Charles K. (Charles Karsner) Mills.

The nervous system and its diseases. A practical treatise on neurology for the use of physicians and students online

. (page 97 of 121)
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lead, malaria, and syphilis. Anemic trigeminal neuralgias occur
especially in overworked and badly nourished young women : they
may affect any branch of the fifth nerve, but are especially liable to
be of the ophthalmic or temporal variety. Eheumatism, gout, dia-
betes, all may cause a true neuritis, but in some instances they give
rise to extreme neuralgic pain without the other symptoms of neu-
ritis, such as hyperesthesia and vasomotor and trophic changes. Lead
is a somewhat infrequent cause of trigeminal neuralgias, but oftener
attacks the nerves of other portions of the body. Occasionally, how-
ever, a striking instance of trigeminal neuralgia due to lead poison-
ing is observed, and that malaria may be a cause is one of the oldest
clinical observations. Malarial neuralgia shows a particular tendency
to attack the supraorbital branch of the ophthalmic nerve, causing
what is sometimes called brow ague. The great distinguishing
feature of trigeminal as of other forms of malarial neuralgia is its
periodicity. The attacks tend to return and to augment to a certain
intensity at regular intervals, as of one, two, three, or more days,
following the same rules in this respect as govern the return of other
forms of malarial paroxysms. In sjieaking of syphilis as a cause of
true neuralgia, it must be understood that reference is not made to
any of the numerous cases in which this virus causes definite specific
lesions like meningitis or gummata, but to those cases in which the
action exerted by the poison of the disease in the blood is to disturb



SYMPTOMATIC FORMS OF TRIGEMINAL NEURALGIA. 867

and pervert the normal functions of the nerve centres. Such casef>,
while perhaps not rare, are difficult of exact diagnosis, and with a
history of syphilis we are likely to fix our attention upon the proba-
bility of a localized lesion rather than upon the possible effects of a
toxic agent in the circulation. Trousseau, and others following him,
have described a form of epileptiform trigeminal neuralgia which is
regarded by some as distinct from prosopalgia, or tic douloureux,
and by others as a variety of this affection. We incline to the latter
opinion. In this disease the patient may have daily one or many
attacks of lightninglike pain, which succeed one another with great
rapidity for a few seconds or minutes and then disappear. The
attacks of pain may recur for days or weeks and then may dis-
appear for months, or even for a longer period, but, as a rule, the
disappearance is not permanent.

Diagnosis, Prognosis, and Treatment of Symptomatic Tri-
geminal Neuralgias. — The etiology and pathology of the sympto-
matic trigeminal neuralgias have been sufficiently indicated in the
description of these affections, which in our consideration of them
have been largely classified from the etiological and pathogenetic
standpoint. Their diagnosis is largely dependent upon a close study
of their history and causation. In the first place, the different classes
of neuralgia of this type must be carefully separated from one an-
other : for instance, those due to focal lesions from the neuritic and
toxic varieties. Safe therapeutic tests will sometimes be of great
service in clinching the diagnosis. It may be found that the cases
yield primarily to an antirheumatic, antilithic, or antisyphilitic
treatment. The urine and blood should be examined, in doubtful
cases, to determine the presence or absence of diabetes and special
blood states. Diabetic neuralgias are said to show a tendency to
bilateral symmetry, to attack the same portion of the nerve dis-
tribution on both sides. Neuralgic affections must be carefully
differentiated from the pains which indicate intracranial lesions,
and especially from tumors in their initial stages. The prognosis
of symptomatic trigeminal neuralgias is comparatively good, but
depends upon the particular variety. Many of the constitutional
forms are amenable to treatment. Those due to focal lesions may or
may not be, according to the extent and the character of the lesion.
Neuritis is especially intractable when it attacks the intracranial
branches of the fifth nerve, but even in these cases it may some-
times be reached by treatment. The epileptiform neuralgia of Trous-
seau is practically incurable. The most that can be said of it is that
the patient may, by good fortune, remain free from the attacks for
a longer or shorter period. In the treatment of symptomatic neu-
ralgias the matter of first importance is to seek and attack the cause.
Anemic neuralgias are best treated by fresh air, an abundance of
good food, and the use of iron, arsenic, and manganese. Mercury and



868 NERVOUS DISEASES.

the iodides are the therapeutic anchors in syphilis, the salicylates in
the rheumatic form, and lithium and colchicum in the gouty form.
In diabetic neuralgias the remedies of most value are the salicylates
and the preparations of codeine. One of the chief indications must
always be the immediate relief of the pain. As the measures to
accomplish this object are practically the same in the symptomatic
neuralgias as for tic douloureux and migraine, the treatment as given
under these affections can be consulted.

PROSOPALGIA, OR TIO DOULOUREUX.

