merely medicolegal and broadly
public or social. The most appealing difficulties lodge within the bosom
of the family. Now and then a case of INCOMPATIBILITY OF TEMPERAMENT,
perhaps complicated by _alcoholism_, occurs which tests prove to be
NEUROSYPHILITIC.[121]
Special attention should be drawn to a certain NEUROSYPHILITIC
FAMILY[122] in which both parents and five children showed a variety of
syphilitic diseases, including syphilis without apparent neural
complications, paretic neurosyphilis, juvenile paresis, aortic aneurysm,
achondroplasia and caries of the spine, and an as yet indefinite
neurosis. There was a sixth child that died shortly after birth, as well
as three stillborn.
One =cannot conclude= from the normal[123] look of a neurosyphilitic’s
family =that the normal-looking members are not syphilitic=, as
illustrated by the family of our draughtsman.
The most =intricate social complications= may arise. We present a case
of a syphilitic man (a well-to-do merchant) who was apparently being
goaded into a second marriage[124] because he was continually being
charged with having caused his first wife’s death. This he had actually
done in a certain sense because his wife had died of general paresis,
having contracted syphilis from him.
In the fifth section on THERAPY, we have attempted to outline some of
the principles and problems that arise in the treatment of
neurosyphilis. Enough has probably been said concerning the attitude of
optimism or pessimistic nihilism that may be adopted toward the whole
subject. It must be borne in mind, however, that a great deal of the
work on treatment of neurosyphilis is still in the experimental stage.
As a rule, each case must be considered separately and individually and
the prognosis can be made satisfactorily only after treatment has been
given. This section contains a group of cases that have been treated
rather intensively and the results of this treatment are indicated. The
section is introduced by _five untreated cases_, the brains and cords of
which have been studied post mortem. These illustrate the pathological
conditions which we have to meet, and from these examples we can draw
the theoretical conclusion that some cases are beyond the aid of therapy
on account of the brain destruction. Others, in which the symptomatology
bespeaks just as grave a situation, turn out on autopsy to have very
little actual damage to the brain tissues and therefore should
theoretically at any rate be amenable to antisyphilitic therapy.
In order to get any adequate conception of the possibilities of
therapeutic results in cases of neurosyphilis, one must consider the
pathological changes that occur and how far these changes are reparable.
In cases in which the destruction of tissue is marked, it is, of course,
out of the question to expect to get any marked clinical improvement. A
case of spastic hemiplegia[125] in paretic neurosyphilis is given with
the autopsy findings as an illustration of irreparable damage that may
occur to the parenchymatous structure, thus precluding any chance of
functional recovery.
On the other hand, there is a group of cases in which the symptoms may
be exceedingly severe and yet the actual destruction of tissue be almost
nil. This point is illustrated by a case[126] in which _total duration
of symptoms_ terminating in death was _only 22 days_. At autopsy there
was very little in the way of macroscopical lesions, and microscopically
there was no marked evidence of destruction in the parenchymatous
tissue. The lesions were represented chiefly by perivascular
infiltration. According to all our modern ideas, this type of reaction
is resolvable under antisyphilitic treatment. Though this case was one
of very short duration, similar pathological pictures may be obtained in
cases of considerably longer standing. It is also of great importance to
remember that symptomatically such a case may be in no way distinguished
from a case with marked atrophy.
Another autopsied case is given which shows an exceedingly =marked
meningitis=.[127] The meningitic processes according to the literature
and experience react very readily to antisyphilitic treatment in the
form either of mercury and iodid or in combination with salvarsan. The
lesion here present would probably have improved had intensive treatment
been given. Clinically the diagnosis of general paresis was made and, as
has been the rule in the past, treatment was not given on the ground
that it had no value in paresis. While this is an extreme case of
meningitis, it is to be remembered that the vast majority of cases of
paretic neurosyphilis show some degree of meningitis. Just as in the
marked meningitis of the diffuse neurosyphilis, so with the meningitis
of the paretic form, improvement is expected under treatment. As a part
or even the whole of the symptomatology in a given case may be due to
this meningitic process, we have reason occasionally to expect marked
improvement as the result of antisyphilitic treatment.
As a contrast to this case with marked meningitis, another case of
=marked atrophy=[128] is given. Here the atrophy was very perceptible on
macroscopical examination and the mere view of the brain at once
indicated that in such a case important results from treatment were not
to be expected.
