APPENDICES:
A. The six tests 471
B. Common methods of treatment 486
INTRODUCTION
It is a privilege to be allowed to write a word of introduction to a
textbook which so richly fulfils its function as does this volume on the
manifold disorders classified under Neurosyphilis, a subject of which
the importance for the welfare of society is found to loom the larger
the more deeply its mysteries are probed.
The case histories with which its pages are so amply stocked are
carefully analyzed in accordance with a broadly chosen plan, and the
generalizations that precede and follow them are obviously based on a
wide and varied personal experience such as alone could render a
familiarity with the literature of the subjects treated adequate to its
best usefulness. Both writers were indeed well adapted for this task.
Dr. Southard, as everyone is aware, has long been a highly
conscientious, ardent and productive worker in the department of
pathological anatomy, and of late years a careful student of clinical
diagnosis and methods, both at the Danvers State Hospital and still
more, at the Psychopathic Hospital which he worked so hard to found;
while Dr. Solomon’s researches, in the special field of neurosyphilis,
have been of the highest order.
Undoubted as are the merits of the case-system of instruction that has
been so much in vogue in recent years, and excellent as is the modern
supplementation of this method by the use of published records, the
danger is still real that the student will have presented to him a
picture of nature in disease that is too diagrammatic, too concise, with
the result that while the task of memory is lightened through simplified
formulation, the training of the doubting and inquiring instincts is
often given too little stimulus and scope. In this book this danger is
deliberately met through the casting of emphasis rather on the
pluralistic aspects of the processes at stake than (primarily) on their
unitary aspects.
The student who utilizes this volume cannot but emerge from his study a
more thoughtful person than he was at the period of his entry. He will
have seen that clinical rules of thumb cannot be followed to advantage,
and that, on the contrary, surprises are to be expected and prepared
for. Let the recognition of this fact, if it seems to increase the
difficulties in the way of diagnosis, not lead to pessimism in that
respect, or to hopelessness in therapeutics. On the contrary the
writers’ bias is towards the worth-whileness of clinical efforts and an
increased respect for accuracy and thoroughness in the utilization of
modern methods of research. The chance is indeed held open that even the
gaunt spectre of “General Paresis” may prove to be less terrible than it
seems, and for this hope good grounds are given.
It is in this way made clear, on the strength of anatomical evidence of
much interest, that even if in the treatment of a given patient, the
time arrives when a fatal or unfavorable result seems manifestly
foreshadowed, it may be still worth while to renew the treatment with
fresh zeal, for the sake of combatting some symptom or exacerbation, for
which a locally fresh process furnishes the cause.
Another noteworthy principle here emphasized and illustrated is that the
relationship between “functional” (hysterical, neurasthenic, migrainoid)
symptoms and the signs (or symptoms) of organic processes is clinically
important and worthy of much further study. This is a matter which, in a
general sense, has interested me for many years. Above and over the
“organic” hovers always the “functional,” as representing the first
indication of the marvelous tendency to repair, or substitution, for
which the resources of nature are so vast. Yet this functional tendency
also has its laws, of which, in their turn, the organic processes
display the action in quasi diagrammatic form. Hysteria, neurasthenia,
migraine, etc., do not arise _de novo_ in each case, but conform to
typical, though not rigid, formulas, susceptible of description. I have
recently had the opportunity to study in detail an analogous series of
transitions between the movements (and emotions) indicative of
apparently purposeless myoclonic movements (on an epileptoid basis) and
the movements of surprise, engrossment, purposeful effort, the
excitement and joy by which the former were excited and into which they
shaded over.
Taken altogether, this book represents work and thought in which, for
amount and kind, the neurologists of Boston may take just pride.
JAMES J. PUTNAM.
ST. HUBERT’S, KEENE VALLEY, NEW YORK.
_August, 1917._
Me miserable! which way shall I fly
Infinite wrath and infinite despair?
Which way I fly is Hell; myself am Hell;
And, in the lowest deep, a lower deep
Still threatening to devour me opens wide,
To which the Hell I suffer seems a Heaven.
Paradise Lost, Book IV, lines 73–78.
I. THE NATURE AND FORMS OF SYPHILIS OF THE NERVOUS SYSTEM
(NEUROSYPHILIS)
PARADIGM to show possible abundance and variety of symptoms and
lesions in DIFFUSE NEUROSYPHILIS (“cerebrospinal syphilis”).
Autopsy.
=Case 1.= Mrs. Alice Morton[1] was in the hands of at least five
well-known specialists in different branches of medicine and surgery
during the nineteen years of her disease. It appears that she acquired
syphilis upon marriage at the age of 23 to a man who later became
tabetic and acknowledged syphilitic infection previous to marriage. Mrs.
Morton remained without children and there were no miscarriages.
At the age of 27, she developed iritis, paresis of the left eye muscles,
and ulceration of the throat, with destruction of the uvula. The
syphilitic nature of her disease was at once recognized and the
classical treatment was given, although, through numerous shifts in
consultants, this treatment was never pushed to the limit. At 28 Mrs. M.
began to suffer from severe headaches resembling migraine and
accompanied by attacks of paræsthesia; at 35, came severe pains in the
back and difficulty in walking.
At 36, the migraine attacks began to be accompanied by blurring of
vision and dizziness. The difficulty in walking became extreme,
affecting particularly the right foot. The legs became spastic, there
were pains and hyperæsthesia of the chest, and severe cramps of the
legs. Antisyphilitic treatment at this time yielded marked improvement.
During her thirty-sixth year, Mrs. M. sustained curious transient losses
of vision and of hearing. She was also irritable, and at this time
developed her first pronounced mental symptoms, namely, delusions
concerning her relatives. There were also a few seizures of an
epileptiform nature.
At 38 there was a spell of total deafness, followed by improvement. The
eye muscles were also subject to a variable involvement with intervening
spells of improvement. The _knee-jerks were lost, but after a time
returned_ in less pronounced form. Shortly, an absolute paralysis and
extensive decubitus developed, and death occurred at 39.
The autopsy is briefly summarized below, but it is important in the
understanding of Mrs. M.’s case (particularly some of the sensory
symptoms and the transiency of certain symptoms) to consider the
pre-infective history. Although there seems to be no doubt that the
patient acquired syphilis at about 23 years of age from a syphilitic
husband, who himself later became tabetic, yet it is of note that the
patient was the only child of parents, both of whom also suffered from
mental disease. Mrs. M.’s father died of what was called softening of
the brain (one should avoid terming _all_ old cases of _so-called_
“softening of the brain” syphilitic, since the older diagnosticians did
not always distinguish between non-syphilitic arteriosclerotic effects
and syphilitic disease). Mrs. M.’s mother also died insane (confusion
and emotional depression). It is clear, then, that we do not need to
suppose that every symptom shown by Mrs. M. is directly due to
destructive or irritative lesions immediately due to the spirocheta
pallida. The case is, in fact, an excellent lesson as to the association
of structural and functional effects in neuropathological cases.
Mrs. M. as a child had shown talent, but was somewhat nervous and
eccentric. At one time, she had an attack of hysterical dysphasia; at
another time, an attack of hysterical dyspnea; during another period, an
apparent obsession (kicking the mopboard at regular intervals).
Moreover, she had for years suffered from migraines of a severe and
unusual type. Both the hysterical tendency and the migrainous tendency
became mingled with the results of the neurosyphilis in later stages of
the disease in such wise that it was hard to tell exactly where the
structural phenomena left off and the functional phenomena began.
For example, at the age of 32, nine years after infection and four years
after the earliest nerve symptoms traceable to syphilis, and at about
the time of the onset of spinal cord symptoms, an attack was described
as follows:
The patient had a very severe attack of migraine (?) yesterday,
preceded and accompanied by paraphasia, so severe that for three
hours she was unable to make herself understood, and indeed felt “as
if her ideas were getting away from her.” This attack was ushered in
by a numbness of the forefinger and thumb of the right hand, which
lasted for about three hours, though the earlier attacks had lasted
for only about ten minutes. During this period the hand felt as if
it had been frozen and the loss of muscular power was so great that
she was unable to hold objects in the hand. In some of the attacks
this paræsthesia has affected the entire left half of the body, and
occasionally the right half. Sometimes the seizures come on with
great suddenness, so that once, when she was attacked while in the
middle of the street, she had considerable difficulty in reaching
the sidewalk. After the worst part of the attack is over a certain
amount of paraphasia may persist for some days, together with
awkwardness in the use of the right hand and numbness. She has had a
great deal of nausea and vomiting, without reference to the taking
of food.[2]
Bearing in mind the mingling of structural with functional symptoms in
this case, let us consider the autopsy findings.
=ANATOMICAL=
=FORMS OF NEUROSYPHILIS=
AUTONOMIC (SYMPATHETIC) NEUROSYPHILIS?
PERIPHERAL NEUROSYPHILIS
CENTRAL NEUROSYPHILIS
MENINGEAL
VASCULAR
PARENCHYMATOUS
MENINGOVASCULAR
VASCULOPARENCHYMATOUS
DIFFUSE ( = MENINGOVASCULOPARENCHYMATOUS)
GUMMA
CHART 1
=CLINICAL FORMS OF NEUROSYPHILIS=
=HEAD AND FEARNSIDES, 1914=
SYPHILIS MENINGOVASCULARIS
CEREBRAL FORMS
HEMIPLEGIA
AFFECTION OF THE CRANIAL NERVES
MUSCULAR ATROPHY
LATERAL AND COMBINED DEGENERATIONS
EPILEPSY
SYPHILIS CENTRALIS
DEMENTIA PARALYTICA
TABES DORSALIS
MUSCULAR ATROPHY
OPTIC ATROPHY
GASTRIC CRISES
EPILEPTIC MANIFESTATIONS
CHART 2
=Peripheral neurosyphilis=: The lesions of the cranial nerves were
characteristically asymmetrical. Whereas the left third nerve looked
entirely normal, the =right third nerve= had its diameter reduced
two-thirds. On the other hand, the fourth nerves were equal and
apparently normal. The sensory portion of the left fifth nerve was
normal; the right fifth nerve was normal. The =right sixth nerve= agreed
with the right third nerve in being atrophic, and was in fact reduced to
a mere thread without contained nerve fibres at a point 2 mm. from its
superficial origin. Although the right third nerve was atrophic, it was
the =left seventh and eighth nerves= which had become atrophic; the
process had spared the right seventh and eighth nerves. The remainder of
the cranial nerves were grossly normal, except that the =optic nerves=
had an outer zone of a translucent nature. So far, no spirochetes have
been demonstrated in any portion of the nervous system of this case, but
such asymmetrical and focal cranial nerve lesions are perhaps due to
local spirochetal infection, punctuating (as it were) the diffuse
process.
How much of the transient blindness, deafness, and ocular paralysis can
be explained on the anatomical findings in these nerves? Possibly a
portion of the phenomena can be so explained. Thus, the mechanical
conditions of pressure inside and outside these nerves, both in their
peripheral course and in their passage through the membranes, can be
readily understood to differ during the acute and subacute inflammation,
during the process of repair in the pial tissues, and during the process
of overgrowth of neuroglia tissue about the superficial origins of the
nerves. Of course, the majority of lesions of these nerves were entirely
extinct at the time of the autopsy, and their history could be surmised
only from the appearances in the _left eighth nerve_. Here occurred a
sharply marked focal area of gliosis with apparently total destruction
of nerve fibres and related with a _lymphocytosis_ of the investing
membrane (one of the few areas of lymphocytosis found anywhere in this
case).
If it were not for the pre-infective history, the hysterical dysphasia
and dypsnea, the youthful obsessions, the migrainous tendency, and the
psychopathic inheritance, we might be tempted to try to explain the
transient blindness, the deafness, and ocular palsies on the basis of
mechanical and toxic variations in the conditions of the peripheral
cranial nerves. The existence of a trace of lymphocytosis in the left
eighth nerve leads to the hypothesis that treatment might still be
effective in this particular region (see below in discussion of spinal
symptoms).
