{1024} lesion is undoubtedly the direct outcome of a syphilitic
diathesis. In these cases the exudation commences primarily in the
membranes of the cord, and may extend into the cord itself. In this
class I would include the first two varieties of syphilitic spinal
disease of Huebner. The number of recorded autopsies is not great; the
only cases with which I am acquainted are those referred to in the
note at the bottom of this page.[73]
[Footnote 73: Winge (_Dublin Med. Press_, 2d Series, vol. ix., 1863);
Moxon (_Dublin Quarterly Journ._, li., 1870); Charcot and Gombault
(_Archiv. d. Physiologie_, tome v., 143, 1873); Schultze (_Archiv.
Psychiat._, xii. 567); Thos. Buzzard (_Diseases of Nervous System_,
1882, p. 407); Julliard (_Étude Crit. sur les Localis. Spinal de la
Syphilis_, 1879); Westphal (_Arch. Psychi._, vol. xi.); Greif (_Arch.
Psychiat._, xii. 579); Homolle (_Progrès méd._, 1876).]
The lesions in these cases are entirely similar to those of brain
syphilis. The disease very rarely or never begins in the interior of
the cord. I know of no recorded case: Wagner's case, in which a yellow
nodule was found within the cervical marrow, was probably not one of
syphilis. If a gummatous inflammation does occur inside of the cord,
it probably starts from the ependyma. The gummatous exudate may occur
in the form of small multiple formations or of an extensive
meningitis, with an infiltration of the membranes and their spaces
with gummatous material. The membranes are usually agglutinated with
one another and with the surface and with the cord. The exudation is
usually made up of roundish cells, and in several cases spindle-shaped
cells have been noticed, as have also the peculiar Deitres corpuscles
already described as they occur in brain syphilis. The changes in the
cord itself vary somewhat. In Winge's case the white matter seems to
have undergone a rapid myelitis from pressure. It was of a grayish
color, with numerous fine granular masses, corpora amylacea,
pigment-masses, and fatty globules, the nerve-fibres being broken up.
In other cases the change has been a sclerosis. The vessels of the
cord have been noticed by various observers in the different stages of
the degeneration seen in syphilis of the brain. They are often greatly
dilated, their walls thickened, and, together with the lymph-spaces,
infiltrated with small cells. Minute hemorrhages have been found.
The so-called syphilitic callus, as described by Heubner, is probably
the remnant of a true gummatous inflammation. It consists of a
circumscribed induration one to several lines in thickness,
originating apparently from the dura mater, and causing sometimes
adherence with the vertebræ, in others with the membranes of the
spinal cord. In a case described by Virchow of this character the
lesion was cervical, and the symptoms were stiffness in the nape of
the neck, pains in the neck and arms, and finally paralysis in both
arms. A second case is elaborately described by Heubner in his article
in _Ziemssen's Encyclopædia_.
SYMPTOMS.—As the lesion of gummatous spinal syphilis affects primarily
membranes of the cord, in the beginning of the attack the symptoms
chiefly arise from the implication of the nerve-roots. Of course these
symptoms vary with the seat of the lesion, for it must be remembered
that the meningeal irritation is at first usually localized in a small
region. As in a majority of cases this lesion affects a posterior
portion of the cord, and as the posterior nerve-roots seem especially
sensitive to irritations of this character, pain is usually a very
marked and {1025} precocious symptom of spinal syphilis. The seat of
the pain varies with the seat of the lesion. At first the pain is
slight, but in most cases it soon becomes severe. It is sometimes
situated at a fixed spot on the spinal column, where, according to
Heubner, it may be increased on pressure. I have seen two or three
such cases, but have and still do believe that under these
circumstances the patient was suffering not simply from a spinal
syphilis, but also from an implication of the vertebral periosteum or
of the vertebræ themselves. In one of my cases this diagnosis was
confirmed at the autopsy. When the lesion is purely meningeal there is
probably no marked local tenderness. The severe pains usually felt in
the extremities or in the trunk are often fulgurant; sometimes they
are described as resembling the thrust of a knife, and not rarely they
closely resemble the pains of locomotor ataxia. In some instances the
pains are comparatively slight and are aching in character.