The =topographical variation= of the lesions in neurosyphilis must be
remembered when treatment is to be instituted. Thus very marked lesions
may exist in portions of the brain which do not give any very definite
localizing symptoms. As a result, one may be led to believe from
clinical evidence that the case is a very mild one though the lesions
may really be very extensive. The topographical distribution must,
therefore, be taken into consideration in trying to estimate the damage
done. This point of topographical distribution of the lesions is
illustrated by a case.[129]
It has been generally recognized that =clinical improvement=, if not
cure, may be =readily obtained in the group of diffuse neurosyphilis=,
i.e., so-called cerebral and cerebrospinal forms of syphilis. These are
cases in which the parenchyma is very slightly, if at all, affected and
in which the lesion is chiefly in the meninges and blood vessels,
irritative rather than degenerative. A case[130] is given to illustrate
this point. In our experience systematic intravenous salvarsan therapy
associated with mercury and iodid gives remarkably good results in the
vast majority of this group of cases.
It is generally conceded that antisyphilitic treatment, particularly
salvarsan, has a very satisfactory result applied to diffuse
neurosyphilis. But the same good results may be obtained in cases which
are not so typically of the diffuse type. An illustration is given in
the case of a machinist in which the diagnosis was in doubt between
paretic, tabetic or diffuse neurosyphilis.[131] The result of treatment
was as satisfactory as could be expected in any type of neurosyphilis
and this in a case of several years’ duration with Argyll-Robertson
pupils.
As a rule, the Argyll-Robertson pupil is taken as a grave omen for
treatment, an idea based upon a conception that the Argyll-Robertson
pupil so frequently represents the old so-called “parasyphilitic” cases,
which, in the past were taught as being incapable of improvement by the
ordinary antisyphilitic methods.
A second case[132] with Argyll-Robertson pupil shows again that the
=prognosis may be very good despite the Argyll-Robertson sign=.
But even in the diffuse neurosyphilis, the symptomatic results of
treatment may not be entirely happy. Under treatment it may be possible
to reduce the spinal fluid tests to negative without, however, as in the
case of our hemiplegic lady,[133] making the physical or mental symptoms
disappear. In other words, it may be possible to stop the active
progress of the disease without removing the symptoms.
One is always warned of the danger of intravenous salvarsan therapy in
hemiplegic cases due to arteriosclerotic conditions. While this warning
is well justified, it does not mean that the most intensive treatment is
contraindicated, as shown in the case of our hemiplegic machinist.[134]
Such may be given over long periods of time with the most satisfactory
results.
A case[135] is given which illustrates the value of antisyphilitic
treatment in cases showing symptoms of intracranial pressure due to
syphilitic disease. In the case of the woman which we cite, we believe
that the symptoms of intracranial pressure were probably due to a
gummatous new growth, although it is possible that they were due to a
marked meningitic process. However, the results of a limited amount of
antisyphilitic treatment in this case were very brilliant. Similar
results may often be obtained in gumma of the brain. This is not always
true, however, and it may become necessary to use surgical procedure in
order rapidly to overcome the effects of intracranial pressure.
While it has always been conceded that treatment would greatly help
cases of diffuse and vascular neurosyphilis, the utmost pessimism has
existed concerning the results to be obtained by treatment in cases of
tabetic and paretic neurosyphilis. Only in the last five or six years,
due to the stimulus of Ehrlich’s discovery of salvarsan and the
introduction of the intraspinous methods of therapy, have intensive work
and study been given to the treatment of these cases. And though it has
been by no means settled in the minds of the various workers in this
field, as to what the ultimate results of such treatment will be and
though some do not believe that there is any good to be expected from
our present methods, still the majority of men who are treating these
cases systematically feel very much encouraged.
=At times very brilliant results= are to be obtained by intraspinous
treatment =in tabetic neurosyphilis= (“tabes dorsalis”). A very striking
illustration is given of a case of this sort in which the symptoms dated
only a few months but which had all the classical symptoms, signs and
laboratory tests. Five intraspinous injections of mercurialized serum
were sufficient to cause the disappearance of the subjective symptoms
and to reduce the spinal fluid test to negative.[136]
It must be emphasized that the best results in cases of tabetic
neurosyphilis are usually to be expected in cases in which the symptoms
are of short standing. Where the process is of long duration and much
destruction of spinal cord tissue has occurred, the best one can expect
is that the activity and progress may be halted. This is illustrated by
our case of a baker, 43 years of age, who had been suffering from the
symptoms of tabes for some years. Under treatment it was possible to get
an entirely negative serology of the blood and spinal fluid.[137]
Despite this evidence that the activity of syphilis had ceased, the
symptoms continued unabated. We are ready to believe, however, that much
good was accomplished. For the patient should not have any further
untoward developments or the appearance of any new symptoms. These,
without such treatment, might well be expected. At times excellent
clinical results are obtained in long-standing cases.