=Spinal neurosyphilis:= Not only the spinal cord but also the posterior
and anterior nerve roots exhibited severe lesions. These lesions were
both meningeal and parenchymatous. The meningeal process differed in its
intensity in different parts of the spinal cord, being severest in the
thoracic region. At one point in this region, the dura mater was so
firmly attached to the pia mater that the line of demarcation between
the two membranes was hard to make out. In fact, it seems clear that
there could have been no free intercommunication between the spinal
fluid above these adhesions of dura to pia mater and the spinal fluid
below the adhesions. Accordingly, it seems that _lumbar puncture_, had
it been practised in this case, _would have failed to show features
representative of the whole cerebrospinal fluid system_. Moreover, since
at no point in this region of adhesions or in the pia mater of the
spinal cord below this point, were found any lymphocytes, it seems clear
that the ordinary lumbar puncture would have failed to reveal a
pleocytosis. Whether this fluid would have yielded a positive globulin
and excess albumin test, it is now impossible to say; but it appears
that the process in the lower part of the spinal cord was to all intents
and purposes extinct.
However, there was one region of more severe inflammatory involvement.
The _spinal cord in the cervical region showed a lymphocyte
infiltration_ of its vessels amounting to a mild myelitis (meaning,
thereby, an inflammatory process of the spinal cord remote from the pia
mater). Moreover, in this region, there was, besides the perivascular
infiltration of the substance, also an infiltration of the overlying
membranes themselves, especially in and near the posterior root zones.
The lessons of this finding are several: The inflammatory process in
this case does not appear to have been entirely extinct! Can we not
suppose that treatment might still have benefited this local
inflammation (perivascular infiltration of the cervical spinal cord
substance and overlying lymphocytic meningitis)? Can we not also picture
the gradual ascent of the inflammatory lesions from lower segments to
higher segments and possibly conceive of the gradual elevation of the
zone of hyperæsthesia manifested in this case as following the gradual
displacement upward of the lymphocytic process? Are there spirochetes in
this tissue? So far none have been discovered, possibly through
inaccuracies of available technique. To the neuropathologist, however,
the lesion looks like a local reaction to organisms.
In addition to the spinal meningitis, chronic and acute, as above
described, there were extensive parenchymatous spinal lesions.
In the first place, the meningitis had affected practically all the
posterior roots so that the explanation of the posterior column
sclerosis of this case is clear. The meningitis had apparently been so
marked, also, that all the fibres anywhere near the periphery of the
spinal cord had been likewise destroyed. The posterior columns and the
posterior root zones were markedly sclerotic; or as we say (having
reference to the overgrowth of neuroglia tissue) gliotic. But there was
as much sclerosis (gliosis) of the lateral columns (particularly in the
posterior two-thirds) as there was in the posterior columns and root
zones. In fact, the entire posterior half or two-thirds of the spinal
cord markedly outstripped the anterior portions of the cord in the
severity of the gliosis (sclerosis) shown.
But although we can explain the posterior column sclerosis, the
sclerosis of the posterior root zones and the marginal sclerosis
(_Randsklerose_) round the entire periphery of the cord, on the basis of
long-standing effects of old meningitis, we cannot thus explain another
finding, namely, the destruction of the fibres in the lateral columns.
This, in fact, is explained through lesions (mentioned below) that
affected the encephalon. The net result of all these lesions of the
spinal cord was to leave only the gray matter and a small amount of
surrounding fibres (belonging to short tracts uniting nearby segments)
intact. Briefly stated, =every long tract in the spinal cord appeared
upon examination to be extensively degenerated=. The genesis of this
parenchymatous loss was, however, double, being in part due to a local
meningeal process (sometimes known as “perimeningitis”) and in part due
to a cutting off of the pyramidal tract fibres on both sides by lesions
higher up in the nervous system.
[Illustration:
CASE I. SPINAL CORD (THREE LEVELS) SHOWING:
A. Marginal sclerosis—effect of old meningitis now extinct.
B. Posterior column sclerosis—effect of meningitis about posterior
roots also now extinct.
C. Bilateral pyramidal tract sclerosis—effect of cerebral thrombotic
lesions.
Note distortion of tissues in B and C, partly artificial (tissues in
places diffluent).
]
=ANATOMICAL FORMULAE=
=MENINGOVASCULOPARENCHYMATOUS INVOLVEMENT=
M, V, P, or Combinations Applied to the Classification of Head and
Fearnsides
I. SYPHILIS MENINGOVASCULARIS
CEREBRAL FORMS M or V or MV[3]
HEMIPLEGIA V
AFFECTION OF THE CRANIAL NERVES M
MUSCULAR ATROPHY M
LATERAL AND COMBINED DEGENERATIONS M
EPILEPSY M or V
II. SYPHILIS CENTRALIS
DEMENTIA PARALYTICA MVP or VP
TABES DORSALIS MP
MUSCULAR ATROPHY P
OPTIC ATROPHY P
GASTRIC CRISES (M? or) P?
EPILEPTIC MANIFESTATIONS P?
CHART 3
Can we offer any explanation of the =partial return of knee-jerks= after
their temporary total loss at a certain period of the disease? We may
assume that the knee-jerks were functionally lost about a year before
the death of the patient through the partial or even almost complete
destruction of the entering posterior root fibres at that level of the
spinal cord which is directly related with the knee-jerk. The later
partial return of the knee-jerks apparently requires us to suppose the
maintenance of some fibres and collaterals by which a functional
connection can be effected between the fibres of the posterior roots and
the anterior horn cells which innervate the quadriceps femoris. Let us
now suppose that _pari passu_ with the actual return of the knee-jerks,
the destructive processes that are affecting both pyramidal tracts high
up in the nervous system are now advancing. It is clear that, whatever
inhibitory influence these pyramidal tracts have been exerting up to
this time upon the knee-jerk reflex arc, that influence is now to be
decidedly reduced in amount and possibly absolutely lost. Upon the loss
of such inhibitory influences exerted from above, the few persisting
connections of the posterior roots and anterior horn cells are now
permitted to resume their functions.
=Encephalic neurosyphilis=: The lesions mentioned above as causing
destruction of the pyramidal tracts of the spinal cord were
symmetrically destructive and atrophic lesions of the gray matter of
both corpora striata with atrophy of the anterior segments of the
internal capsules. There was a degenerative process of the corpus
callosum especially affecting the forceps minor of the tapetum. The
ventricles were largely dilated, indicating a considerable destruction
and atrophy of the white matter in general.
After the above discussion of the possible effects of pyramidal tract
lesion in this case, it is unnecessary further to discuss the paraplegia
produced by the cystic lesions of the corpora striata. The theorist
might inquire how these cystic lesions are produced: whether by vascular
blocking or by toxic effects of the accumulations of spirochetes.
Evidence is lacking which would completely sustain either hypothesis.
Still, we do know that lesions almost identical in appearance may be
produced by the necrosis consequent to the plugging of nutritive vessels
in an organ like the brain supplied with end arteries. Therefore, it is
probable that most pathologists would believe these lesions of the
corpora striata to be produced by vascular plugging of the nature of
thrombosis.
It is worth while to note that there was a suggestion of foci of
encephalitis made out upon the gross examination. The cortex in general
showed strikingly few lesions. However, the convolutions did show in
places numerous ill-defined areas of hyperemia and slight swelling.
These areas were of irregular distribution and only a few mm. or cm. in
diameter. No gross vascular lesions were demonstrable in connection with
these focal areas. Microscopically, however, venous plugs of
polymorphonuclear leucocytes were found, and the local hyperemias were
found to be largely due to venous congestion. However, very few
polymorphonuclear leucocytes were found outside the blood vessels.
The white matter of numerous convolutions showed microscopically certain
pale spots suggestive of an early atrophic process. Very possibly these
represent a general tendency in the cerebrum to the same process of
parenchymatous loss which had proceeded to such a marked degree in the
spinal cord.
There was a single large so-called cyst of softening in the cerebellum
(1.5 mm. across by 0.5–7.5 cm. in depth).
How far can we explain the symptoms of this case on the basis of these
encephalic lesions? We can offer no correlation with the cerebellar
lesion; and possibly this lack of correlation is to be expected on
account of its failure to affect the vermis. As to the cystic lesions of
the corpora striata, their effect in producing paraplegia at the close
of life is obvious, and their possible relation to the partial return of
knee-jerks has been discussed. Literally amazing was the comparative
integrity of the cortical gray matter of this case when the spinal cord
and the interior structures of the encephalon had been subjected to such
severe and numerous lesions. The only mental symptoms noted in the case
were sundry delusions directed against the patient’s relatives and a
certain optimism which led the patient to cling as if with an obsession
to the belief that in the end she would get well.
=VARIOUS FORMS OF NEUROSYPHILIS COLLECTED FROM SEVERAL SOURCES=
=MENINGEAL NEUROSYPHILIS (M)=
GUMMA OF DURA MATER M
GUMMATOUS MENINGITIS (PIAL) M
SYPHILITIC MENINGITIS (PIAL) M
SYPHILITIC CRANIAL NERVE PALSIES (PRIMARILY PIAL) M
SYPHILITIC BULBAR PALSY M
SYPHILITIC ROOT NEURITIS M
SYPHILITIC TRANSVERSE MYELITIS M
SYPHILITIC NEURITIS (SOME CASES BY EXTENSION) M
SYPHILITIC EPILEPSY (SOME CASES) M
SYPHILITIC MUSCULAR ATROPHY (SOME CASES) M
=VASCULAR NEUROSYPHILIS (V)=
SYPHILITIC ARTERIOSCLEROSIS V
SYPHILITIC CEREBRAL THROMBOSIS V
SYPHILITIC APOPLEXY V
ANEURYSM V
SYPHILITIC EPILEPSY V
=PARENCHYMATOUS NEUROSYPHILIS (P)=
GUMMA P
CEREBROSPINAL SCLEROSIS P
SYPHILITIC PARANOIA P?
SYPHILITIC CHOREA P
SYPHILITIC EPILEPSY P
TABETIC PSYCHOSIS P?
SYPHILITIC MUSCULAR ATROPHY P
SYPHILITIC NEURITIS P
CHART 4A
=MENINGOVASCULAR NEUROSYPHILIS (MV)=
CEREBRAL SYPHILIS MV
CEREBROSPINAL SYPHILIS MV
SYPHILITIC EPILEPSY MV
=MENINGOPARENCHYMATOUS NEUROSYPHILIS (MP)=
CEREBRAL SYPHILIS MP
CEREBROSPINAL SYPHILIS MP
TABES DORSALIS MP
ERB’S SYPHILITIC SPASTIC SPINAL PALSY MP
=VASCULOPARENCHYMATOUS NEUROSYPHILIS (VP)=
CEREBRAL SYPHILIS VP
CEREBROSPINAL SYPHILIS VP
PARETIC NEUROSYPHILIS (GENERAL PARESIS) VP
LISSAUER’S GENERAL PARESIS VP
=MENINGOVASCULOPARENCHYMATOUS NEUROSYPHILIS (MVP)=
CEREBRAL SYPHILIS MVP
CEREBROSPINAL SYPHILIS MVP
PARETIC NEUROSYPHILIS MVP
TABOPARESIS MVP
=DOUBTFUL (TOXIC?, IRRITATIVE?) NEUROSYPHILIS (?)=
“PARESIS SINE PARESI”
SYPHILITIC NEURASTHENIA
TABETIC PSYCHOSIS
SYPHILITIC PARANOIA
SYPHILITIC POLYURIA, POLYDIPSIA
SYPHILITIC NEURALGIA
CHART 4B
=Summary:= We have here dealt at length with a long-standing DIFFUSE
NEUROSYPHILIS affecting to some extent the entire =meninges= and
producing a destruction of posterior column fibres and numerous other
fibres of the spinal cord (=tabetiform= portion of the neurosyphilis
=picture=). We have also found central lesions of the corpora striata
affecting the destruction of both pyramidal tracts (=paraplegic= portion
of the neurosyphilis =picture=). We have found evidences of acute
inflammation (=lymphocytosis=) in the cervical region of the spinal cord
and in the left eighth nerve (=progressive inflammatory= neurosyphilis
=picture=). In short, we have presented a case of =diffuse=
(meningovasculoparenchymatous) =neurosyphilis= characterized by an
ascending character in a course of at least 16 years; we have indicated
a number of possible clinical correlations, not only with the major
portion of the clinical course (symptoms of myelitis and pyramidal tract
destruction), but we have also mentioned, merely for their suggestive
value, a number of finer correlations between histological findings and
certain clinical features (notably transient losses of vision and
hearing, and a partial return of the lost knee-jerks). Bearing in mind
the clinical and anatomical findings of this case, we shall be able to
discuss the cases that follow in a briefer and more condensed fashion.