Paræsthesiæ are not rare phenomena: such are formications, tingling in
the extremities, numbness and feeling as though the limb were asleep,
intense sense of coldness on the surface, sensation of water running
over the limb. Early in the disorder there is sometimes very marked
hyperæsthesia, but later, even though the pain persists, blunting of
sensibility is marked, and there may be a complete anæsthesia. This
anæsthesia is sometimes localized in certain parts of the limb. Thus,
in a case reported by Alfred Mathieu,[74] although there was complete
anæsthesia of the outer side of the left leg and foot, the inner side
retained its normal sensibility. In some cases there is the abdominal
cincture of ordinary myelitis. The records show that even in these
early stages there may be diplopia, amblyopia, or other disorder of
vision, and the pupil may be distinctly affected. In these cases it is
probably the upper portion of the cord which is affected.
[Footnote 74: _Ann. de Dermatol. et Syph._, vol. iii., 1882.]
Disturbances of motility in the majority of cases do not develop until
some time after sensation has been affected, but may come on very
early. Usually, the first symptoms are those of irritation, such as
rigidity of the neck, back, and limbs or even of isolated groups of
muscles. Tremors have been described as frequently present. These may
be convulsive, and are often plainly reflex in their origin; indeed, I
am inclined to believe that they are always reflex tremblings, and
never true tremors. Heubner describes a case in which a paralyzed limb
was thrown into violent tremblings whenever passive motion was
attempted. The patella-reflex is usually grossly exaggerated, although
it may be lost in the later stages of the disorder. Not rarely there
is the condition which has received the misnomer of spinal epilepsy.
This exaggeration of the reflexes may be limited to one leg, when it
is almost pathognomonic. In some cases severe cramps are excited by
movement. Usually there is no tenderness. These symptoms of the
meningitic stage may continue for weeks or months without there being
pronounced paralysis, although locomotion is not rarely interfered
with by the stiffness of the legs. Finally, if the case progresses the
patient notices a weakness in one or both legs, or (if the disease be
situated high up in the spinal cord) in one arm, which rapidly
increases until there is almost complete loss of power. This rapid
increase of palsy following long-continued disturbance of sensation is
almost pathognomonic. In most cases one side of the body is more
{1026} affected than the other. The sphincters are prone to be
implicated, and in advanced stages of the disease there is usually
complete loss of control over the bladder and rectum. The patient may
live for months without very distinct change of this condition, or
bed-sores and other trophic disturbances may rapidly develop and death
ensue in a short time. I have seen under these circumstances marked
elevation of temperature, rapid feeble pulse, mental weakness, and the
general symptoms of septicæmia last for many weeks. Ammoniacal
cystitis is of course prone to be developed during this stage. When
motility fails, sensibility is usually blunted, although the pains may
even increase. Heubner affirms that an incompleteness of the
anæsthesia is characteristic of the disorder.
The typical course of spinal syphilis, such as has been described, may
be variously departed from. Sometimes the power of co-ordination is
early affected, and the symptoms may resemble those of locomotor
ataxia. I doubt, however, whether under any circumstances there is a
loss of the patella-reflex in the early stages of the gummatous
disease of the spinal cord. In other cases the paralytic symptoms may
be very prominent from the beginning: thus, in the case of R. P——,
aged 27, which I believed to be gummatous disease of the spinal cord,
the first disorder was a feeling of malaise lasting for about a week,
followed by the sudden, rapidly-developed paralysis of the bladder,
loss of power in the legs, and to a less extent in the arms, the only
pain being a dull, steady ache in the arms. The bowels were
obstinately costive. Double vision was soon very pronounced. When I
first saw the patient, about three weeks after this, there was decided
impairment of sensibility in the legs, but not in the arms; marked
muscular weakness of both legs and arms; no loss of co-ordinating
power; dropping of the right eyelid, with double vision; and only some
slight aching pains in the arms. By the use of large doses of iodide
of potassium and other appropriate measures a good recovery was
secured.