The results of treatment in paretic neurosyphilis (“general paresis”)
have been considered even less hopeful than in tabetic neurosyphilis
(“tabes dorsalis”); indeed, it has often been stated that the patients
are made worse by treatment. Recent work, however, supports a much more
optimistic viewpoint. We feel that =intensive treatment has been of the
greatest value in a number of cases of paretic neurosyphilis=. Two cases
are given which show the most satisfactory and brilliant results of
intensive intravenous salvarsan therapy in cases diagnosed as general
paresis. The first case, an excellent salesman, 46 years of age, with
most aggravated mental symptoms, recovered symptomatically and all his
tests were rendered negative.[138] He has now remained entirely well and
economically efficient for about two years without further treatment.
The other case,[139] a housewife, also with very marked symptoms
suggestive in all ways of general paresis, also recovered rapidly under
treatment and her tests became negative. Her remission has now lasted
for nearly three years without further treatment.
At times it is not possible to get the spinal fluid tests to become
negative in cases of paretic neurosyphilis under the most intensive
salvarsan therapy. In spite of this, the clinical condition of the
patient may improve so greatly that the patient can be considered
=clinically recovered=. An illustration is given of an undertaker[140]
who was brought from a condition of the greatest cachexia and mental
confusion to a condition of robust appearance and mental efficiency
under intravenous salvarsan therapy, in spite of the fact that his tests
were very slightly if at all reduced in intensity. He has been able to
resume his former occupation and his former life with great satisfaction
to himself and his family.
Improvement in paretic neurosyphilis under treatment is not to be
expected very early. =Two or three months of active treatment= may
elapse before one sees signs of improvement. Indeed, as illustrated by
our case of the shipping clerk, this improvement may begin to make its
appearance only after more than four months of intensive treatment
consisting of two injections of salvarsan per week.[141] In spite of the
long delay in this case, complete clinical recovery occurred and the
tests became almost negative at the end of a year of treatment.
It is not only in the central nervous system that the syphilitic process
may resist the most intensive treatment. In the case of the speculator,
a victim of paretic neurosyphilis, which we cite, a perennially
recurrent iritis appeared after several months of the most intensive
salvarsan treatment which was apparently sufficient to reduce the
symptoms of the paretic neurosyphilis,[142] but not of non-neural
syphilis.
We give the case of a charwoman having the diagnosis of paretic
neurosyphilis, who, under intensive treatment, made a symptomatic
recovery. The interesting point in her findings is that all the tests in
the spinal fluid became negative except the gold sol reaction which
remained of the “paretic” type.[143] There is no general rule as to the
reaction of the spinal fluid tests under treatment. At times one test is
the first to disappear under treatment; again it is another. We have
seen many cases in which the gold sol was the first test to become
negative and others, as the case given, in which it is the last to show
any change. As in our undertaker, symptomatic clinical improvement may
be practically complete without any change in the spinal fluid tests.
One must remember that it is the condition of the patient that is of
first importance; not so much the laboratory tests. Having shown the
clinical recoveries with the tests remaining positive, we now have to
report two cases in which there was =improvement= as shown =by the tests
but no clinical improvement=. The first patient, a bank teller[144] of
39 years, with a diagnosis of paretic neurosyphilis, received intensive
intravenous salvarsan for several months. Under this treatment all the
tests became negative except the gold sol which remained of the paretic
type. In spite of this, there was not the slightest improvement in his
mental condition.
The second case, a young man of 29 years in whom the symptoms of
neurosyphilis had recently appeared, under treatment showed a marked
diminution in the intensity of the spinal fluid tests, notwithstanding
which the patient became more and more demented and died after a series
of convulsions.[145]
Of course, good results indicated above in some of our cases of paretic
neurosyphilis are not to be expected in every case no matter how
intensive the treatment. We give a case of paretic neurosyphilis in
which the most intensive intravenous salvarsan therapy gave no
satisfactory results. This was followed by several intraventricular
injections of salvarsanized serum. The results of this combined
treatment, however, were still not satisfactory, and the patient
died.[146]
In order to emphasize as strongly as possible what we believe is a great
=advantage of systematic intensive treatment= for neurosyphilis, we
offer two cases in different time periods of neurosyphilis. The first is
a printer with the symptoms of diffuse neurosyphilis six months after
the appearance of his chancre.[147] These symptoms appeared despite
three injections of salvarsan, injections of mercury and mercury by
mouth. Under intensive treatment (meaning injections of salvarsan twice
a week and continued injections of mercury), complete recovery occurred
in a few weeks.