=TABETIC NEUROSYPHILIS (“tabes dorsalis,” “locomotor ataxia”)
complicated by vascular neurosyphilis (hemiplegia). Autopsy.=
=Case 2.= Francis Garfield had been a successful lumberman and had
enjoyed good health until his forty-fifth year. Suddenly one day, while
walking on the street, Garfield lost the use of his legs and for a time
was quite unable to walk. However, he recovered locomotion and after a
time there was nothing wrong with his leg movements except a slight
ataxia.
At the age of 52 Garfield had to give up work. It appears that he had
been becoming cranky, sometimes, for example, shouting, whistling and
slamming doors, apparently to annoy the family. His intellectual
capacity seemed to be maintained, although his memory was slightly
impaired.
At 67 years there was an ill-defined seizure, followed a few days later
by another seizure with aphasia (wrong words used and lack of
understanding of things said).
For years Garfield had been totally deaf in the right ear (following
explosion of a gun?). Now, however, the left ear also showed a sensory
impairment. Slight slurring of speech had been noticed first in the
sixty-sixth year.
=Physically= there was a slightly enlarged heart with accentuated second
aortic sound and irregular rhythm. =Neurologically=, inability to stand
or walk; marked ataxia in his leg movements; upper extremities quite
well controlled; the pupils were small and unequal, the left being
larger than the right; although the reactions were difficult to test,
the pupils seemed to react slightly to direct light stimuli; the
knee-jerks were absent; tests for sensibility so far as could be
determined did not show any abnormalities; there was much complaint of
sharp pains in the legs.
There is no doubt that we are here dealing with a case of TABES DORSALIS
plus certain complications due to VASCULAR LESIONS. The case went on to
death from rupture of =aortic aneurysm= (also doubtless a syphilitic
complication). The death occurred at 71, four years after admission to
Danvers Hospital.
=MAIN FORMS OF NEUROSYPHILIS=
=(CLASSIFICATION OF THIS BOOK)=
DIFFUSE NEUROSYPHILIS
(non-vascular forms of “cerebral,” “spinal” and “cerebrospinal
syphilis”)
VASCULAR NEUROSYPHILIS
(“cerebral arteriosclerosis,” “cerebral thrombosis”)
PARETIC NEUROSYPHILIS
(“general paresis”)
TABETIC NEUROSYPHILIS
(“tabes dorsalis”)
GUMMATOUS NEUROSYPHILIS
(“gumma of membranes, of brain”)
JUVENILE NEUROSYPHILIS
(paretic, tabetic, diffuse)
CHART 5
=POSSIBLE INVOLVEMENT=
=BRAIN AND CORD SYPHILIS=
[M]embranes, [V]essels, [P]arenchyma
[MVP] EARLY, LATENT?, SYMBIOSIS?, ATTENUATION?....
MVP CEREBRAL, CEREBROSPINAL SYPHILIS, PARESIS MVP
[M]VP PARESIS; SYPHILITIC ARTERIOSCLEROSIS VP
M[V]P ?SYPHILOTOXIN FROM MENINGITIS MP
MV[P] SYPHILITIC MENINGITIS; CEREBRAL OR CEREBROSPINAL SYPHILIS MV
[MV]P SYPHILOTOXIC ATROPHY OR SCLEROSIS P
M[VP] SYPHILITIC MENINGITIS M
[M]V[P] SYPHILITIC ARTERIOSCLEROSIS V
M, V or P in brackets [] means not involved.
CHART 6
=NEUROSYPHILIS=
=SIX TESTS=
BLOOD WASSERMANN
SPINAL FLUID WASSERMANN
SPINAL FLUID CYTOLOGY
SPINAL FLUID GLOBULIN
SPINAL FLUID ALBUMIN
SPINAL FLUID GOLD SOL
CHART 7
This case has been especially worked up and published by Dr. A. M.
Barrett on account of the fact that the vascular lesions of the brain
had produced a condition of pure word-deafness. Reference is made to the
Journal of Nervous and Mental Disease, Vol. 37, 1910, for a complete
description of the brain findings and an analysis of the word-deafness,
a summary of which is as follows:
“Reaction to Words and Sounds.—Total deafness to words spoken, but
gives attention to sounds; no ability to recognize meaning of sounds
heard; no ability to repeat words heard. Spontaneous
Speech.—Retained ability to speak spontaneously, with rare
paraphasic utterances; occasional inability to speak readily the
word desired, but later always giving the correct reaction;
calculation fair; spelling good except for occasional paraphasia;
spelling good for words pronounced. Reaction to Things Seen.—Objects
correctly recognized and named except for an occasional paraphasic
reply; mistakes in pronunciation not recognized; correct color
recognition. Reaction to Things Felt.—Good for familiar objects; an
occasional paraphasic reply. Reaction to Words Seen.—Reads printing
and writing understandingly; unimpaired reading except for an
occasional paraphasic reply; meaning of familiar signs recognized;
slight difficulty in readily understanding meaning of arithmetical
signs. Writing.—Spontaneous writing and drawing ability retained;
ataxia (tabetic) in writing movements; no ability to write from
dictation. Internal language.—No evidence of impairment.”
The brain post mortem showed severe atheromatous degeneration of the
arteries at the base of the brain. Both middle cerebral arteries showed
scattered atheromatous patches. The pia mater was transparent and
delicate, except in the regions of both Sylvian fissures. There were
residuals of old softening in both temporal lobes. In the fresh brain
the regions of the right and left first temporal convolutions were
sunken inward, and the pia intimately adherent to the softened areas.
The limits and more exact localizing of these softenings were worked out
from serial sections.
Barrett found in his serial sections that, although the transverse
temporal convolutions of the left hemispheres were intact, these
convolutions were undermined throughout their entire extent by
degenerations in the fibres of the center of the first temporal
convolution. Barrett, accordingly, regarded his case as essentially a
case of subcortical tissue destruction. He agrees with various authors
that the pure word-deafness of his case is the result of an isolation of
the receiving station in the transverse convolutions of the left
hemisphere. The tissue destruction produced by the vascular lesion had
cut off the transverse convolutions from the internal geniculate body.
We are here, however, not considering the origin and relations of pure
word-deafness but present the case as one of =tabes dorsalis= of 20
years standing, terminated by two characteristic syphilitic
complications, first, an extensive destruction of brain tissue through
=cerebral thrombosis= and secondly, =fatal aortic aneurysm=.
=Summary=: We have here dealt briefly with a long-standing case of
NEUROSYPHILIS of the TABETIC type: A characteristic but not necessary
complication of the case is the LATE CEREBRAL VASCULAR INVOLVEMENT. The
=posterior column sclerosis= is virtually the only spinal change. Spinal
meningeal changes are absent (although it is to be assumed that chronic
inflammatory changes in the posterior roots were at one time present in
some quantity and although the spinal fluid characteristically shows
lymphocytosis in tabetic neurosyphilis).
Whether the spirochetes produce special toxic components able to cause
tabes or whether special kinds of spirochete are the tabes-making kinds
is hard to say. Special qualities of individual tissue may be involved.
The =cerebral lesions= of a =cystic= nature are of vascular origin, like
the differently localized encephalic lesions of Case 1 (Alice Morton).
Vascular syphilis is not a special property of the vessels of the
nervous system. In fact this very case died of =aortic aneurysm=.
=PARETIC NEUROSYPHILIS (“general paresis,” “dementia paralytica,”
“softening of the brain”). Autopsy.=
=Case 3.= James Dixon, 44, was first seen at the Danvers Hospital,
reciting verses in a dramatic and noisy way. He remained good-natured
and jolly; nor was there any change in his euphoria until he had become
physically weaker and more generally demented. In fact, Dixon appeared
to become more and more expansive as he became physically weaker. He was
in the habit of describing himself as “O. K., No. 1, Superfine.”
=Physically= the patient was gray and bald on vertex, had a dusky
complexion, was very thin (6 ft. in height, weight 155 lbs.); the mucous
membranes were pallid; the teeth rather poorly preserved; the heart was
somewhat enlarged; the pulse irregular in rhythm, of poor volume and
tension.
=Neurologically=, the patient showed a characteristic Romberg sign and
ataxia in walking a straight line. The tremulous tongue was protruded to
the left, and there was a coarse tremor of the extended fingers. The
knee-jerks were absent, and the Achilles jerks could not be obtained;
the plantar reactions were slight; the arm reflexes were present. The
pupils were stiff to light. There was a marked vocal tremor. The
sensations could not be tested on account of the patient’s mental state.
It appears that Dixon had left school at about 16, at about 22 had gone
into the provision business, and later had become a hotel clerk. He had
married at 28; there had been two miscarriages, at three months and six
weeks respectively; one child was stillborn; four children were living.
The patient was not very alcoholic. The patient’s wife thought the
symptoms had been coming on since his forty-first year when irritability
set in, but he was not discharged from work until about a year since. He
was taken back again after his wife’s pleas, and remained at work about
three months; but for ten months before admission to the hospital, Dixon
had done practically nothing, had shown a marked memory failure and
speech defect, at the same time claiming to be a person capable of doing
and accomplishing everything. He had become careless of his personal
appearance, collected a drawer-full of stumps of cigars, carried lumps
of coal in his pocket, laughed causelessly, and spat on the carpet.
We here deal with a case of unknown duration from the initial infection,
but with symptoms lasting about three years and three months. Aside from
the cause of death (empyema of left pleural cavity associated with acute
hemorrhagic splenitis, acute ileitis, and bronchial lymphnoditis), the
body showed a number of other lesions outside the nervous system. There
was the usual sclerosis of the aorta, though perhaps less marked than
usual. There was a curious acute arteritis with fusiform dilatation of
the arteria profunda femoris, with an edema of the thigh muscles and
blebs of the overlying skin. There were also multiple chronic caseating
lesions of the liver, without evidence of fibrosis. The explanation of
these liver lesions is not yet clear. There was a cloudy swelling of the
kidney.
The calvarium was dense and the dura mater thick and adherent. There was
a chronic leptomeningitis, which, however, was rather unusual in being
most marked in the posterior cisterna and along the sulci of the
cerebellar hemispheres. There was a general cerebral sclerosis, with a
question of atrophy of the superior temporal gyri (suggesting the
so-called Lissauer’s paresis). There was a marked cerebellar sclerosis
with a consequent sclerosis (grossly palpable) of the commissural fibres
of the pons. There was a generalized slight spinal sclerosis. As a fair
sample of the variety of head findings in paretic neurosyphilis, the
details of the =head examination= are presented.
[Illustration:
A. Normal postcentral cortex. (Compare B.)
]
[Illustration:
B. Nerve cell losses. Perivascular deposits of mononuclear cells,
amongst which are numerous plasma cells. Note decrease in number of
nerve cells. Note irregular disposition of nerve cells. From paretic
neurosyphilis.
]
Crown bald, with a slight fuzzy growth of short hairs. Scalp
slightly adherent to calvarium; latter of usual thickness but denser
than normal. Dura adherent to calvarium in region of vertex; dura
not remarkable. Sinuses normal. Arachnoid villi moderately
developed. Pia mater a trifle thickened and rather evenly throughout
the cerebral portion. Linear sulcal markings are remarkable for
their absence. The wall of the cerebellomedullary cisterna is thick
and opaque. The most prominent pial thickenings are over the
cerebellum. These are linear or may show feathery out-growths and
are seated over the sulci, particularly in the neighborhood of the
fissure and about the great cerebellar notch. They correspond fairly
well with the focal variation in consistence of underlying tissues
noted below.
=Brain= weight, 1265 grams. Consistence somewhat increased
throughout and somewhat evenly increased. The prefrontal region
shows the maximal increase of consistence but the remainder of the
frontal region and corresponding occipital region are much firmer
than normal. The two superior temporal gyri appear to be firmer than
adjacent gyri and are possibly slightly diminished in superficial
diameter. The hippocampal gyri are fairly firm. The substance on
section is a trifle more moist than normal. The gray and white
matter cut quite evenly. Diminution in depth of gray matter, if
existent, could not be demonstrated. The ventricles show a moderate
sanding throughout, best marked in the fourth ventricle. The basal
ganglia are not remarkable except for the development of numerous
dilated perivascular spaces about the lenticulostriate vessels. The
=pons= is atrophic, but more so on the right side. The pons, like
the prefrontal cortex, shows on section a distinct increase of
consistence immediately beneath the pia mater. The white bands of
the pons on section are distinctly firmer than the intervening
substance. The olives are of equal consistence. Weight of
cerebellum, pons, and medulla, 155 grams. The =cerebellum= shows an
obvious atrophic and gliotic process of a symmetrical character. The
superior surface, including both vermis and hemispheres, shows a
consistence above normal and general reduction of the depth measured
from the white matter. The reduction in depth gives rise to a
visible depression as compared with tissue posterior to the
postclival sulci. The lobus cacuminis, though slightly raised from
the surrounding lobes, is equally firm, if not firmer. The superior
and inferior surfaces show practically an equal increase of
consistence. The dentate nuclei are not especially increased in
consistence. The flocculi are reduced in size about one-third.
