progressive course. In a few instances, after having attained a
certain development, it seems to have remained stationary for many
years. Nothing could better illustrate the slow progress or stationary
character of this affection than the study which Virchow made in 1880
of a patient whose case had been described by Romberg more than twenty
years before. After comparing the patient's present condition with
that described in notes taken twenty-one years before, Virchow was not
able to convince himself that any material alteration had occurred in
the atrophic parts; in fact, he regarded the disease as having long
ago become stationary. Cases reported by Tanturri, Baerwinkel, and
others bear out the same view as to the stationary character of the
affection in some cases.
COMPLICATIONS.—In a case of Virchow's, in addition to the facial
atrophy the patient presented atrophy which, commencing in the
distribution of some of the dorsal cutaneous nerves of the scapular
region, affected the musculo-spiral nerve from its origin downward,
most markedly in the forearm and head. Mendel[20] describes a case of
facial hemiatrophy occurring in a monomaniac, the diagnosis being
complicated by the somatic signs of degeneracy—congenital facial and
bodily asymmetry. The patient was twenty-eight years old, and the
disease could be traced back to the seventh year. In several reported
cases the disease has followed or has been associated with epileptic
seizures. Buzzard[21] has published a case in which the affection came
on in a girl aged thirteen in the course of a second attack of chorea.
In another it followed typhoid fever. In a case reported by Parry[22]
left hemiplegia with temporary disturbance of intelligence preceded
the disease by about two years. In another, observed by Axman and
Hueter, the patient, a man aged thirty-two, had suffered from
irregular contractures of the left masticator muscle, which commenced
in the seventh year. These spasms were associated with an increased
delicacy of sensation in the region of the left trigeminus. Neuralgia
is sometimes a complication. In a case reported by Holland,[23] the
patient for several years suffered from paroxysms of fronto-temporal
neuralgia.
[Footnote 20: _Neurologisches Centralblatt_, June 15, 1883, quoted in
_Journal of Nervous and Mental Disease_, 1883, x. p. 571.]
[Footnote 21: _Transactions of the Clinical Society of London_, vol.
v., 1872, p. 146.]
[Footnote 22: Eulenburg.]
[Footnote 23: _American Practitioner_, vol. xxxi. No. 182, Feb., 1885,
p. 72.]
PATHOLOGY.—Up to the present time, so far as I have been able to
learn, not a single autopsy in a case of typical progressive
unilateral facial atrophy has been made. Conclusions as to pathology
can therefore only be drawn from an analysis and study of symptoms and
from comparisons with other degenerative affections. Most authorities
hold that it is neurotic in origin. {700} Lande, however, regarded it
as having its origin in the connective tissue, affecting the nutrition
of the other tissues by interfering with the circulation.
Different views have been held by those who have believed in its
neurotic origin. Moore regarded it as a form of progressive muscular
atrophy attacking the muscles supplied by the portio dura, but what
has already been shown with reference to the symptomatology is
sufficient to show that this is an erroneous hypothesis. The muscles
supplied by the facial nerve may diminish in volume, but they do not
undergo a true degeneration.
Brunner attributed the affection to continued irritation of the
cervical sympathetic by some continuing lesion. Irritation of such a
lesion, according to him, causes vascular spasms, diminishes
blood-supply, and thus leads to the gradual development of the
atrophy. This view, to say the least, has not been made out.
Virchow holds that the affection is peripheral, and not central. He
believes, however, that a primary process being once set up in the
nerve-trunks, owing to inflammations of the surrounding tissues it may
then extend upward to the spinal and basal ganglia. The disease,
according to this view, has some analogy to herpes zoster. The manner
in which nerve-districts are unequally attacked is similar to what is
observed in the various forms of lepra and in morphœa. The doctrine of
a tropho-neurosis is the one that is best upheld by clinical facts. In
the first place, various arguments can be adduced to show that the
disease is neurotic in origin. It is limited to one side of the face
and to special nerve-distributions. Affections of sensibility in the
domain of the trigeminal nerve are frequently present. In the case
given under Symptomatology, although anæsthesia was absent, the
patient complained of peculiar paræsthesia in the form of stinging
sensation. Either trophic centres or trophic fibres, which have the
same connection and course as the trigeminal, are affected by a
degenerative process. Whether nuclei, ganglia, or peripheral fibres
are primarily affected has not yet been satisfactorily determined.