The second case is that of a waiter with signs and symptoms of
neurosyphilis in whom the diagnosis lay between the diffuse and paretic
forms.[148] This patient developed his symptoms in spite of continuous
antisyphilitic treatment during the six years since his infection. This
treatment had been comparatively mild, consisting in great part of
mercury by mouth. However, he had had courses of injections of mercury
and several injections of salvarsan. Under a systematic course of
intravenous injections of salvarsan twice a week for a number of months,
all symptoms disappeared and the spinal fluid tests became negative as
well as the W. R. in the blood serum.
A final case is offered which indicates that antisyphilitic treatment
may occasionally be of service in improving the mentality of a
FEEBLEMINDED CONGENITAL SYPHILITIC.[149]
No attempt has been made in this section to give a per cent evaluation
of the results of treatment in any one group of neurosyphilis. Two
charts (charts 25 and 26), however, are appended which give an
indication of some of our results. It seems to us, however, that it is
too early to make any definite statements as to how far treatment will
take us in the groups of neurosyphilis. We do feel decidedly, however,
that many patients, in whatever group of neurosyphilis the diagnosis may
place them, will respond to intensive systematic antisyphilitic
treatment. =It is unfair to give an entirely grave prognosis in any case
of neurosyphilis until the effect of treatment has been tried.=
In a separate section, entitled NEUROSYPHILIS AND THE WAR, we have
presented fourteen cases selected from British, French and German
writers in the war literature of 1914–16. Most of these cases were
naturally somewhat inadequately reported under the critical conditions
of literature made in the war. We present the cases for what they are
worth: at all events they draw attention to the extraordinary interest
of the neurosyphilis problem in relation to the war.
Such cases as A, one of tabes dorsalis apparently developing paresis by
a process akin to shell-shock, is of value in the interpretation of the
development of paresis in civil life. By “shell-shock” we commonly refer
to a condition in which there is no actual traumatic injury of the
brain. The hypothesis must be then that the explosion in some way
indirectly caused an alteration of living conditions of the spirochetes,
permitting the development of paresis.
Case B similarly seems to be a case in which a latent syphilis has
turned shell-shock into tabes dorsalis.
Cases C, D, E bring up the question of aggravation of neurosyphilis _by_
service and _on_ service, respectively.
Case F likewise shows how, in the determination of amount of pension,
the probable duration of the neurosyphilitic process is important.
Case G seems to show that war stress alone, without the emotional or
physical effects of shell-shock, may kindle a latent syphilis into
paretic neurosyphilis.
Case H similarly suggests that the “gassing” process may effect the same
result.
Case I seems to show that the neuropathically tainted person may have
latent epilepsy brought out through syphilis, the syphilis in this case
having been acquired during the first summer of the war.
Case J was an interesting case of a syphilitic who, after the stress of
the Battle of Dixmude, became an epileptic.
Syphilitic root-sciatica was developed in Case K at work in the war
zone.
Case L is one of a civilian who apparently would not have developed
paresis at precisely the moment when he did, if he had not been
discharged as a German Jew from his long-held bank position in London.
Two cases, M and N, are cases of shell-shock, non-syphilitic; yet the
picture of paresis in the one case and of tabes in the other was for a
long time almost convincing to the examiners. They are better termed
cases of pseudoparesis and pseudotabes, using the prefix “pseudo”, as
usual, to signify a non-syphilitic imitation of the disease in question.
To sum up in the most general way the lessons of this book, we may
emphasize again (1) _the unity-in-variety of the phenomena of
neurosyphilis_, (2) _the value of a hopeful approach to the therapy of
all cases of neurosyphilis_, _even the paretic form_, and (3) _the value
of applying syphilis tests to every case of neurosis or psychosis_.
(1) RE _unity-in-variety of neurosyphilitic phenomena_.
The unity of these phenomena is confirmed, theoretically, by the common
factor of spirochetosis: practically, by the Wassermann reaction,
positive in serum or spinal fluid! Almost at this point the unity of
phenomena ceases. Neither chronicity, nor evidence of mononuclear cell
deposits, nor evidence of serious structural damage to the nervous
system, nor presence of other positive tests than the W. R.,[150] nor
existence of mental or nervous symptoms or signs, is a common feature of
neurosyphilis. Sometimes the nervous system appears to harbor
spirochetes in the most cordial manner as guest-friends (_paresis sine
paresi_.) Again, perhaps as an expression of elaborate processes of
immunity, the spirochetes take effect in relatively huge gummata.