There was slight universal increase in consistence of =spinal cord=,
best marked in lumbar region.
=Microscopic findings= are here presented merely in sufficient
detail to establish the diagnosis. The left superior frontal gyrus
shows extensive and somewhat irregular cellular and fibrillar
gliosis of the plexiform layer, together with an increase of
thickened vessels having lymphocytes and plasma cells in their
sheaths.
The perivascular infiltrations are most extensive in the lower
layers of the cortex. The lamination is in places thoroughly
obscured, except that representatives of the layer of large external
pyramids are almost always demonstrable.
The layer of medium-sized pyramids has undergone more numerical loss
of elements than have the other layers.
Gliosis of white matter.
Specimens from the cerebellum show a destructive process of great
severity, but a little irregular in extent, affecting chiefly the
Purkinje cell belt. The Purkinje cells are often absent throughout
one side of a given lamina, and there has ensued a dense
accumulation of neuroglia cells along a former Purkinje cell belt,
together with a considerable gliosis of the molecular layer.
Considerable gliosis of the white matter, both diffuse and
perivascular in distribution.
Perivascular plasma cell infiltrations as in cerebrum, but largely
meningeal or in the white matter.
Sections from the corpora striata demonstrate a mild and early
granular ependymitis, considerable subependymal gliosis of cellular
type, considerable perivascular gliosis in the white portions of the
tissue, and a moderate infiltration of perivascular sheaths with
pigmented cells, lymphocytes, and plasma cells. There is little
evidence of alteration in the nerve cells. Some are unevenly
pigmented.
=Summary=: We here present a case with numerous and widespread
neurosyphilitic lesions. However, the gross cerebral vascular
complications of Case 1 (Alice Morton) and of Case 2 (Francis Garfield)
are notably absent in James Dixon. Rather atypical (there seems to be
_always something atypical in cases of neurosyphilis!_) are the liver
lesions and arteritis of the leg, atypical, that is to say, for PARETIC
NEUROSYPHILIS. Highly typical of paretic neurosyphilis and almost
constant therein is the aortic sclerosis.
[Illustration:
Apparent new formation of small blood vessel. Photographed by Dr. A.
M. Barrett.
]
[Illustration:
Rod cells (Stäbchenzellen) in paretic neurosyphilis. Photographed by
Dr. A. M. Barrett.
]
[Illustration:
Granular ependymitis—microscopic appearance of a marked example of
“sanding” of ventricle.
]
Characteristic and constant in paretic neurosyphilis is the
=Plasmocytosis and Lymphocytosis, Perivascular= in distribution about
small cortical vessels. There is also a characteristic (though
characteristically less prominent) =Plasmocytosis and Lymphocytosis,
Meningeal= in distribution. The pleocytosis of the spinal fluid, almost
constant though variable in amount in life, is an indicator of the
meningeal picture and less directly of the parenchymatous picture.
=Granular Ependymitis= (“sanding” of ventricle floors) is characteristic
and may be regarded as part of the parenchymatous picture. This
ependymitis is an indicator how chemical changes could be readily
produced at least in the ventricular fluids, since the limiting
membranes of the nerve tissue are here subject to multiple breaks. The
“sanding” is a neuroglia reaction to these multiple small breaks
(Weigert’s explanation).
Parenchymatous losses have led to =Atrophy and Sclerosis=, of very
varying extent in different parts of the encephalon. The atrophy is
characteristic in paretic neurosyphilis, but by no means constant.
Numerous cases have come to autopsy without clearly defined gross
atrophy. Sclerosis is also characteristic and even more frequent than
atrophy, doubtless because sclerosis represents an earlier phase of a
process eventuating in gross atrophy.
A =Tabetiform Picture= characterizes the spinal cord, but in this case
the tabetic clinical picture did _not_ precede the paretic clinical
picture. We are consequently to regard the tabetic spinal process as
incidental and on all fours with the =Cerebellar and Pontine Atrophy=.
=VASCULAR NEUROSYPHILIS (“syphilitic cerebral thrombosis”).
Autopsy.=
=Case 4.= James Pierce was an almshouse transfer to the Danvers Hospital
in his fiftieth year. He died three years later. The accompanying brain
pictures demonstrate so extensive a lesion of the left hemisphere that
it is of great interest to determine if possible the genesis and course
of his disease. It appears that syphilis had been acquired somewhere
about the age of 38 or 40, so that the total duration of the process was
between 13 and 15 years. In Pierce’s forty-third or forty-fourth year,
he had a shock while walking in the streets of his native city,
whereupon he was subsequently transferred to the Danvers Hospital, whose
data have been summed up as follows (we are obliged to Dr. Charles T.
Ryder for these data):
=Neurological examination:= Neuromuscular condition: Barely able to
walk or stand without assistance; hemiplegia of right side; swings
foot out and drags toe out and around in attempting to walk. Right
hand held by side, flexed at right angle; fingers contracted and
thumb thrown across palm. Can lift arm from side; practically no
movements of forearms or fingers; atrophy of deltoid, arm, forearm,
and hand. Muscular movements of left upper extremities fairly well
performed; good strength.
=Cranial nerves:= Refuses to respond to any tests to determine
hearing or vision, but evidently hears what is said to him, and in
his movements gives no evidence of deafness. Right corner of mouth
droops; tongue protrudes straight.
=Reflexes:= Pupils dilated; margins irregular; left pupil larger;
they vary in size but it is impossible to determine whether the
variation is due to light or accommodation reflex. Reflexes of right
side extremely exaggerated throughout; there is little ankle clonus;
Babinski is not obtained, patient holding his toes in flexed
position in resisting attempts to elicit reflexes.
=Sensations:= Reaction to pain stimuli on either side. Evidently
some anesthesia on right side, but pressure is apparently very
painful. There is considerable spasticity of limbs on right side on
passive motion. Too demented to make accurate tests.
The above examination was made on May 6, 1904. On May 20th the
record states:
There is almost complete sensory aphasia with word-deafness; some
paraphasic circumlocution. Many of his words are very well
enunciated but have no meaning. Is apparently unable to recognize
objects or their uses.
Brother stated that he was always supposed not to be over bright.
Physician’s certificate states that he is epileptic, averaging two
attacks per week. On the 15th of May he had a general convulsion;
was unconscious for half an hour, and dull and drowsy for two hours
afterwards. On the 19th, he had a similar attack in the afternoon,
the convulsion lasting a minute, and he was stuporous for an hour.
On November 8th he had a severe epileptic convulsion. His body was
curled up to the right. The convulsive seizure lasted for two
minutes and was followed by complete unconsciousness for an hour,
when the patient roused and appeared as usual in a few minutes. From
that time to December 15th he had five epileptic convulsions; he was
much more feeble, and unable to help himself as much as formerly.
Nov. 7, 1905: Patient has had occasional convulsions since last
note, but none during the last three months. He is confined to bed,
has become very much demented, and shows very marked speech defect,
so that he is almost unintelligible. He understands only the
simplest directions. Legs are considerably contracted and knees are
flexed. Arm and hand on the right are paralyzed and show some
atrophic changes; partially flexed. Left elbow jerk is very lively.
On May 23, 1906 he was reported as having Achilles on right side
only, and Babinski on right side. He died January 5, 1907.
=The autopsy findings= were as follows:
=Head:= Calvarium of moderate thickness; diploë present; dura
slightly adherent over bregmatic region. Longitudinal sinus contains
cruor clot. Dura is somewhat thickened and slightly more opaque than
normal. Pacchionian granulations, small but fairly numerous. Pia
contains throughout a considerable excess of clear serous fluid. The
convolutions in general are of good breadth and proportion. There is
an atrophic area roughly circular in outline and about 2 cm. in
diameter in the posterior part of the right third frontal
convolution corresponding to Broca’s area on the opposite
hemisphere. The space thus formed is filled with edema held by the
pia. On the left side is a similar subpial collection which covers
the site of the posterior portions of all of the third frontal
convolutions, parts of the lower end of the precentral convolution,
and the whole of the first temporal convolution, which have
disappeared entirely. The basal vessels show slight changes.
Cerebellum and basal ganglia are grossly normal.
The spinal membranes are negative. The regions of the pyramidal
tracts in the cord are firm, project slightly from surface of
section, and are china white.
=Summary:= Here is a picture made up almost purely of VASCULAR
NEUROSYPHILIS, with SECONDARY SPINAL (PYRAMIDAL TRACT) CHANGES.
Doubtless the genesis of this picture is allied to that of Case 1 (Alice
Morton) and to that of the terminal vascular complications in a tabetic,
Case 2 (Francis Garfield).
The absence of meningeal and parenchymatous (i.e., outside the region of
necrosis produced by the vascular disease) lesions is characteristic of
an important group of neurosyphilitic diseases. It is clear that the
case, although one of _extensive_ lesions, is _not_ one of _diffuse_
lesions in the sense of Case 1 (Alice Morton).
The spinal fluid picture in life may nevertheless show (as other cases
amply demonstrate) a certain amount of lymphocytosis and possibly
plasmocytosis, together with a variety of other changes. Treatment might
be expected to keep down these associated changes, although obviously
the effects of the necrosis are final and definite. Franz in Washington
has succeeded in “reeducating” some of these hemiplegics, employing
lower mechanisms of the nervous system.
[Illustration:
Vascular neurosyphilis—effects of syphilitic thrombosis of Sylvian
artery 10 years before death. (Case 4.)
]
[Illustration:
=Case 4.= (See previous figure for brain lesion.) Three levels of the
spinal cord showing unilateral pyramidal tract sclerosis, 10 years
after cerebral thrombosis.
]
=JUVENILE PARETIC NEUROSYPHILIS (“juvenile paresis”). Autopsy.=
=Case 5.= John Lawrence was an under-sized negro, who came under
hospital observation when he was 23 years of age. There was some
evidence that the patient’s father was a neurosyphilitic although
accurate data were out of the question. At all events, John had
Hutchinsonian teeth, a forward bowing of the tibiae, and
Argyll-Robertson pupils. These findings together with a history of
backwardness at school seem to stamp the diagnosis. It seems that there
had been a change for the worse from the age of 18, though the boy had
been able to sell newspapers and black shoes up to within a year of his
arrival at the hospital. During the last months of his life, he showed a
general incoördination, with false movements suggesting those of a
drunken person. There were numerous tremors, the glance was shifting,
and there was a tendency to nystagmus. Some of these phenomena (taking
into account that the Hutchinsonian teeth were not entirely typical and
there was even at times some doubt as to whether the pupils were
actually stiff) led to a question of the diagnosis multiple sclerosis.
There was, however, little doubt that the case was one of juvenile
paresis. Among the symptoms found at various times in this case are the
following: disorientation for time, place and persons, confusion, with
coarsely irrelevant replies to questions, ill-defined and transitory
delusions of persecution, auditory, tactile, and visual hallucinations,
and defective memory.
Early in life, the patient had had a habit of falling asleep in school
hours, and had experienced a number of falls at various times. During an
attack of measles he had had a number of spasms, each of which lasted
ten minutes or more.
The =autopsy= showed death to be due to an early bronchial pneumonia.
The thymus was persistent, measuring 3 × 2 × .5 cm. The marrow of the
femur was red.
There was a moderate degree of =sclerosis of the aorta= confined to a
few plaques in the arch (not a characteristic syphilitic scarring of the
aorta). The spleen was small and had a thickened capsule.
The majority of the lesions, however, were in the =nervous system=, and
the following description is taken from the routine hospital records to
exemplify the findings in a fairly characteristic case of JUVENILE
PARESIS.
=Head:= Scalp closely adherent to =calvarium=. Calvarium heavy
without diploë. =Dura= adherent to calvarium in bregmatic region.
Sinuses contain liquid blood. Arachnoidal villi in considerable
quantity. =Pia mater= contains considerable clear fluid and shows
diffuse haziness and focal thickenings. The diffuse haziness is
almost universal and is best marked over the superior surface of the
cerebellum. The focal thickenings are of general distribution over
the veins of the sulci on the superior surface of the brim and are
heaped up to form considerable linear mounds near the region of the
arachnoidal villi. The superior surface of the cerebellum is
traversed by similar linear mounds of fibrous tissue running at an
angle to the laminæ. There is no notable increase of fibrous tissue
at the base.