The case of Romberg, observed by Virchow twenty years after, was
considered by the latter to entirely overthrow the vaso-motor theory,
owing to the condition of the blood-vessels. While the surrounding
tissues from the skin inward were wasted and shrunken, the veins and
arteries of the parts appeared absolutely unchanged, the larger even
projecting above the surface of the skin. Under stimulation the vessel
dilated as in a normal individual, appearing to take no part in the
atrophy.
DIAGNOSIS.—The chief affections from which progressive unilateral
facial atrophy are to be diagnosticated are congenital asymmetry of
the face and head, facial paralysis, progressive muscular atrophy, and
hypertrophy of the opposite side of the face.
Congenital asymmetry of the face is observed in institutions for the
idiotic and feeble-minded. Such asymmetry, however, is usually
associated with corresponding defects of other parts of the body, as
of the head and limbs. In the nervous wards of the Philadelphia
Hospital is now, for instance, a patient the entire right half of
whose body is very decidedly wasted, the case being one of epileptic
idiocy with paralytic and choreic manifestations which point to
atrophy of the opposite half {701} of the cerebrum. At the
Pennsylvania Training-School for Feeble-minded Children want of
development of one-half of the head is often observed, but in these
and in other congenital cases the special changes in nutrition, in the
skin, in the color and growth of the hair, and in sensation, are
absent. In some cases of congenital asymmetry of the head atrophy of
the face or limbs, if present, will be upon the opposite side of the
body; and this is what might be expected, as arrested development of
the cranial vault will probably correspond with arrested development
of the cerebrum of the same side and bodily defects of the opposite
side.
Eulenburg, Maragliano, and others refer to an acquired want of
symmetry of the face sometimes observed in cases of wry neck or
scoliosis with deviation of the spinal column. Eulenburg especially
speaks of the so-called habitual scoliosis, where a curve in the
dorsal region in one direction has a compensatory cervical curve, the
side of the face which corresponds to the dorsal curve being often
smaller than the other. This asymmetry is supposed to result from
compression of the vessels and nerve-roots in the concavity of the
cervical curve. Here, again, the absence of special nutritional
changes will assist in the diagnosis. A study of the spinal curvature
will also be of value. Indeed, only the most careless observation
would allow this mistake in diagnosis to be made.
Facial paralysis—Bell's palsy of the usual type—would not be likely,
except by a careless observer, to be confounded with the disease under
consideration. When the muscles in the facial distribution are
involved, it is only in a very slowly progressive deterioration, which
may be due to mechanical causes, as compression by the contracting
skin. Moore,[24] as already stated, was wrong in speaking of the
morbid changes as entirely confined to the portio dura of the left
side. It is the fat, the connective tissue, and the skin which are
first and chiefly affected. In unilateral facial atrophy no true
paralysis of the facial muscles is present; consequently, the forehead
can be wrinkled, the eye closed, the mouth drawn up or down, or
sideways, etc.; in short, all the movements of the face are possible.
The opposite of this, it goes without saying, is present in general
facial paralysis. The drawing of the face to the side opposite the
paralysis in Bell's palsy is not here observed; the mouth is sometimes
drawn upward slightly on the atrophied side. In facial paralysis of
any severity reactions of degeneration are present. In unilateral
facial atrophy, as in progressive muscular atrophy, the muscles
respond both to faradism and galvanism. As minor aids in making the
diagnosis, the absence of loss of taste in one-half of the tip of the
tongue, and of deflection of the tongue and uvula, are in favor of
facial atrophy.
[Footnote 24: _Dublin Journal of Medical Science_, vol. xiv., August
and Nov., 1852, p. 245.]