Sometimes the neurosyphilitic process rises as if by a regular process
of siege from spinal nerve-root to spinal nerve-root (tabes dorsalis and
diffuse neurosyphilis). Again, the nervous system is taken by storm, as
it were (disseminated encephalitis). Very frequently the neurosyphilis
is simply an indirect effect of blood-vessel disease, and huge masses of
tissue are scooped out in necrosis with dependent secondary
degenerations; and later the extinct lesions of vascular origin may or
may not betray evidence of their syphilitic origin. Sometimes diffuse
processes run on, apparently, with perfect fatalism to a mortal issue in
a few years both with and without treatment. Again treatment appears to
accomplish much (see fuller discussion under 2). The laws governing the
preference of processes to lodge in membranes, vessels, and parenchyma,
and in all combinations of these, have not been worked out. Hardly a
case of neurosyphilis, properly studied ante mortem and post mortem, but
would throw important light on our medical approach to one of the great
problems of civilization, the problem of syphilis as a whole.
(2) RE _value of a hopeful approach to the therapy of neurosyphilis_.
The prognosis of neurosyphilis is not worse than that of the chronic
diseases in general. In fact, the prognosis of neurosyphilis _quoad
vitam_ is either good or dubious, certainly not bad. The surprising
reversals of form which the spirochete shows in certain remissions are
always to be awaited. Treatment of neurosyphilis has certainly effected
amazing results, not so much by way of Ehrlich’s _therapia sterilisans
magna_ as by means of systematic intensive treatment. Even paretic
neurosyphilis (general paresis) seems to have been cured. Preparetic
phases are theoretically hopeful. Nor is it so certain that paretic
neurosyphilis will ultimately prove a perfectly distinct species of
neurosyphilis. General paresis seems to us at least to be more closely
related to diffuse neurosyphilis than is tabes dorsalis to diffuse
neurosyphilis. In any particular case, moreover, =during a good part of
the early months or years=, =it is difficult or impossible to tell the
paretic from the non-paretic forms of diffuse neurosyphilis by any
combination of clinical observations and tests=. In the instance of more
protracted neurosyphilis, e.g., tabetic, the outlook for vascular
complications is such that antisyphilitic treatment directed at
prevention of these complications is scientifically warrantable, even if
the tabetic process itself proves unassailable. The old distinction of
syphilis and parasyphilis, so striking and apparently satisfactory when
introduced by Fournier, seems to be a false distinction which should be
dropped. Therapeutically, we should approach all cases of neurosyphilis
without bias or nihilistic prejudgments.
(3) RE _universal applicability of syphilis tests in nervous and mental
cases_.
The importance of putting every neurosis or psychosis through syphilis
tests is not based alone on the frequency of neurosyphilis, though
neurosyphilis is surely frequent enough. The importance of universally
applying these tests is established by the experience of lingering
doubts both in the physician’s mind and (nowadays increasingly) in the
patient’s and friends’ minds, so long as these tests are not applied.
Nor should the positive serum Wassermann reaction fail to be followed by
lumbar puncture and appropriate tests. The general practitioner
confronting neuroses or psychoses—and what practitioner does not?—must
not expect valuable results from consultation with neurologists and
psychiatrists when he does not carry to these specialists the results of
at least the serum W. R. in his patient. Not only are practitioners,
specialists, and patients subject to discomfiture on the eventual and
delayed proof of syphilis or neurosyphilis, but valuable time has been
lost to treatment. How often the physician of yore (and really not so
long since) had to be regarded as an eccentric virtuoso if he tested
urine as routine! Well, for routine use in nervous and mental diseases,
the Wassermann serum reaction is at least as important as urinalysis.
Nor would we cease our homily with the general practitioner. We know
neurologists and psychiatrists who use the Wassermann test _only when it
is likely to be positive_! But they are dying out.
APPENDIX A
In appendix A a brief outline is given of the six tests (W. R. on blood
serum and spinal fluid, cell count, globulin test, albumin test, gold
sol test). This is not intended as a complete working manual but rather
as indicating the methods used in diagnosis in the cases presented
herein. For more complete details the reader may be referred to
textbooks on the subject of serology, among which may be mentioned
Kaplan: “Serology of the Nervous System”; Plaut, Rehm and Schottmüller:
“Leitfaden zur Untersuchungen der Zerebrospinalflüssigkeit”; Kolmer:
“Infection, Immunity and Specific Therapy,” and, for the Wassermann
technique, an article by Dr. W. A. Hinton in M. J. Rosenau’s “Preventive
Medicine and Hygiene.”
Our own W. R’s. have been performed at the Wassermann laboratory of the
Massachusetts State Board of Health (formerly the Neuropathological
Testing Laboratory, Harvard Medical School), under the supervision of
Dr. W. A. Hinton. The other tests are performed at the Psychopathic
Hospital. It is very important that a close relationship should exist
between the clinician and the Wassermann laboratory if the most is to be
obtained from the reactions. This relationship has been effectively
close between the authors and the above-mentioned laboratory; and has
enabled us to get very much clearer ideas about certain cases than could
otherwise have been obtained.