=Brain:= Weight 965 grams. The sulcation is roughly symmetrical
except in the occipital poles where there is unusually rich and
complex but shallow sulcation. The cortical substance is everywhere
firmer than normal, but the sulci fail to flare notably. In a few
places there is a focal increase of consistence of still greater
degree with apparent local hypertrophy (or gliosis with increase of
substance). These foci are in the right second temporal gyrus (3 cm.
in diameter) and in the left first temporal gyrus (of same size but
somewhat less firm) and are of a whitish, waxen appearance, being
visible several feet away by reason of their color and apparent
encroachment upon the adjacent sulci. The foci are sharply limited
by the sulci laterally, but pale out gradually before and behind.
The convolutions of the vertex show another type of lesion. The
tissue of the greater part of the vertex resembles that of the
flanks and base in being firmer than normal and of a grayish pink
color. Behind the fissure of Rolando on the right side and behind
the anterior limits of the ascending frontal region on the left side
the brain tissue of the vertex becomes suddenly still firmer and of
a yellowish gray color. This lesion disappears gradually into the
occipital microgyria behind and the gyri gradually lose their
yellowish tint. The lesion fades away gradually so that it fails to
involve the temporal convolutions.
The cerebral tissue cuts firmly and smoothly. The tissue of the
frontal region is a little edematous. The white matter is of a
normal appearance. The ependyma of all the ventricles is somewhat
sanded. The fourth ventricle is most affected.
The =cerebellum= is not edematous and is as firm as the normal
olivary bodies. The cerebellar hemispheres are symmetrical and of a
normal appearance, save that the laminæ are slightly narrower than
usual and very compactly set. The color, where not obscured by the
haziness of the pia mater, is of a grayish pink somewhat suggestive
of freshly tanned shoe leather. The substance cuts smoothly and
firmly. The dentate nuclei are unusually firm. The =pons= is small,
but of the usual color. Lower structures normal except the =cord=
which is small and shows curious deviations from the normal
markings. The posterior horns and gray commissure are at many levels
the only structures to preserve the normal gray appearance, so that
the H or butterfly appearance is replaced by a crescent. At these
levels, traces of gray matter often stand out in the loci of the
anterior horns.
The important =anatomical diagnoses= in the nervous system are as
follows:
Atrophy of cerebrum, 965 grams (there is of course a question whether we
are not dealing with a degree of cerebral hypoplasia).
Focal scleroses of cerebrum, suggesting the tuberous scleroses of
Bourneville.
Occipital microgyria.
Cerebral and cerebellar gliosis.
Chronic ependymitis.
Gliosis of the gray matter of the spinal cord.
Chronic diffuse and focal leptomeningitis.
The =microscopic examination= confirmed the diagnosis of paresis. The
hypertrophic nodules were of special interest. They were found to be
overlain by a characteristic though thin exudate of lymphocytes and
plasma cells, together with pigmented cells. The nodules appeared to be
supplied with an unusual number of vessels of small calibre, about which
were a few lymphocytes. The large vessels and those with well developed
adventitiæ were surrounded by more numerous lymphocytes and by more
focal accumulations of pigmented cells. The cortex in the middle of a
nodule had almost lost its characteristic cortical layering. The cortex
was here reduced (specimen from temporal lobe) to about one-quarter of
its normal thickness, and was found to be composed largely of expanded
neuroglia cells and vascular tissue, with a few nerve elements, small,
shrunken, and dark-staining. The destructive process appeared to have
borne hardest on the layer of internal large pyramids and the fusiform
layer. There was, however, nowhere any evidence of focal necrosis such
as ought to characterize a true gumma. The sections stained by the
Marchi method failed to show evidence of fatty degeneration within the
focus, although there was a marked diffuse accumulation of fatty
granulations along the nerve fibres in the underlying white matter. A
special study of the cerebellar material was made by one of the
authors.[4] Occasional Purkinje cells showed the characteristic
binucleate condition, which has frequently been noted in recent
literature.
The cerebellum of this case was perhaps the most markedly diseased of
all portions of the nervous system. As noted, the cerebellar tissue was
exceedingly firm. How far the notable incoördination of the case (he was
observed on staff rounds characteristically curled up in a heap, showing
quite an unusual degree of general incoördination) was due to the
cerebellar lesions, it is perhaps not possible to say.
=Summary=: John Lawrence, JUVENILE PARETIC NEUROSYPHILIS, is a foil to
Case 3 (James Dixon), paretic neurosyphilis due to acquired syphilis.
Both showed =Cerebral Atrophy=, but Lawrence the more markedly because
of hypoplasia incidental to the congenital origin of his condition.
Whereas Dixon gave little or no sign of =stigmata=, Lawrence (besides
being under-sized, having suspicious teeth, and showing at autopsy a
persistent thymus) showed a =Hydromyelia= and curious trefoil shape to
the spinal cord. Dixon on the other hand had liver lesions and arterial
lesions of the leg.
The suggestion of =Tuberous Sclerosis= in Lawrence is not found in
Dixon; but we have not found it elsewhere. Bourneville did not describe
tuberous sclerosis as syphilitic.
Binucleate Purkinje cells emphasize the congenital source of the lesions
in Lawrence.
=Plasmocytosis and Lymphocytosis=, =Perivascular=, and (less marked)
=Meningeal=, are found in both the congenital and the acquired cases, as
also parenchymatous changes, both =nerve cell losses= and =gliosis=.
Both also show granular =ependymitis=.
It is clear that, over and above the factors of destruction evident in
both Lawrence and Dixon, the congenital case, Lawrence exhibits also the
effects of arrest (in brief not merely atrophy but also hypoplasia).
Early treatment is, therefore, theoretically indicated in the juvenile
group, which means early diagnosis. Early diagnosis and treatment are
still more to be recommended because these juvenile cases progress often
very slowly at first.
=FOCAL BASILAR MENINGEAL NEUROSYPHILIS (“syphilitic extraocular
palsy,” plus other symptoms). Autopsy.=
=Case 6.= Flora Black, a housewife of 43 years, had been tired out for a
year but had been apparently in fair health. She awoke one day with
double vision due to a left internal strabismus. The visual difficulty
gradually passed away so that five months after the sudden seizure she
was apparently quite well again. There was one exception: about three or
four months after the attack of diplopia, Mrs. Black had begun to feel a
kind of weakness in various parts of the face and there were also fairly
definite paresthesiæ. In the sixth month after the initial attack, the
patient began to be unable to chew and was fain to support the lower jaw
with a bandage to aid in mastication. Deglutition was, however, quite
unaffected and there was never any regurgitation of food. There were
pains in the face, the forehead and the back of the neck.
Upon =physical examination= at entrance to a general hospital, no
changes in the body at large were discoverable. There was a slight edema
of the ankles, otherwise no sign of bodily disease.
Conditions in the =head= were as follows: The facial lines were (notes
by courtesy of Dr. E. W. Taylor) smoothed out; both upper and lower
eyelids and the corners of the mouth drooped slightly and more markedly
on the left side. There was slight photophobia and considerable
lachrymation. The patient was unable to pucker forehead, nose or mouth.
The unsupported lower jaw fell and the patient was unable to open the
mouth widely. The movements of the tongue were normally performed.
Speech was mumbling. Sensations of touch, heat and cold were preserved
all over the face except that the left cheek below the level of the
mouth yielded a less accurate registration of tactile sensations. A hot
test tube did not feel as hot in the lower left cheek as elsewhere.
Quinine and sugar could not be tasted over the left half of the tongue
in front. Smell and hearing were also diminished on the left side. It
appeared that there was a complete paralysis of the 5th and 7th nerves
and a partial paralysis of the 8th, 11th and 12th, as well as a defect
in smell.
The patient died suddenly, three weeks after admission, running a slight
temperature during her stay. The autopsy showed (rather surprisingly) a
double ovarian carcinoma with metastases into the retroperitoneal
glands. Both kidneys were found to be riddled with nodules of carcinoma.
The pelvic veins were thrombosed and there was a complete occlusion of
the pulmonary artery. There was a riding embolus in the foramen ovale
and there was coronary embolism.
The striking nature of these complications and the interest of the case
neurologically would warrant its publication in complete detail. We here
present the case with utmost brevity as an example of a SYPHILITIC
CRANIAL NEURITIS by extension from the meninges.
The =brain= was in general without change but there was a considerable
exudate over the entire =pontine region= which had involved several
cranial nerves. The 5th nerves, especially the left, showed gross
effects of the inflammatory lesion. There seems to be little or no doubt
that this neuritis was of syphilitic origin despite the complication of
the case with carcinoma of the ovary and despite the fact that the case
was observed and came to autopsy before the modern methods of systematic
diagnosis could be applied. It is the best case available to us for the
demonstration of a focal cranial nerve lesion of the type characteristic
of neurosyphilis. We may well suppose that similar conditions would have
been found at various stages in the development of Case 1 (Alice
Morton). The pontine region of Case 1 was entirely free from lymphocytic
exudate at the time of the autopsy. Possibly the clearing up of the
pontine pia mater in Case 1 was a therapeutic effect of the thorough
treatment therein used. Whether a case like Mrs. Black’s could be cured
(aside from the ovarian carcinoma and its complications) by the
institution of vigorous systematic treatment is a matter of doubt.
Still, in a general way, these cases of focal syphilitic neuritis are
among the most favorable cases for treatment.
=Summary=: We present the case of Flora Black to emphasize how slight in
extent and theoretically curable neurosyphilis may be. We fear that Case
1 (Alice Morton) may present too unrelieved and pessimistic a picture.
The extensive vascular lesions and complications of Alice Morton, of
Case 2 (Francis Garfield), of Case 4 (James Pierce) arrest attention by
the incurability of their residual effects (if we omit modern attempts
at reeducation of lower arcs). On the other hand the unrelenting
progress to destruction of important parenchymatous structures, as shown
in the paretic James Dixon (Case 3) and his juvenile replica John
Lawrence (Case 5), as well as in Alice Morton (Case 1) and the tabetic
Francis Garfield (Case 2), lead to a certain justifiable pessimism. For
it is only the meningeal and fine vascular infiltrations of these cases
that we can theoretically hope to combat, probably by destroying the
spirochetes in these meningeal and perivascular loci. We seem
theoretically less able to stop the progress of the often highly
systemic and symmetrical, parenchymatous lesions of the tabetic and
paretic group.
The condition in Flora Black is clearly much more hopeful, both being
more focal and being almost purely meningeal and therefore accessible to
therapy.
The two cases which conclude our general survey of neurosyphilis are
also focal cases, one of gumma (Lecompte) and one of focal dural lesion
(Wyman).
[Illustration:
1. Pons, normal except for focal infiltration of left fifth nerve.
]
[Illustration:
2. Higher power view of infiltrated left fifth nerve.
]
[Illustration:
3. Detail of infiltrated left fifth nerve, showing: 1, diffuse
infiltration with mononuclear cells; 2, perivascular infiltration;
3, strands of relatively unaffected nerve fibers.
]
Microscopic appearances in Case 6. Extraocular palsy (focal meningeal
syphilis, especially of left fifth nerve). Illustrates exquisite
focality of the syphilitic process sometimes found, as well as its
unilaterality (giving rise to asymmetrical symptoms and signs). Process
in itself probably curable.
=GUMMATOUS NEUROSYPHILIS (“gumma of brain”). Autopsy.=
=Case 7.= Mrs. Lecompte was a woman of middle age, who, according to the
history given by her son, had been entirely well until her final
illness, which began approximately two years before admission to Danvers
Hospital. The beginning of her trouble seemed to be chiefly headaches,
which would last continuously for several days, or more than a week at a
time. These headaches lasted throughout the course of the disease. In
the morning, on arising, she would feel very dizzy, but this would pass
away during the day. She had had a number of spells of unconsciousness,
lasting about fifteen minutes. In these attacks she would breathe
heavily, there was frothing at the mouth, twitching of the hands, and
the eyes would roll about. Her memory failed gradually, her disposition
changed and she became very irritable. Vomiting occurred almost every
day, and at times was of a projectile character. She became
hallucinated; the hallucinations were chiefly of a visual nature.