A case illustrating the occasional difficulty of diagnosing spinal
syphilis is reported by C. Eisenlohr.[75] The first symptom was
obstinate constipation, with very great discomfort after defecation;
then appeared incontinence of urine with weakness of the legs:
finally, a sudden complete palsy of the right leg occurred, with
marked anæsthesia in both legs, partial loss of power in left leg,
violent boring abdominal pains, and distress in the bladder. In the
last stages there were severe neuralgic pains in both legs, with
complete loss of sensation, bed-sores, atrophy of the leg-muscles,
with reactions of degeneration, and death from exhaustion. At the
autopsy an advanced meningitis was found which had apparently
commenced in the regions of the cauda equina, and given rise to
complete degeneration of the nerves. The only alteration of the cord
was an ascending degeneration of the posterior columns.
[Footnote 75: _Neurolog. Centralb._, 1884, p. 73.]
Again, owing to the diseased condition of the vessels, a spinal
syphilis may be suddenly interrupted by an apoplectic accident.
In a patient of my own, who was believed to be suffering from
gummatous spinal meningitis, there was an abrupt development of
violent tearing pains, loss of power and sensibility, and all the
other symptoms which are characteristic of meningeal spinal
hemorrhage. A. Weber {1027} reports a case in which, after doubtful
premonitory symptoms, such as vertigo, loss of power on the right
side, pressure on the top of the head, and tinnitus aurium, there was
a sudden development of convulsions, and death. At the autopsy a
syphilome of the right vertebral artery was found with a recent
thrombosis of the basilar artery.[76]
[Footnote 76: _American Journ. of Neur. and Psychiat._, vol. ii.]
{1028}
TUMORS OF THE BRAIN AND ITS ENVELOPES.
BY CHARLES K. MILLS, A.M., M.D., AND JAMES HENDRIE LLOYD A.M., M.D.
DEFINITION.—Under the head of Tumors of the Brain and its Envelopes
will be considered all forms of growths occurring within the skull,
whether these involve the cranium itself, the membranes of the brain,
the brain-substance, or several of these parts conjointly. A large
majority of these growths spring from the brain-membranes.[1]
[Footnote 1: To this article will be appended a table of one hundred
cases of brain tumor. Our researches included the investigation of
many more cases, four to five hundred in all. Such a table, indeed,
could be indefinitely extended. Our object, however, has been not so
much to present a large number of cases, and these in great detail,
but rather in the most condensed manner to give a definite number,
carefully selected, from which to draw conclusions. The cases have not
been chosen with the view of upholding any peculiar or preconceived
views as to pathology, diagnosis, localization, etc., but because of
the carefulness with which they have been recorded. They have been
selected also, as will be seen, with the view of determining by
clinico-pathological data the possibility of localizing such growths
during life. Many of our general conclusions as to pathology,
symptomatology, and diagnosis have been drawn from this table.]
With the exception of such merely substitutional terms as cerebral or
intracranial growths, neoplasms, or adventitious products, we have no
general synonyms for brain tumors.
The literature of the subject of brain tumors is second only to that
of such subjects as syphilis and hysteria. Vol. ii. of the _Index
Catalogue of the Library of the Surgeon-General's Office, U. S. A._,
contains no less than 632 references to this subject: number of books,
43; articles, 589. The books and papers occur in different languages,
as follows: British, 142; French, 174; German, 133; American, 91;
Italian, 30; Latin, 15; Swedish, 14; miscellaneous, 33.
ETIOLOGY.—Under predisposing or constitutional causes are first to be
classed such special inheritances as tuberculosis or carcinoma and
tendencies to vascular degeneration. The occurrence of syphilis of
course predisposes its victims to cerebral or membranous growths, as
it does to other so-called tertiary forms of this disorder.
Hereditary predisposition only enters in so far as the individual
inherits a general tendency to the development of such affections as
cancer and tubercle. Hereditary tendency to a brain tumor per se does
not exist, but the individual who is of the tuberculous or cancerous
diathesis under special causes may develop an intracranial growth. As
to the comparative frequency with which diathetic tumors originate, a
reference to the tabular statement which will be given under {1029}
Pathology will be sufficient. Gliomata are very common, but with them
vie in frequency sarcomatous, tubercular, and gummatous growths. Any
table, unless it includes a very large number of cases—at least a
thousand or more—would be misleading as to the proportionate frequency
of these different forms of intracranial constitutional diseases; but
it is safe to say that syphilitic, tuberculous, and carcinomatous or
sarcomatous tumors are of comparatively frequent occurrence.