=Cell Count.= In order to obtain the number of cells per cmm., the
examination should be made of the fresh fluid as soon as possible after
this is withdrawn. The most convenient counting chamber for this purpose
is the so-called Fuchs-Rosenthal counting chamber, the ruled spaces of
which contain slightly over 3 cmm. (an ordinary blood cell counting
chamber may be used). According to the method used by us the cells are
stained in a pipette with Unna’s polychrome methylene blue. Using a
white-counting pipette, stain is drawn up to the first or second marking
and the remainder of the pipette filled with spinal fluid. This makes no
change in the dilution for practical purposes. After two or three
minutes the staining is satisfactory and the counting may be done. With
this stain a differential count may be made. Plasma cells stain a
lavender as contrasted to the blue of the lymphocytes. The
characteristic halo surrounding the eccentric nucleus is visible. The
blood cells do not assume color with this stain; hence it is unnecessary
to add any acetic acid.
For permanent preparations, and more accurate differential counts of the
spinal fluid, the Alzheimer method may be used. The technique is given
in a paper by H. A. Cotton and J. B. Ayer as follows:[151]
1. Lumbar puncture in the usual manner.
2. 96% alcohol, in proportion to twice the amount of cerebrospinal
fluid, is added drop by drop and well mixed.
3. Centrifuge the mixture for one hour at high speed in a glass tube
with conical end. (An ordinary electric urinary centrifuge apparatus can
be employed, the tube to be well stoppered to prevent evaporation.)
4. The supernatant fluid is poured off, leaving a small coagulum in the
bottom of the tube.
5. Add absolute alcohol—alcohol and ether—ether, each separately for one
hour, to dehydrate and harden coagulum.
6. The coagulum can now be gently loosened from the bottom of the tube
by a long needle. The tube is then inverted, and the coagulum allowed to
fall into the hand by a quick tap on the end of the tube. Care must be
taken not to squeeze or handle the coagulum. The hand is placed over a
small homeopathic vial, containing thin celloidin, and the coagulum
allowed to drop into the celloidin, where it remains over night (twelve
hours usually).
7. Coagulum is placed in thick celloidin which is allowed to evaporate
slowly. It is then mounted on blocks and sections cut 14µ in thickness.
8. The sections are stained and mounted according to the following
procedure:
(_a_) Remove celloidin by absolute alcohol and ether.
(_b_) 80% alcohol.
(_c_) Water.
(_d_) Sections are carried on glass or platinum needle into a dish of
Pappenheim’s pyronin-methyl green stain and kept in a water bath at 40°
C. five to seven minutes.
(_e_) Quickly cool dish in running water.
(_f_) Wash off superfluous stain in plain water.
(_g_) Absolute alcohol to differentiate—until no more stain comes away
from section.
(_h_) Clear in Bergamot oil.
(_i_) Mount in balsam.
The normal cell count may be stated as being up to 6 cells per cmm.;
from 6 to 12 cells may be considered as suggestive of pathological
condition and more than 12 cells per cmm. as definitely pathological.
The type of cell in syphilitic diseases is preponderantly the small
lymphocyte. A low percentage, that is, very rarely over 20%, of large
lymphocytes, endothelial phagocytic cells, polymorphonuclear leucocytes
and plasma cells may also be found. The finding of plasma cells in any
number in the spinal fluid is suggestive although not conclusive
evidence for the diagnosis of paretic neurosyphilis.
=Globulin= is an albumin which is precipitated by half saturation with a
salt. A very simple and satisfactory test is known as the Nonne-Appelt
test, which has been modified by Ross-Jones. Into a test tube of small
diameter, run 1 cc. of spinal fluid. Place under this fluid with a
pipette, 1 cc. of a saturated solution of ammonium sulphate
((NH_{4})_{2}SO_{4}). If any globulin is present a white,
sharply-defined ring will form at the junction of the two fluids.
According to our readings, a ring that is just visible with the aid of a
black background is called 1+, a ring that is just visible without the
black background, 2+; a ring easily perceptible, 3+ and a relatively
very heavy ring, 4+. On shaking the tube, if globulin is present, the
fluid will show turbescence.
Another simple globulin test used in our laboratory as a check on the
Nonne-Appelt test is the Pandy test. A few cc. of a clarified 10%
solution of phenol are placed in a watch glass. One drop of spinal fluid
is run into this solution. A milky turbescence indicates globulin.
The presence of globulin in the spinal fluid is always an indication of
abnormality of the cerebrospinal axis. There is nothing differential in
this finding as it occurs in all inflammatory processes. However, it is
characteristically present in most cases of neurosyphilis (exception to
the rule: the pure vascular type does not show globulin in a very high
per cent).