It is possible that a case of progressive muscular atrophy might begin
in almost any muscle or group of muscles in the body. In progressive
muscular atrophy, however, the appearance of the skin is different; it
is not mottled; it is not thin, as can be demonstrated by manipulation
of it; it can be raised by the fingers; it is soft and flaccid. In
progressive muscular atrophy also first one muscle and then another,
just as likely as not at some distance from each other, are attacked
by the wasting process; in unilateral facial atrophy the affection
spreads uniformly and regularly {702} over one-half of the face.
According to Duchenne of Boulogne, when progressive muscular atrophy
first appears in the face the orbicularis oris is the muscle most
likely to be attacked, causing the lips to appear enlarged and
pendent, and giving a stupid look to the patient. In unilateral facial
atrophy this appearance is not present. Progressive muscular atrophy
commonly begins at a later age than unilateral facial atrophy.
Hypertrophy of the opposite side of the face is suggested by Eulenburg
as a possible source of mistake; but a little study, however, will
suffice to exclude this affection.
The following points are given by Eulenburg with reference to vitiligo
and porrigo decalvans: “In vitiligo we find the same white
decoloration of the skin, the cicatricial feel, the turning gray and
falling out of the hair, but not the loss of volume, which is the
special characteristic of this disease. In porrigo decalvans
inflammatory symptoms and œdema of the skin come first; the disease
first appears in regular circular spots; the hairs fall out without
previous loss of color; and finally, the disease is contagious, and
fungi can be demonstrated (microsporon Audouini).”
PROGNOSIS.—The prognosis as to recovery is bad, but, as already shown,
the disease may long remain stationary.
TREATMENT.—No well-authenticated case of cure under any method of
treatment has yet been reported. Electricity, either in the form of
galvanism or faradism, has been resorted to in a majority of cases. In
most of these temporary benefit has been produced by the employment of
these agencies. Baerensprung reports two cases as having been greatly
benefited by faradism. In Moore's case it also produced temporary
improvement. Carefully administered massage should be tried. With
reference to friction or massage, however, it is well, as advised by
Eulenburg, to be cautious, as excoriations are easily produced.
Measures directed to building up the nervous centres are
indicated—such medicinal agents, for instance, as the salts of silver,
gold, zinc, and copper, arsenical preparations, strychnia, and iron.
{703}
DISEASES OF THE MEMBRANES OF THE BRAIN AND SPINAL CORD.
BY FRANCIS MINOT, M.D.
The membranes or envelopes of the brain and cord were formerly
reckoned as three in number: the dura mater, lying next to the inner
surface of the bones of the cranium and spinal column, forming an
internal periosteum; the pia mater, which is in immediate contact with
the brain and cord; and the arachnoid, which was described as a
distinct membrane loosely attached to the pia mater, but more firmly
united to the dura, and constituting a closed sac or serous cavity
called the cavity of the arachnoid. This view is now abandoned by most
anatomists, who regard the external arachnoid as simply a pavement
epithelium covering the internal surface of the dura, and the visceral
arachnoid as constituting the external layer of the pia. The term
cavity of the arachnoid has, however, been generally retained for the
sake of convenience; and it gives rise to no obscurity if we remember
that by it we simply mean the space between the dura and the pia.
The diseases of the membranes which I shall consider are—congestion
and inflammation of the dura, including hæmatoma; congestion and
simple and tubercular inflammation of the pia; and meningeal apoplexy.
The membranes of the brain and those of the cord may be separately
diseased, or both may be affected at the same time. Simultaneous
inflammation of the cerebral and spinal meninges is apt to occur
epidemically, and is supposed to be zymotic in its origin. This is
described in another article. (See EPIDEMIC CEREBRO-SPINAL MENINGITIS,
Vol. I. p. 795.)
Inflammation of the Dura Mater of the Brain.