Tumors of the brain occur oftener among men than women. This fact is
dependent largely upon the difference between the habits and
occupations of the two sexes. Men, in the first place, are much more
addicted to alcoholic, venereal, and other abuses which give rise to
special degenerations or constitutional infection; and secondly, they
are more exposed to traumatisms. In 100 cases the tumors occurred
among males in 58 cases, among females in 40 cases, and sex was not
recorded in 2 cases.
Statistics show that intracranial growths are more likely to occur
between puberty and middle age. Although gliomata may be found at any
age, brain tumors in children are more likely to be of this character.
This is what might be expected from the nature of these growths.
Histologically, gliomata are most closely allied to the embryonal
stage of the connective tissue, and, according to Cohnheim, tumors
generally are the result of a surplusage of embryonal tissue—tissue
which has remained over after the requirements of normal development
have been met. Of 16 gliomata, 3 occurred in patients under ten years,
2 between the ages of ten and twenty, and 4 between twenty and thirty.
One hundred cases gave the following results as to age:
Under 10 years . . . . 10 cases.
From 10 to 20 years . . 12 "
" 20 to 30 " . . 18 "
" 30 to 40 " . . 24 "
" 40 to 50 " . . 12 "
" 50 to 60 " . . 13 "
" 60 to 70 " . . 3 "
Over 70 years . . . . . 1 "
Not recorded . . . . . 7 "
----------
100 cases.
It is now generally admitted that injuries play a most important part
as exciting causes of brain tumors. Frequently in our experience an
apparently direct relation has existed between a head injury and the
origin of the neoplasm. In 6 out of 12 cases reported by one of us,[2]
a history of traumatism was present, although in 5 of these a history
of syphilis was also present. The great frequency with which injuries
of all kinds occur must of course not be overlooked in this
connection. It is said by those who oppose the idea of the direct
causal relations of injury that almost every one could trace such
disease to falls or blows which few escape. In some of the cases of
brain tumor, however, the history of injury bears a direct relation in
time to the initial symptoms of the tumor. Certain tumors, as the
fibromata, osteomata, angiomata, would appear to be of more frequent
occurrence as the direct result of traumatism. The part played by
injuries in the production of carcinomata and sarcomata, whether in
the brain or elsewhere, has not {1030} infrequently been the subject
of dispute. We have no doubt that, a constitutional predisposition
existing, an injury frequently leads directly to the development of
some form of malignant growth. In not a few of the syphilitic cases
the history would appear to show that an injury to the skull had
localized the constitutional poison.
[Footnote 2: _Archives of Medicine_, vol. viii. No. 1, August, 1882.]
Echinococci and cysticerci are found within the cranium, and sometimes
give rise to tumors, but the statement of Obernier can hardly be
regarded as true, that they play an important part in the production
of cerebral tumors. Our table shows only two cases of this kind.
SYMPTOMATOLOGY.—The symptoms of intracranial tumors from the
standpoint of the course or progress of the affection can be divided
into an early or beginning, a middle or developed, and a late or
terminal stage.
Headache, vertigo, and vomiting are early symptoms, varying in
severity from slight to very serious manifestations, and slight and
changing mental phenomena are present. Eye symptoms, such as slight
diminution or blurring of vision, may or may not show themselves; the
ophthalmoscope may reveal the earliest appearances of choked disc or
neuro-retinitis.
In the second or middle stage, the period of the fully-developed
disease, we have an intensification and greatly increased constancy of
all the general symptoms, with some additional manifestations. Mental
disorder increases; headache becomes more violent, and sometimes more
localized; amblyopia tends to advance to complete blindness, with
marked swelling of the papilla, or special disorders of vision, such
as hemianopsia, occur; palsies, ataxia, convulsions, contractures,
rigidity of limbs, altered reflexes, local spasms, anæsthesia,
hyperæsthesia, paræsthesiæ, neuralgias, appear according to the extent
and location of the growth. Certain special phenomena, according
largely to the seat of the tumor, may also appear, such as disorders
of hearing, taste, or smell, polyuria, diabetes, albuminuria,
polyphagia, or dysphagia.