About four months before admission to the hospital, after one of her
seizures, the entire right side was found to be completely paralyzed,
and she complained that it was numb. At this time, she had difficulty
with her speech. In a few days, however, she was able to talk correctly
again, and in a week she was back at work, although the right side was
weak and awkward. She continued to grow worse, and then began to have
spells lasting several days, so that it became necessary to have her
placed in a hospital.
On admission to the hospital, aside from obesity, the general viscera
showed no points of special interest, and there was no evidence of any
new growth outside of the nervous system. She was unsteady on her feet,
standing with them wide apart. The gait was quite ataxic; the whole
right side was weaker than the left and used more awkwardly. There was a
paralysis of the right side of the face; the right angle of the mouth
drooped; the right eyelid could not be closed but remained continuously
open; nor could the right side of the forehead be wrinkled. Vision and
hearing were not affected. She miscalled tastes and smells; whether this
was due to aphasic difficulties or to cranial nerve involvement could
not be divined. There seemed to be some difficulty in deglutition. The
knee-jerks were markedly exaggerated; slight clonus was obtained but was
not always present. Both pupils reacted well to light and distance and
consensually. Sensation could not be readily tested. There was marked
ataxia, especially with the eyes closed. The speech was thick and
mumbling. The patient was unable to write or copy. Mentally the patient
was quite dull; at times, stuporous; when aroused, was found to be
entirely disoriented. Memory almost entirely absent. In general she
showed herself to be very much confused.
She remained practically in this condition, even gaining in weight, for
the following two years, when suddenly one morning, she had an epileptic
seizure, vomited, coughed a great deal, with bleeding from the mouth and
ears, and died in a few hours.
The symptoms in this case pointed to brain tumor. The only inconsistent
thing was the long-continued life,—four years,—after the symptoms were
observed. As she lived before the W. R. and spinal fluid tests were
known, no light was gained in these ways. The post mortem examination
showed the patient had a GUMMA OF THE BRAIN.
The =summary of the anatomical diagnoses= at autopsy was:
Decubitus.
Lymphadenitis of the mesenteric nodes.
Chronic fibrous peritonitis.
Chronic fibrous myocarditis.
Pulmonary hypostasis.
Thrombosis of vein in right adrenal, with hemorrhage.
Syphilitic leptomeningitis.
Gumma of left hemisphere.
Focal softenings in the pons.
The =anatomical description of the head= (Dr. A. M. Barrett) is as
follows:
The sutures in the =calvarium= are well outlined; diploë large in
amount. The =dura= is diffusely but lightly adherent to the
calvarium; it is very dense, especially over the left hemisphere.
The meningeal arteries are thickened but not atheromatous. The
sinuses contain a small amount of fluid blood and post mortem clot.
The inner surface shows nothing abnormal. There is a great
flattening of the convolutions of the left hemisphere, which is not
the case on the right side. Over the convexity, the =pia= is thin
and not abnormal except for some slight adhesions between the
frontal lobes and the two lips of the Sylvian fissures. The pia at
the base over the cisterna, pons, and medulla is thick, cloudy, and
of a grayish gelatinous appearance. It is so thick that it is easily
removable in a large piece.
The surface of the left hemisphere is dry, and the whole brain is
flabby and bulges as if from internal pressure. A section through
the hemispheres at the region of the optic chiasm shows a hard, firm
area in the left hemisphere deep down in the white substance. It is
about 2½ cm. in diameter, with a wavy border. The central part is of
a silver-gray gelatinous-like appearance, with red spots and whitish
streaks radiating from the centre. In the pons on the right side, in
a plane passing through the posterior corpora quadrigemina, are two
pinhead size softenings among the pyramidal fibres. The ependyma of
the fourth ventricle is granular.
=Microscopic examination of the tumor=: The area evidently contains
several central necrotic foci surrounded by zones of infiltration
and proliferation, with bordering areas of nervous tissue showing
secondary reactions. The necrotic area stains poorly. From the edge
there are projections of reddish homogeneous bands, some intermixed
with well-differentiated fibrillæ, probably glia fibrils. The
bordering zone is densely infiltrated with lymphoid, plasma, and a
few epithelial cells. The nerve tissue outside of this zone is
spongy and infiltrated with lymphoid and plasma cells. There are a
few scattered, shrunken nerve cells. In this zone and in the zone of
infiltration near the necrotic area, there are scattered cells
resembling giant cells. There are many obliterated vessels in the
area, and other vessels show many infiltrating lymphoid and plasma
cells in the walls. The examination of the specimen stained by the
methods for bacilli of tuberculosis are negative. The growth is a
classical gumma.
=GUMMATOUS NEUROSYPHILIS (gumma of spinal meninges, “meningitis
hypertrophica cervicalis of Charcot?”). Autopsy.=
=Case 8.= John Wyman was first seen in his thirty-sixth year by Dr.
James J. Putnam. He denied syphilitic infection and stated that the
first symptoms had come four months before. He had begun to notice a
numbness of the fingers, at first of the right hand and shortly
thereafter of the left hand. After a few weeks there had been difficulty
in walking, and a few weeks later headaches, especially on the right
side, developed. Two weeks before he was first seen medically, he had
begun to have a feeling of tightness or constriction in his arms.
It appears that micturition had been impaired early, that is to say, a
few weeks after the initial sensory disorder had begun. A catheter was
used for a time and improvement followed. Shortly before consultation
retention of urine developed again, this time associated with rectal
incontinence. The feet began to feel heavy and dead. Then the legs began
to be increasingly weak so that the patient was almost bedridden. Vision
appeared to be normal except that reading was followed by fatigue. The
speech was also slow but the slowness could be attributed to fatigue.
Notes of Dr. Putnam’s =physical examination= are as follows: The patient
lay in bed on the left side, without motion, and almost incapable of
motion. The tongue was protruded, and there was no paralysis of facial
muscles, or of the eye muscles (the right pupil had been reported to be
slightly larger than the left). There seemed to be a disinclination to
move the head, but with some effort it could be moved, and without pain.
The arms and hands were held rigidly in median positions; many movements
were possible, but all were imperfect and of slight amplitude. The
fingers were flexed to a moderate degree, and could not easily be
straightened, and there was, in fact, a general rigidity of most of the
muscles of the body below the neck, and even, in some degree, of the
neck. The immobility was so great that the general impression made was
almost that of a patient with fracture of the spine in the cervical
region. Even the breath, and especially the inspiration, was imperfect.
The legs were more freely movable than the arms, but still the motions
were very stiff and awkward, and of slight amplitude; with effort the
whole leg could be lifted from the bed, and flexed or extended with
moderate force. The right leg was rather stronger than the left, but the
left hand and arm were stronger than the right. The sensibility was
almost absent over the hands and lower part of the arms, and was
impaired over the entire head and neck, except the forehead, the middle
part of the face, and the nose. It is interesting to compare the
conditions of the sensibility here present with those seen in cervical
syringomyelia. The sensibility of the upper part of the forehead was
less good than of the lower part, and there was slight impairment even
over portions of the lower jaw. The sensibility of the left (stronger)
arm was rather more impaired than that of the right arm, while on the
contrary the sensibility of the left leg was better than that of the
right leg, though the difference between them was not great. These
statements apply to sensory tests by contact, heat, cold, and pricking.
Knee-jerks were highly exaggerated, and likewise the wrist-jerks. All
forced attempts at movements were attended by a high degree of muscular
tremor, especially when the patient was fatigued or under emotional
strain. The fingers especially were the seat of coarse tremor.
The remainder of this clinical description (courteously supplied us by
Dr. Putnam) may be quoted. A second examination which included also a
few facts not given in the first examination was made on the following
March 28, 1905. This report says “the ends of the fingers became numb
about June 1, 1904. Work was given up on July 3, and at that time the
patient was walking very badly. No treatment was used and no
satisfactory diagnosis made. In the course of July he improved somewhat,
and during August he was able to ride out a little (these spontaneous
improvements are of interest for the diagnosis). He went away from home
for a short time, but from the time of his return, about the last of
September, he grew worse rapidly, and fell into the condition above
described, in which he was wholly unable to help himself, even to
turning in bed. At times he had a great deal of pain in the neck and
forehead. Antisyphilitic treatment was recommended, and for a time
potassium iodid and other iodid preparations were given, but at first in
relatively small doses (grs. 75 daily). Under this treatment the
excretion of urine rose to four quarts daily as a maximum though
sometimes the quantity was not so great.”
Under this treatment the patient began soon to improve, and continued
doing somewhat better till about five months later. He became able to
walk downstairs and out of doors, and regained considerable use of his
hands. The quantity of urine passed became greatly increased by the use
of the iodid.
About the middle of March he became worse again. A careful examination
of the sensibility showed that in general the condition was much the
same as that previously reported. The iodid treatment, with perhaps some
mercurial, was resumed; the potassium iodid was given in doses which
were increased up to 850 grains daily, although this maximum dose was
taken only for about one week. This large quantity gradually impaired
the sense of taste for the time being, and blurred his vision, but
otherwise did him no harm. Under this he improved, so that he became
able to run more or less, and went about freely, and attended to his
business, though still retaining some stiffness in his movements.
This improvement continued until about two years later, when he again
had a relapse, and was seen medically once more. His condition at this
time was still a pretty good one, but the movements were stiff and
awkward. The bin-iodid of mercury was advised, which was taken in doses
of 9⁄25 grain daily. It will be remembered that this was long before the
days of salvarsan treatment.
This was toward the end of June, 1907. Contrary to expectation, there
was no material gain from this treatment, and the patient died early in
October, without being seen again.
The =autopsy= was limited to the =nervous system= and the findings were
as follows (Dr. A. R. Robertson):
=Head=: Hair abundant, fair, of fine texture and rather curly. Scalp
of medium thickness and strips readily from calvarium. The latter
appears normal and upon removal is of about the normal thickness. It
lifts readily from the dura mater, except for the numerous
attachments of Pacchionian granulations.
=Meninges=: The dura is smooth, moderately injected and shows no
areas of thickening; it lifts readily from the pia-arachnoid. The
pia-arachnoid shows discrete and in many places diffuse areas of
opacity. There is a moderate amount of subpial clear fluid and the
vessels are moderately injected. Over the anterior surface of the
medulla and lower portion of the pons and largely confined to the
right side there is a very marked thickening of the pia-arachnoid to
which the dura is densely adherent. This thickening extends down
anteriorly and laterally on the right side over the upper part of
the cervical cord. The thickened meninges over the upper part of the
medulla completely surround the right vertebral artery, shortly
before it joins its fellow of the opposite side to form the basilar.
Dissection of the arteries shows them to be patent and thin walled.
Over the =cerebrum= and cerebellum the pia-arachnoid strips readily
leaving a smooth surface. Section of the cerebral cortex, basal
nuclei, pons and cerebellum show no gross lesions. The ventricles
are moderately distended with fluid. The ependyma contains numerous
small cysts. Section of the =pons= shows no lesions of the nervous
tissue, but very marked thickening of the surrounding meninges as
noted above.
=Cord=: Throughout the cervical and dorsal region the dura is quite
tensely distended with an abundance of clear, light, straw-colored
fluid. Upon snipping the dura this fluid escapes with a small spurt,
as if under considerable pressure. The cord within, for the most
part, lies free, but over the upper three or four centimeters of the
cervical portion it is densely adherent to the dura anteriorly and
laterally on the right side. Cross sections were made through the
upper three or four centimeters of the cord, and over this area the
cord is constricted by very marked thickening of all the meninges.
The meninges here average from one to three millimeters in
thickness. On the right side and somewhat anteriorly opposite the
junction of the atlas and axis there is a single nodular, firm mass
which on section shows a yellowish, firm center surrounded by very
dense, pearl-gray tissue. The demarcation between the homogeneous
yellowish centre and its surrounding gray tissue is very sharp. This
nodule measures about 0.75 to 1 cm. in diameter. The adjacent cord
is deeply indented by it. Below this nodule there is a translucent,
grayish appearance of both posterior sensory columns which extends
downwards and diminishes in intensity until it finally disappears in
the upper dorsal region. This same appearance is well marked on the
right outer margin of the upper cervical cord corresponding to the
crossed pyramidal tract, and extends downwards diminishing in
intensity until it disappears about the mid-dorsal region. The left
pyramidal tract appears to be similarly but very slightly involved;
section of the lower dorsal cord entirely negative.
=Microscopically=, characteristic GUMMA.
It is a question whether this case is one of the group described in 1871
by Charcot under the name of _pachymeningitis cervicalis hypertrophica_.