=Albumin Test.= Albumin in small quantities is present in all spinal
fluids. Increase over the normal amount occurs in pathological
conditions such as most cases of neurosyphilis, especially in those in
which globulin is found. Any albumin precipitant may be used for rough
clinical calculation, comparing the amount of precipitate with that from
the normal fluid. Our method is to place 1 cc. of spinal fluid in a
small test tube of about 5 mm. diameter and to precipitate the albumin
by the addition of 3 drops of 33⅓% of trichloracetic acid. This test has
its chief value as confirmatory of the globulin test, since in the vast
majority of instances where globulin is found there will also be found
an increase in albumin.
The =Gold Sol Reaction= is an empirical test discovered by Carl Lange in
the utilization of the work of Zsigmondi with solutions of colloidal
gold and albumins. Briefly the details of the test are as follows:
Ten tubes are set up in a rack. To the first tube 1.8 cc. of a 0.4% of
salt solution is added and to each of the following tubes 1 cc. of this
solution. Then to the first tube containing 1.8 cc. of salt solution one
adds 0.2 cc. of the spinal fluid to be tested. This gives a dilution of
1 to 10. From this tube 1 cc. is pipetted into the second tube and this
process continued through the ten tubes. This gives dilutions of spinal
fluid of 1 to 10, 1 to 20, 1 to 40, etc., to 1 to 5120 in the last tube.
Then 5 cc. of colloidal gold solution is added to each tube. A positive
reaction is indicated by the precipitation or throwing down of the
colloidal gold into its metallic form. This produces a change in color.
This precipitation may be partial or complete and the amount of
precipitation is indicated by the color and is read as follows:
The unchanged fluid is called 0; a slight change giving a red-blue as 1;
a further change giving a blue-red as 2; a straight blue as 3; a
lavender or violet as 4; and the colorless fluid representing complete
precipitation as 5. The numbers are placed in a row, indicating the tube
in which the color occurs. The fluid from a case of paretic
neurosyphilis will give a complete precipitation beginning in the first
tube and running through a number of tubes and then grading off. It may
be indicated 5 5 5 5 4 3 1 0 0 0. The characteristic reaction of fluids
from tabetic and diffuse neurosyphilis is less strong than from the
paretic. The greater part of the reaction will take place, however, in
the first five tubes, but as a rule it will not begin very strongly in
the first two. A characteristic reaction is 1 2 3 3 2 1 0 0 0 0. Another
reaction that may be considered characteristic of the tabetic or diffuse
form is 3 3 3 2 1 0 0 0 0 0. Fluids from non-syphilitic cases as a rule
give a reaction having its greatest intensity beyond the fifth tube,
that is, in the high dilutions.
A reaction characteristic of brain tumor or tuberculous meningitis is 0
0 0 0 1 3 3 2 1 0.
The conclusions that may be drawn from the gold sol reaction have been
summarized by one of the authors as follows:
1. Fluids from cases of general paresis will give a strong and fairly
characteristic reaction, especially if more than one sample is tested,
in the vast majority of cases.
2. Very rarely a general paresis fluid will give a reaction weaker than
the characteristic one.
3. Fluids from cases of syphilitic involvement of the central nervous
system other than general paresis often give a weaker reaction than the
paretic, but in a fairly high percentage of cases give the same reaction
as the paretics.
4. Non-syphilitic cases may give the same reaction as the paretics;
these cases are usually chronic inflammatory conditions of the central
nervous system.
5. When a syphilitic fluid does not give the strong “paretic reaction,”
it is good presumptive evidence that the case is not general paresis;
and this test offers a very valuable differential diagnostic aid between
general paresis, tabes and cerebrospinal syphilis.
6. The term “syphilitic zone” is a misnomer, as non-syphilitic as well
as syphilitic cases give reactions in this zone; but no fluid of a case
with syphilitic central nervous system disease has given a reaction out
of this zone (test thus valuable negatively). Any fluid giving a
reaction outside of this zone may be considered non-syphilitic.
7. Light reactions may occur without any evident significance, while a
reaction of no greater strength may mean marked inflammatory reaction.
8. Tuberculous meningitis, brain tumor and purulent meningitis fluids
characteristically, though not invariably, give reactions in higher
dilutions than syphilitic fluids.
9. The unsupplemented gold sol test is insufficient evidence on which to
make any diagnosis, but used in conjunction with the Wassermann
reaction, chemical and cytological examinations, it offers much
information looking toward the differential diagnosis of general
paresis, cerebrospinal syphilis, tabes dorsalis, brain tumor,
tuberculous meningitis, purulent meningitis.