SYNONYM.—Pachymeningitis. Either surface of the dura may be inflamed
separately, and in many cases the symptoms are sufficiently definite
to render it possible to diagnosticate the situation of the disease in
this respect during life, especially when taken in connection with the
causes. For this reason it is usual to speak of external and internal
pachymeningitis. A peculiar form of the latter is accompanied by the
effusion of blood upon the arachnoidal surface, which sometimes forms
a tumor called hæmatoma. Congestion is also sometimes included in the
diseases of the dura mater, but is rarely alluded to by writers on
pathology or clinical medicine.
{704} Congestion of the Dura Mater.
Except as the first stage of inflammation, congestion of the dura can
hardly exist unless in connection with the same condition of the pia
or of the brain. Its causes are either local, such as thrombosis of
the sinuses, syphilitic or other growths in the membranes; or remote,
the principal being obstructions to the general circulation, including
valvular disease of the heart, emphysema and other affections of the
lungs, renal disease, compression of the superior cava or jugulars by
aneurism and other tumors, delayed or suppressed menstruation, etc.
The symptoms cannot be distinguished from those of congestion of the
pia or of the brain—namely, headache, a sensation of throbbing,
pressure, or weight in the head, somnolence, etc. The absence of fever
would serve to distinguish the affection from an inflammatory
condition of the membranes or brain.
Inflammation of the External Surface of the Dura.
SYNONYM.—External pachymeningitis.
ETIOLOGY.—The most frequent causes of external pachymeningitis are
injuries to the cranial bones from violence, caries and necrosis of
the same, and concussion from blows on the head. Next in order of
frequency, if not even more common, comes the propagation of
inflammation from disease of the inner ear and of the mastoid cells.
It is only of late years that this important subject has been brought
prominently forward and the danger of ear diseases in this respect
fully pointed out. The channels of communication, as indicated by Von
Tröltsch[1] in his valuable article on diseases of the ear, are along
the arteries and veins which pass from the skin of the meatus and the
mucous periosteal lining of the middle ear to the contiguous bone,
while the capillaries of the petrous bone are in direct connection
with the dura mater, so that the vessels of the latter are in
communication with the soft parts of the external and middle ear. The
vessels of the ear and the membranes of the brain are also directly
connected through the diploëtic veins of the temporal bone, which
discharge into the sinuses of the dura, as well as through the venæ
emissariæ, which, arising from the sinuses, pass through the bone and
discharge their contents into the external veins of the head. Von
Tröltsch also points out that the extension of an inflammatory process
may occur along the sheath of the facial nerve, the canal of which
(canalis Fallopii) is a branch of the internal auditory canal and is
lined by the dura mater. The latter is separated from the mucosa of
the tympanum only by a thin, transparent, and often defective plate of
bone. Inflammation may also be transmitted from the scalp by means of
the vessels which pass through the bones of the skull. In this way
erysipelas and other diseases of the integument sometimes give rise to
external pachymeningitis.
[Footnote 1: “Die Krankheiten des Gehörorganes,” von Anton von
Tröltsch, in _Gerhardt's Handb. der Kinderkrankheiten_, Tübingen,
1879, 5 B., ii. Abt., p. 150; also English translation of the same, by
J. O. Green, M.D., New York, 1882, p. 107.]
SYMPTOMS.—There are no known symptoms which are characteristic of
inflammation of the external surface of the dura. In cases of death
{705} from other diseases an autopsy may show traces of previous
inflammation, such as thickening of the membrane and its firm adhesion
to the cranial bones, which were not manifested during life by other
symptoms than those which accompany meningeal disease in general; and
in some instances none at all were known to have occurred. At a
variable time after the receipt of an injury to the head the patient
may complain of headache, followed by a chill, with high fever,
vomiting, vertigo, delirium, unconsciousness, convulsions, etc.,
arising from compression by the products of inflammation. The same
phenomena may follow the transmission of inflammation to the dura from
caries or other disease of the bones, or from otitis medea purulenta.