Charcot did not regard his new disease as syphilitic, and it is very
probable that syphilis is not responsible for all cases. Charcot,
however, noted that his new disease was not incurable: he noted that the
resulting paraplegia, although it might be very marked and accompanied
by flexion of the leg on the thigh and although the paraplegia might
have lasted a very long time, might end in recovery. Charcot thought
that surgical intervention was necessary. He described three periods in
the disease, the first or neuralgic (pseudo-neuralgic) was characterized
by sharp pains in the neck and by the sensation of constriction in the
upper part of the thorax. The second phase of the disease was, according
to Charcot, the paralytic phase, in which a cervical paraplegia
accompanied by muscular atrophy developed. Sometimes cases were found to
remain in this paralytic phase and even to end spontaneously in cure. If
the muscular atrophy was degenerative, then the atrophy was never
replaced; but, according to Charcot, some cases of atrophy were simple
and accordingly curable. If, however, the spinal cord itself became
involved in the meningeal inflammation, then phenomena of transverse
myelitis set in with a spastic paraplegia and involvement of the bladder
and rectum. Muscular atrophy never developed in the legs, at least in
typical cases.
Among the causes of this condition the following have been mentioned:
cold, overexertion, alcoholism, tuberculosis and syphilis. Syphilis
undoubtedly plays the major part. Even before the days of the W. R.,
observers, among whom may be mentioned Dejerine-Tinel and Pförringer,
discovered syphilis in nearly all sufferers from _pachymeningitis
cervicalis hypertrophica_.
It should be differentiated from caries of the spine and cord and
meningeal tumors. The spinal fluid examination makes this somewhat easy.
Antisyphilitic remedies are indicated, and should be tried even when the
etiology is obscure, if only as a therapeutic test.
But what have been thy answers? What but dark,
Ambiguous, and with double sense deluding,
Which they who asked have seldom understood,
And, not well understood, as well not known?
Paradise Regained, Book I, lines 434–437
II. THE SYSTEMATIC DIAGNOSIS OF THE MAIN FORMS OF NEUROSYPHILIS
=PARETIC NEUROSYPHILIS (“general paresis”) sometimes persistently
receives the diagnosis NEURASTHENIA simply through omission to apply
approved diagnostic methods.=
=Case 9.= Greeley Harrison, a man of 46, certainly looked like a
neurasthenic. He wanted aid for nervous indigestion of years’ standing,
headache, insomnia, nervousness, failing memory, and deafness. He
volunteered, in fact, that he had neurasthenia, and that he had been
treated for this by hypophosphites.
During the practically negative =physical examination=, Harrison
complained of headache and throbbing in the head, and during examination
of the abdomen felt much nauseated and proceeded to vomit rather
persistently. There were hemorrhoids.
=Neurological examination= showed that the left pupil was smaller than
the right, was irregular, failed to react consensually, and reacted very
slowly to direct light. For the rest, however, the neurological
examination was negative. On account of the nausea and vomiting, special
examination of the gastric contents was made, but nothing abnormal was
found.
=Mentally=, it was rather striking that the patient’s memory was quite
inaccurate both for remote and for recent events. His school knowledge
was very meagre. As for delusions, the only approximation thereto was
the patient’s continually dwelling upon his bodily symptoms.
Emotionally, he varied between depression and a sanguine attitude.
Although there was no symptom directly suggesting syphilis in the
Harrison case, the slightly abnormal pupillary reactions and the amnesia
warranted the suspicion of syphilis. The blood and spinal fluid both
proved positive to the W. R.; the gold sol reaction was of the “paretic”
type; there were 18 cells per cmm.; there was considerable globulin, and
an excess of albumin. On the whole, therefore, we felt entitled to make
the diagnosis GENERAL PARESIS. Why should not a careful observer have
considered syphilis seriously? Yet in our experience such cases are
frequently diagnosticated neurasthenia, thus entailing dangerous delay
in treatment (in this case, five years’ delay).
Going over the history of the case with still greater detail, we learned
that for a number of years past, there had been symptoms of a
neurological nature. For instance, five years before, at the age of 41,
the patient had been apparently overcome when working near a stove, and
went upstairs talking incoherently, but recovered shortly. Thereafter,
such spells occurred almost every month; later, more frequently; still
later, the attacks were associated with unconsciousness and amnesia.
Occasionally preceding the attack there would be twitching of the mouth,
jerking of the arms, and incoherent talk. Throughout these last five
years, in point of fact, the patient had been unable to do regular work,
had been given to much complaining, and had been far less efficient than
formerly. In short, it would seem that, with the improved technique now
in the possession of medical science for the diagnosis of general
paresis, cases like that of Harrison will be diagnosticated earlier and
earlier.
1. How typical is the insidious onset of symptoms in the case of
Harrison? The onset of symptoms in neurosyphilis is ordinarily
considered to be sudden, and this statement is generally true
despite the fact that after the diagnosis is established a number
of mild prodromal symptoms can be remembered by the relatives.
However, some cases, of which Harrison is an example, have an
exceedingly insidious onset without sudden access of striking
symptoms. Joffroy and Mignot remark that with the improvement of
clinical methods, the course of paretic neurosyphilis must now be
stated to take some six or seven years for completion. In point of
fact, there were early episodic symptoms (seizures almost monthly)
which should not have escaped medical attention. They did escape
medical attention, however, and Harrison was wont to say “Why
wasn’t I told that my disease was syphilis five years ago?”
2. Is there such a disease as syphilitic neurasthenia? According to
Kraepelin, syphilitic neurasthenia has been described as occurring
shortly after infection and in the first stages of syphilis. There
are milder and severer forms; the milder forms show discomfort,
difficulty in thinking, irritability, insomnia, cephalic pressure,
indefinite variable, uncomfortable sensations, and pains. The
severer cases acquire anxiety, more pronounced emotional disorder,
dizziness, disorder of consciousness, difficulty in finding the
right word, transient palsies, pronounced sensory disorders,
nausea, and increase of temperature. Kraepelin is in doubt whether
there is any definite clinical picture of this sort, and whether
there is any causal relation between the syphilitic infection and
such symptoms as those described. If the effect of knowledge
concerning infection is a merely psychic effect, then it is
improper to term the neurasthenia in question a syphilitic
neurasthenia. For the relation of hysteria to the acquisition of
syphilis, see below the case of Alice Caperson (46). In point of
fact, modern work has shown even in the primary and secondary
stages of general syphilis more or less pronounced neurosyphilitic
phenomena in the shape of the so-called meningitic irritation of
French authors. (Besides the case of Caperson (46), see the case
of Fitzgerald and the discussions under these cases.)
3. What is the relation of the early symptoms of this case to the
so-called preparesis of Dana? The case might well have been an
example of Dana’s preparesis. For a discussion of this, see Case
of William Twist (13).
4. What is the classical differential diagnosis between paretic
neurosyphilis and neurasthenia? The testing of the blood by the W.
R. is unconditionally necessary. If the W. R. is negative, the
diagnosis of paretic neurosyphilis is extremely improbable. (It
must be borne in mind that a number of cases of paretic
neurosyphilis have been shown to have a negative W. R. in the
serum, and receive a proper diagnosis only after spinal fluid
examination.) Next to the serum W. R. stand the pupillary and
aphasic symptoms. In the presence of Argyll-Robertson pupil or
even a slight speech defect, the diagnosis of neurasthenia must
certainly be made with caution if at all. Kraepelin remarks: The
sudden occurrence of neurasthenic disorders in a male of middle
age without any evident cause therefor is always suspicious. Yet
it must be emphasized that a complaint of occasional dizziness,
slight speech defect, tremor of tongue, and a moderate increase of
tendon reflexes do not possess any marked diagnostic significance.
Clear insight and understanding of the nature of the disease
phenomena, a persistent search for recovery, reasonableness in
conversation, progressive improvement under appropriate treatment,
speak for neurasthenia.
Joffroy and Mignot differentiate what they call preparetic
neurasthenia from other neurasthenic states, not only on the basis
of its etiology but on the basis of its symptoms. They also call
attention to the fact that neurasthenia, being a pure neurosis,
develops either on a manifestly hereditary basis or upon some
physical injury, weakening disease, or moral shock. The pure
neurotic suffers a great deal more than the patient who is
destined to become a victim of paresis. The character change in
neurasthenia does not amount to that entire transformation of
personality (even to the performance of criminal acts) that we
find in paretic neurosyphilis; at the most, the neurasthenic shows
minor emotional disturbances and a certain pathological egoism.
The psychotherapeutic test also rather readily dissipates many of
the neurotic, hypochondriacal fears and feelings. Although both
pure neurasthenia and the paretic pseudoneurasthenia are
characterized by sexual weakness, the sexual anæsthesia of the
preparetic is practically always preceded by a stage of sexual
over-excitement. These finer clinical indications, however, fade
into insignificance beside the data that can and should be
obtained from laboratory tests.
5. How exceptional is such a case as that of Harrison? We have in
our experience seen many patients with a similar course and
configuration of symptoms, although the majority of these cases in
a community advanced enough to provide easy access to a Wassermann
laboratory are now diagnosticated far earlier than was the case of
Harrison.
6. What attitude shall we take toward so-called syphilophobia? It
seems to us that resort to a serum W. R. is indicated, both from
the standpoint of the community and still more importantly from
the standpoint of the patient. We are even inclined to suggest for
a case of persistent syphilophobia, when the serum W. R. has
proved negative, a lumbar puncture. Syphilophobia must be
considered, not as a syphilitic psychosis, but as a phobia to be
classified among the psychoneuroses. It becomes a difficult
question to decide at times whether a patient who has had
syphilis, has had a considerable course of treatment and shows the
symptoms of a syphilophobiac should be further treated for
syphilis or merely for his phobia. We have seen recently such a
patient who gave a certain history of syphilis and who was greatly
disturbed lest he should be developing paresis. This fear bothered
him greatly. Examination showed irregular pupils, but no other
signs of syphilis. The W. R. in blood and spinal fluid was
negative as were the other spinal fluid tests. It was considered
wise to treat him only for his phobia and under this treatment he
was given some relief.
=PARETIC NEUROSYPHILIS (“general paresis”) may look precisely like
MANIC-DEPRESSIVE PSYCHOSIS.=
=Case 10.= The mental picture in Lyman Agnew, an architect, 58 years of
age, was wholly characteristic of manic-depressive psychosis. In the
first place, there had been (at 55) a previous attack of depression,
lasting a few months, from which Agnew had completely recovered. He had
remained entirely well up to four months before consultation.
(Manic-depressive psychosis is, at least in a majority of cases,
hereditary. There had been mental disorder in one maternal cousin, and
mental impairment in the patient’s mother some time before her death
from cerebral hemorrhage. There was no other report of mental disease in
the family.)
It appears that in the interval between attacks, Agnew had been working
very hard and had been fairly successful in paying off a mortgage on his
house. A marked elation, somewhat natural, followed this success and
continued to an abnormal degree. Agnew labored under considerable
excitement, was over-fussy, and at times showed a flight of ideas. His
mania or hypomania gradually diminished and depression set in, in which
depression he arrived for consultation. He had marked ideas of
self-accusation, was emotionally unstable, wept much, and showed a
characteristic retardation of activities and unrest.
=Physically=, there was no neurological disorder. The patient appeared
rather under-nourished. The heart borders lay 2 cm. to the right and at
11½ cm. to the left of the mid-sternal line. The aortic second sound was
very loud. There was a moderate radial arteriosclerosis. Systolic blood
pressure was 210, diastolic 155.
The high blood pressure suggested nephritis, possibly of
arteriosclerotic origin, but urine examination and blood-nitrogen tests
yielded no evidence of kidney disease. Moreover, it is our experience
that a manic-depressive psychosis in persons past middle life is not
infrequently complicated by high blood pressure. In point of fact, some
authors insist upon a relation between manic-depressive psychosis and
the arteriosclerosis which rather frequently sets in in this disease.
Routine examination of the blood serum, however, yielded a positive W.
R. Following the approved rule of making an examination of the spinal
fluid in all mental cases having a positive serum W. R., we proceeded to
lumbar puncture. The fluid was clear and contained 35 cells per cmm.,
the albumin was in excess, and there was a positive globulin reaction.
The gold sol reaction was of the “paretic” type; the W. R. was strongly
positive.
On this basis, it seems worth while to consider the diagnosis of GENERAL
PARESIS or that of some form of non-paretic neurosyphilis. The former is
the diagnosis which we prefer.