In the third or terminal stage paralysis, anæsthesia, convulsions,
etc. become more profound; mentality sometimes decreases to nearly
complete imbecility, although in many cases a fair amount of mental
power is preserved almost to dissolution. Involvement of the bowels
and bladder becomes a distressing symptom, involuntary evacuations
adding to the torments of the patient. Bed-sores, acquired or trophic,
appear; the patient's limbs are painfully contractured; great
emaciation is usually present. With agonized head, often blind or
deaf, with torturing pains in anæsthetic, paralyzed, and wasted limbs,
covered with painful sores, filthy involuntarily, imbecile or
demented, death comes as a thrice-happy relief.
Cases have been reported in which intracranial tumors of considerable
size were discovered on autopsy, and had evidently been present for
years without their presence having been discovered or even suspected;
but these are extremely rare. In general terms, however, it may be
said that every case of brain tumor must give rise to symptoms. These
symptoms may be few or many, slight or severe, brief or protracted,
constant or paroxysmal, according to circumstances. Our experience and
study of the literature of the subject lead us to the conclusion that
the pathological character of the growth would have some influence as
to the severity and prominence of symptoms. Gliomata—which are {1031}
sometimes hard to distinguish from the brain-substance, particularly
the soft gliomata, producing comparatively little pressure—when
located in certain so-called latent areas of the brain might escape
detection during life; so might also very small tumors of any kind
situated deeply in the substance of the brain.
The position taken by Obernier, that individuality has much to do with
the activity of symptoms of brain tumor, has some strength. He refers
particularly to the psychical response to the irritation caused by
brain tumors, which differs very much in different individuals. The
fact, which has been pointed out in the article on Hysteria, that
hysterical symptoms are often present in cases of brain tumor as well
as in other forms of organic disease, is also sometimes the cause of
mistake by the diagnostician. The cases of Hughes Bennett and Eskridge
will be referred to in this connection under Diagnosis.
When the subject of local diagnosis is considered farther on, those
regions which are most active and those which hold an intermediate
position in the production of particular and distinctive symptoms will
be more particularly discussed.
* * * * *
The possibility of the existence of multiple tumors should never be
overlooked. These are of comparatively frequent occurrence, and
sometimes give rise to a puzzling symptomatology. In our search
through the literature of the subject for cases we met with many
examples of multiple tumors. In preparing our table multiple tumors
were usually omitted. They give sometimes symptoms and signs so
conflicting as to make a local diagnosis impossible, and even to
confuse the expert as to general diagnosis. In other cases, however,
one growth of larger size or in an active region takes command of the
situation, and leads clearly to its diagnosis in spite of other less
significant tumors. This was shown, for example, in one of our cases
(Case 14). From the symptoms not only was the presence of the larger
tumor diagnosticated, but its exact location was indicated during
life. Post-mortem examination, however, showed a smaller tumor at the
inferior angle of the right lobe of the cerebellum, and also some
basal meningitis with effusion which had not been suspected. The first
tumor was one of the ascending frontal convolution, extending into the
fissure of Rolando. Its presence and location had been diagnosticated
by the right brachial monoplegia and paresis of the leg, which later
increased to paralysis of the limb, with right facial paresis, ptosis,
partial anæsthesia of right side of face, and slight clonic spasms of
the right arm. At the present time, when the question of operating for
the removal of brain tumors is so prominently before the profession,
and is now generally regarded as justifiable for growths in this one
location, the cortical motor area, the possibility of the coexistence
of another tumor should be carefully weighed. In this case, owing to
the clear diagnostic indications, the question of operation was
considered at the time of consultation, but fortunately—in large part
because the patient was almost in extremis—it was decided not to
operate. The operation would have been futile, the autopsy showing
that the probable immediate cause of death was the basilar effusion
which accompanied the cerebellar growth.
In another case reported by one of us[3] three gummata were found in
{1032} three different locations: one in the prefrontal region,
another in the retro-central fissure, and a third in the supramarginal
convolution. The general symptoms of brain tumor were present in this
case, but no localization was possible.