10. We believe that no cerebrospinal fluid examination is complete for
clinical purposes without the gold sol test.
The =Wassermann reaction= as carried out in the Wassermann Laboratory is
based on the principles of the original method—the only essential
modification consists in the employment of cholesterinized alcoholic
extracts of human hearts as antigen instead of aqueous extracts of
foetal livers from cases of congenital syphilis. Experience has shown
that properly standardized antigens made from human hearts are much more
sensitive in the detection of true cases of syphilis.
=Antigens.= Three antigens are used, each being an alcoholic extract of
human heart which is saturated at room temperature with cholesterin.
These antigens differ slightly in their sensitiveness. Before the test
is made each antigen is diluted with 0.85% salt solution in the
proportion of four parts of the cholesterinized antigen extract to
sixteen parts of 0.85% salt solution. The amount to be used, the dosage,
is carefully determined by testing each antigen against a large number
of known positive and known negative specimens of blood. The dosage of
the antigens employed is less than one-half the amount which inhibits
hemolysis when the antigen is incubated for one hour with the hemolytic
system which consists of complement, amboceptor and cells in the proper
proportions. These antigens are designated as A, B, and C. Antigen A is
the most sensitive. B and C are very similar to each other
quantitatively and qualitatively.
=Specimens to be tested.= The serum which separates from the clot is
withdrawn, centrifugalized if necessary, and then heated at 55 degrees
for thirty minutes. 0.1 cc. of serum is used in the test and 0.2 cc. of
each specimen is used as a control to exclude the presence of
anti-complementary substances. Spinal fluids are tested in two ways. As
a routine 0.5 cc. of the spinal fluid is used in the test and 1.0 cc. is
used in the control; or when especially requested spinal fluids are
titrated by using respectively 1.0, 0.7, 0.5, 0.3, and 0.1 cc. of the
spinal fluid for each test and 1.0 cc. of spinal fluid for the control.
Spinal fluids are not inactivated.
=Complement.= The complement is obtained from the serum of guinea pig’s
blood. No complement is used when older than eighteen hours. A 10%
solution and 0.85% salt solution is used in the test. The amount used is
twice the minimum quantity necessary to hemolyze the sensitized cells.
=Sheep’s Corpuscles.= A 5% suspension of sheep’s corpuscles in 0.85%
salt solution is prepared from defibrinated sheep’s blood. The
corpuscles are washed three times and for each washing four to five
times as much 0.85% salt solution is used as the original volume of the
defibrinated blood.
=Amboceptor.= The amboceptor is prepared by injecting sheep’s corpuscles
into a rabbit. The serum of this rabbit which contains amboceptor is
diluted with 0.85% salt solution so that 0.25 cc. will hemolyze 0.5 cc.
of a 5% suspension of sheep’s corpuscles. In the test twice the quantity
or 0.5 cc. of amboceptor is used.
=Sensitized Cells.= The sensitized cells consist of equal parts of
washed sheep’s corpuscles and diluted amboceptor. This mixture is
incubated in a water bath at 37° C. for a half hour to effect the
sensitization of the cells.
=Technique of the Wassermann Test.= One-tenth cubic centimeter of each
inactivated specimen of serum and 0.5 cc. of each uninactivated specimen
of spinal fluid is pipetted into a separate tube. A mixture is freshly
prepared in salt solution, each cubic centimeter of which contains the
proper amount of antigen A (the most sensitive antigen), and two units
of a 10% solution of guinea pig serum (complement). One cubic centimeter
of this mixture is pipetted into each test tube. These tubes are then
incubated for forty minutes in a water bath at 37° C. At the end of this
period, sensitized cells are added, and the tubes are again incubated in
a water bath at 37° C. for one hour. Each specimen which shows any
degree of inhibition of hemolysis is retested in the afternoon. For this
second test antigen A is again used and in addition antigens B and C. A
control is also made for each specimen retested to eliminate any
possibility of the inhibition of hemolysis being due to
anti-complementary substances in the serum or spinal fluid tested. The
technique of the second test differs in no wise from that of the first,
except for the use of a control in each retested specimen and the
employment of three antigens instead of one. The degree of positiveness
is noted for each retested specimen and compared with the degree of
positiveness obtained for the corresponding specimen with the same
antigen-complement-salt solution mixture in the morning’s test. The
specimen is retested on the next day when discrepancies occur between
the morning reading for antigen A and the afternoon reading for antigen
A. From the above description it will be noted that the negative
specimens have but a single test with one antigen only, while the
positive specimens are retested, thus permitting a confirmation of any
positive reaction. In this way attention is focalized on the positive
specimens.