1. What is the classical differential diagnosis between
manic-depressive psychosis and neurosyphilis? The laboratory tests
have naturally supplanted the older purely clinical methods of
differential diagnosis. The difficulties lodge, in the first
instance, in depressive states. It would appear to be impossible
on purely clinical grounds in certain cases to tell the depression
of neurosyphilis from the depression of manic-depressive
psychosis, since the slightly greater interest in the outer world
taken by manic-depressive patients and their greater
responsiveness to diagnostic threats (suggestion that patient is
to be pinched or cut) are of no special value in the individual
case. Identical considerations hold for the maniacal phases of
manic-depressive psychosis, for these maniacal phases may even
develop delusions (Kraepelin) of precisely the same nature as the
characteristic expansive delusions of the excited paretic.
2. If the clinical symptoms are insufficient in differential
diagnosis, are not the pupillary signs and the speech defect of
greater value? They are of value if present, but as in the case of
Agnew, the victim of neurosyphilis may show no pupillary or speech
disorder. Instances are familiar, also, in which the pupillary and
speech signs are absent in very advanced cases of non-paretic or
even of paretic neurosyphilis.
3. Would not a circular course or recurrence of attacks be decisive
for manic-depressive psychosis? Paretic neurosyphilis sometimes
exhibits the same circular or recurrent course. We conclude that
neither the clinical symptoms, the classical pupillary and speech
signs, nor the ups and downs of a particular disease, are at all
decisive as between manic-depressive psychosis and paretic
neurosyphilis. Resort must be had to laboratory tests.
4. What is the significance of the high blood pressure in paretic
neurosyphilis? Work from our laboratory (Southard and Canavan) has
shown plasma cells in the kidneys in 17 out of 30 paretics (56%),
and in 16 of these 17 paretics with renal plasmocytosis, the
plasma cells were found in the periglomerular region. What the
relation of these findings may be to heightened blood pressure is
as yet unknown. The severe syphilitic involvement of the aorta so
characteristic in paretic neurosyphilis, as in other forms, may
possibly have a bearing on blood pressure.
=A POSITIVE SERUM WASSERMANN REACTION associated with mental
symptoms (even with grandiosity) does NOT prove the EXISTENCE OF
PARETIC NEUROSYPHILIS (“general paresis”).=
=Case 11.= Juliette Lachine came to a general hospital with pain in the
right upper quadrant of the abdomen, wherein was found an enlarged
liver. This liver was regarded as syphilitic on the ground that the
patient had a positive serum W. R. and that her two elder children were
clearly suffering from congenital syphilis. The liver mass was promptly
reduced by antisyphilitic treatment of the classical sort. When,
however, the patient was given an injection of salvarsan, she shortly
began to develop marked mental symptoms, whereupon she was removed to
the Psychopathic Hospital.
The =mental picture= at the Psychopathic Hospital was as follows: Lack
of orientation for time, marked distractibility of attention, with a
certain jumping from one subject to another, delusions of a religious
nature, claims of wonderful powers possessed by the patient, moods
variable, though as a rule of a euphoric and elated nature, with
laughing and singing. The activity seemed to be of a mental rather than
a peripheral nature. The patient did not regard herself as mentally
abnormal. The liver was still 4 cm. below the costal margin in the
nipple line. We found the W. R. to be positive in the serum but negative
in the spinal fluid. In fact, the spinal fluid was entirely negative.
So far as we are aware the picture presented by this case is one of
MANIC-DEPRESSIVE PSYCHOSIS. We regard the disease as merely complicating
the syphilis, although it is entirely possible that some visceral
condition incidental to the syphilis might be proved (in a higher stage
of psychiatric science) to have produced the mania.
In any event, the patient quite recovered from her mental symptoms in a
month. She was then able to tell us of a previous attack of depression
some 12 years previously, namely, at the age of 26. It appears that she
had at that time been committed to a hospital for the insane.
1. In this case, in which the diagnosis of manic-depressive
psychosis and not paretic neurosyphilis was made, are we sure that
the symptoms that we term manic-depressive psychosis were not
actually produced by syphilotoxins? In other words, in the absence
of spinal fluid signs of inflammation or chemical change, might it
not be possible for generalized syphilis outside the nervous
system to produce manic-depressive symptoms? There is so far in
the literature no experimental or other evidence of syphilotoxins.
The existence of products and substances permitting the W. R. and
the gold sol reaction is not of course evidence of syphilotoxins.
Although there is no evidence of soluble syphilotoxins, it is
thought that in the so-called Järisch-Herxheimer reaction (the
intensification of clinical symptoms after salvarsan injection)
effects may be due to the liberation of products from the killed
bodies of spirochetes. Such endotoxins are not here in question.
2. Is visceral syphilis, such as gumma of the liver, able to produce
characteristic syphilitic reactions in the spinal fluid? We have
had an autopsied case in which there was a “paretic” gold sol
reaction of the fluid (though without other signs). The autopsy
showed gummata of the liver. However, the finer anatomy of the
nervous system showed a mild but definite meningo-encephalitic
process, which was doubtless responsible for the gold sol
reaction.
3. What is the value of grandiose ideas? Ballet distinguishes two
groups of grandiose ideas: (_a_) ideas of self-satisfaction,
including ideas concerning extraordinary capacity, strength,
power, and wealth on the part of the patient; and (_b_) ideas of
ambition; the latter being of a more exact, constant, uniform and
systematizing nature. The more vague and less systematized ideas
of self-satisfaction rest in a phase of contentedness and
optimism; the more definite ideas of pride and ambition are
responsible for striking transformations of personality. General
paresis shows, according to Ballet, these ideas of
self-satisfaction in their most developed form. A certain
variability, absurdity, incoherence, and contradictoriness
characterize these ideas and the patient has little or no insight
into their nature. When such ideas occur at the outset of the
disease, they naturally may be of medicolegal interest. Cotard
explains these ideas of megalomania on the part of paretics on the
ground that they are essentially motor or will disorders and rest
upon a sort of hyperbulia, exhibiting itself in exuberant
activity. Régis has thought that the delusional generosity and
liberality of the paretic, and his willingness to lend his wealth
and talents to social progress, is helpful for diagnosis when
contrasted with the more personal egoism of the victim of
manic-depressive psychosis. The self-satisfaction of the
manic-depressive patient often does not reach a delusional stage,
but remains a mere feeling of pathological well-being or euphoria.
The maniacal patient may compare himself with some great man but
he does not identify himself with him. It must be remembered that
these ideas of self-satisfaction occur also in alcoholism, but
according to Ballet they occur only in the dementing phase of
chronic alcoholism, and have no special diagnostic value. They may
be a clinical stumbling-block for a time in the cases of alcoholic
pseudoparesis. As for the ideas of ambition in which the patients
believe themselves to be princes, emperors, divine messengers, and
the like, these are less characteristic of paretic neurosyphilis
than of delusional psychoses of a non-syphilitic nature. At all
events, such ideas if definite, of long-standing, and systematized
by the patient to form a thorough-going portion of his life, are
not characteristic of neurosyphilis. The victim of paretic
neurosyphilis can as a rule be persuaded out of his delusions, at
least for the time being. These distinctions, it must be added,
are hardly of value in the early cases of any of the psychoses in
question, and cannot be made as a rule in either private or
psychopathic hospital practice. Typical examples of grandiosity,
although not so frequent as might be thought from textbooks, are
always on display in institutions for the chronic insane.
=PARETIC NEUROSYPHILIS (“general paresis”) may look precisely like
DEMENTIA PRAECOX. Autopsy.=
=Case 12.= Henry Phillips remains a striking case in the memory of those
who knew him and his medical findings. Phillips came to the hospital
voluntarily at 42 years of age from the bank where he worked as a clerk;
he came at the suggestion of his employer. It seems that he had been
annoying his associates because he had fallen into a habit of
continually scratching himself. Phillips was entirely sure that he was
the victim of what he called the “Scotch itch,” and explained off-hand
that this itch had been put upon him by the Free Masons as a matter of
revenge because he would not join their order. He said once, for
example: “At times I feel like raising Hell; then I get a psychic
intimation; and then I get to using a foot-rule on my back and to
slapping my face.” He explained this psychic intimation as coming from
the order of Scottish Rites. Another example of talk is as follows: “My
father is a fighting man; that is part of it. They mean to throw me
down. I am through now trying for membership in the Free Masons. They
have good cause, they must fight. They do not want me for some personal
matters. I can go just so far in agreeing and seconding their advances,
but in the end it fails. I have no strength nor endurance.”
Aside from these delusions, there was little abnormality to be found,
though his recollection for minor events of the immediate present was
inaccurate. He was rather abnormally impulsive, gesticulating a good
deal while talking, and was of the appearance that the laity call
“nervous.” It appears that he had always been peculiar, subject to
violent fits of temper, in which fits he might throw things at other
members of the family. He always had pronounced likes and dislikes which
he never concealed. He had never had friends, had always been secretive;
and he was often termed a great student. For some five years he had been
studying Japanese from time to time, associating himself with a
Japanese.
It never does to jump at the diagnosis dementia praecox. However, the
picture seemed characteristic enough for the paranoid form of this
disease. Physically, Phillips had no particular abnormality; the
knee-jerks were a little lively, and the pupils reacted a little
sluggishly. However, the routine W. R. of the serum proved to be
positive. Examination of the spinal fluid was resorted to,—as in all
cases with a positive serum W. R.—and it also proved to be positive and
strongly so; the globulin and albumin were increased, and there was a
pleocytosis. A diagnosis of neurosyphilis was hardly avoidable. Phillips
later admitted a chancre, which he claimed was located on the mucous
membrane of the cheek and acquired by using the same utensils as his
Japanese friend, which friend, he stated, had active syphilis.
Antisyphilitic treatment of considerable intensiveness was begun, with
intravenous injections of salvarsan and intraspinous injections of
salvarsanized serum, but the patient grew steadily worse. His mental
symptoms became more marked, although not especially characteristic of
general paresis. =Neurologically,= he did develop signs more suggestive
of general paresis, and 18 months later died.
The =autopsy= showed features of GENERAL PARESIS. It is not necessary to
enter into the question of the details of histological correlation at
this time.
1. What conclusion can be drawn from lively knee-jerks? Lively
knee-jerks are of very little significance. Not only certain
neurosyphilitics but also a variety of neurotic persons, victims
of dementia praecox and hysteria, are very prone to have active
tendon reflexes. Of course, extreme degrees of exaggeration are of
importance, and especially an association of the hyperreflexia
with the Babinski reaction, the Gordon, or Oppenheim reflexes,
ankle clonus, and the like.
2. Is there any special or differentiating factor in an extragenital
chancre as against a genital chancre? Probably this question
should be answered in the negative. Some have claimed that
chancres draining by lymphatic channels of the head are more
likely to lead to cerebral syphilis. This idea cannot be said to
be established.
3. Is there any significance in the story, if true, that Phillips
acquired his syphilis from a Mongolian? It seems to be fairly well
established that syphilis of the nervous system is extremely rare
in China and Japan, whereas bone syphilis is very frequent there.
It has been held that this has to do (_a_) with strains of
spirochetes, (_b_) with the state of civilization, or (_c_) with
the degree of “syphilization.” Apparently when a race is first
infected with syphilis the lesions are chiefly of the cutaneous
and osseous systems; only in later generations the vascular and
nervous systems suffer. However, involvement of the nervous
systems of Mongolians resident in this country is no rarity, a
point possibly in favor of the theory of special strains affecting
the nervous system as prevalent in western countries. Little or
nothing is known as to the effect of transmission from one race to
another, as from Mongolian to Caucasian in Phillips’ story.
=NEUROSYPHILIS is NOT to be entirely ruled out by a negative serum
Wassermann Reaction; for the fluid Wassermann Reaction may be
positive.=
=Case 13.= William Twist is a case of note in the matter of the
so-called preparetic period (the idea of Charles L. Dana which was
scoffed at when first proposed by him in 1910). The patient, a very
successful traveling salesman, 35 years of age, was admitted to the
Psychopathic Hospital showing a typical picture of general paresis.
Thus, =mentally=, the patient showed elation, grandiosity (millions of
dollars to give away), intellectual weakness, disorder of memory, lack
of judgment, rambling talk, speech defect, omission of letters in
writing and spelling.
=Neurologically=, there was tremor of the lips, slight irregularity of
the pupils, which however reacted well, and lively knee-jerks.