These symptoms usually continue without interruption, though there is
sometimes more or less complete remission of the pain, and the patient
may recover his consciousness for a time, thus giving rise to
fallacious hopes. In a large proportion of cases the disease extends
to the inner surface of the membrane and to the pia, without any
noticeable change in his condition other than coma. In chronic
external pachymeningitis the principal symptom is pain in the head,
which may persist for weeks or months without other manifestations
except drowsiness. Sometimes, on the contrary, there is obstinate
vigilance. Mental symptoms, such as loss of memory, hallucinations,
dementia, or mania, are sometimes noticed, ending, as in the acute
form, in coma.
PATHOLOGICAL ANATOMY.—In chronic cases often nothing is found but
thickening of the membrane, which generally becomes firmly united to
the inner surface of the cranium. Indeed, these appearances are not
unfrequently observed, to a limited extent, when there has been no
history of disease to account for them. In other chronic cases the
connective tissue of the membrane is found to be in part ossified, and
the osteophytes of the cranium sometimes found in pregnant women and
in patients with rheumatic cachexia are supposed to be due to a
chronic inflammatory process of the dura. The first changes observed
in acute external pachymeningitis are increased vascularization, shown
by red lines corresponding to the blood-vessels, with punctiform
extravasations, swelling, and softening of the tissue. Later, there is
thickening of the membrane from new formation of connective tissue,
and exudation of lymph, and sometimes of pus, which latter may
accumulate between the dura and the cranial bones, or in traumatic
cases may escape through openings in the skull. The dura and the pia
become united in most cases of acute inflammation. The lateral sinuses
frequently contain thrombi, which, when ante-mortem, are firm in
structure, reddish-brown in color, often closely adherent to the walls
of the vessel, and may extend to other veins, sometimes reaching as
far as the jugulars. When purulent inflammation of a sinus occurs, its
walls are thickened, softened, and discolored, and the inner surface
is roughened. The thrombus becomes more or less purulent or sanious,
disintegrates, and the infecting particles are carried into the
circulation, giving rise to embolism and disseminated abscesses of the
lungs, kidneys, liver, or spleen. Other lesions, such as injury or
caries of the cranial bones and purulent inflammation of the middle
ear and of the mastoid cells, are frequently found in conjunction with
pachymeningitis, to which they have given rise. When the disease of
the dura extends to the pia, the adjacent portion of the brain is
often found implicated in the {706} inflammation. In traumatic cases
the dura may be detached from the bone and lacerated to a greater or
less extent.
DIAGNOSIS.—The existence of external pachymeningitis may be suspected
from cerebral symptoms following traumatic injury of the skull,
erysipelas, or suppurative otitis medea, but apart from the etiology
it would not be possible to distinguish it from internal inflammation
of the membrane, or even from leptomeningitis.
PROGNOSIS.—In cases of suspected external pachymeningitis the
prognosis will depend upon the evidence of effusion of pus or blood
between the dura and the skull, and the possibility of their removal.
A large proportion of cases are fatal, especially those arising from
caries of the cranial bones and from the propagation of disease from
the middle ear, the mastoid process, or the external surface of the
skull, the inflammation extending through the membrane to the
arachnoid surface of the dura, and also to the pia mater. This would
be shown by a high temperature, rapid pulse, pain, and delirium,
followed by coma and perhaps convulsions. The frequency with which
external pachymeningitis occurs in connection with diseases of the ear
should put the physician on his guard in cases of otorrhœa or pain in
the ear, that he may warn the patient or his friends of the
possibility of danger, and may employ an appropriate treatment.
TREATMENT.—In all cases of injury to the skull or of severe concussion
the possibility of subsequent external meningitis should be borne in
mind. The patient should be confined to the bed and be carefully
secluded from excitement. Cold applications should be made to the
head, the diet should be simple and somewhat restricted, and the
bowels kept free without active purging. If there be evidence or
suspicion of the presence of pus or of blood between the dura and the
skull, means should be taken to evacuate the effusion. In case of
inflammatory symptoms leeches should be applied behind the ears, and a
stimulating liniment or croton oil rubbed on the scalp. There is no
specific treatment. Pain and sleeplessness must be relieved by opiates
and sedatives, and the strength must be sustained by nourishing diet
and stimulants.
Internal Pachymeningitis.