Definition and Synonyms. — Prosopalgia is a form of trigem-
inal neuralgia of extreme severity, paroxysmal in type, and usually
having associated with the pain attacks of spasm involving the
muscles of the face. We have used the term prosopalgia as best
because, meaning simply "pain in the face," it does not involve any
theory as to the local or other cause of the disorder. It should, how-
ever, be regarded as a severe type of trigeminal neuralgia. Its most
common synonyms are tic douloureux, trifacial neuralgia, facial neural-
gia (not a good name, because misleading as to the nerve affected),
and FothergilV s face ache or neuralgia. This form of trigeminal neu-
ralgia is a special disease, and should be clearly separated from those
forms of trigeminal neuralgia which depend upon inflammatory or
other disease of special portions of the nerve. These have been con-
sidered in the preceding section of this chapter.

Clinical History. — In typical cases the disease presents a some-
what uniform method of onset, development, and climax. The pa-
tient, frequently in or past middle life, is without warning attacked
with sharp pains in some special portion of the face, as in the upper
or the lower lip, near the angle of the jaw, under the eye, or on the
forehead. These pains, ordinarily intense and lancinating, may soon
pass away, but they generally recur, and as the case progresses usu-
ally become more severe and diffuse. Often they remain for a long
time in the portion of the distribution of the nerve first attacked ;
later, pain appears in other branches of the same subdivision, or it
may appear in an area of another of the divisions. Beginning in one
of the subdivisions of the third branch, eventually the third, second,
and first may all be more or less implicated in the painful attacks.
The second and first and the third and second are more likely to be
conjointly involved than the third and first. This tendency to dif-
fusion from the subdivisions of one of the three great divisions of the
nerve to subdivisions of one or of both of the other branches throws
light upon the probable nature of the disease, to a certain extent
favoring the view of its ganglionic origin. As the disease progresses,
various exciting causes will readily bring on the attack of pain.
The paroxysm may be precipitated, for instance, by drinking, by
touching the face, by moving the jaws, by the slamming of a door,



TIC DOULOUREUX.



869



Fig. 410.




by the noise of the falling of an object, or by anything tending to
cause actual movement or to produce emotional disturbances. The
patient lives in a state of constant anxiety, fearful of everything.
The patient's face may become hyperemic or even suffused and
swollen, or the conjunctiva alone may be injected. Excessive lach-
rymation may be present. In some cases unilateral sweating, and in
rare instances trophic changes, such as attacks of herpes and dis-
coloration of the skin and possibly thickening of the skin, may be
observed. Hearing, taste, and smell are rarely disturbed, but vision
is oftener affected. The usual disturbances of vision are transitoi-y
paresis of the ocular muscles or trophic changes in the retina. S^jasm
in the facial muscles accomjjanies the paioxysms of pain, and this
spasm is often very violent, and has given the affection one of its
names, tic douloureux. In tlie illustration (Pig. 410) this sj^asm is
well shown, the face
being forcibly drawn
to the side affected.
The ocular muscles
are sometimes spas-
modically involved,
as are also the mus-
cles supplied by the
motor subdivision of
the fifth nerve. Occa-
sionally the patients
become hallucina-
tory or even mani-
acal. Fere reports a
case in an epileptic
complicated by zona
in which menacing
voices were heard on
the affected side, but
the patient was con-
scious that the voices
were unreal. Intense
sialorrhea came on
with the neurotic at
tack, and the voices
disappeared with
them. It is probable

that in this case the epilepsy predisposed to the hallucinations. As
a rule, tic douloureux is more severe and recurs with gieater fre-
quency in winter and sirring.

Etiology. — Prosopalgia is more frequent among women than
among men. It occurs usually in or after middle life, and is a not



I .

Case of tic douloureux : appearance of the patient's face during
one of the severe paroxysms.



870 NEEVOUS DISEASES.

infrequent disease of the aged. It is very rare in childhood and
youth. The tendency to its occurrence is marked in neuropathic
families, and especially in families in which epilepsy is present.
Toxic and infectious agents probably act as exciting causes ; certainly
anything which tends to lower the tone and vitality of the system
will lead to tic douloureux in those who have a constitutional ten-
dency to the affection. It will be remembered that Anstie held that
neuralgia was the prayer of the starved nerve for blood. In this
consideration of the etiology of tic douloureux I have purposely
omitted the discussion of the numerous focal lesions which are usu-
ally enumerated as causative factors in its production, believing
that the disease is essentially an irritative degenerative affection of
the Gasserian ganglion, nearly all its so-called causes being simply
exciting factors in those in whom a persisting pathological state pre-
disposes to the disease.

Pathogenesis. — The history of the development of the disease,
the paroxysmal nature of many of its symptoms, its associated phe-
nomena, and the unsatisfactory results of treatment lead to the con-
clusion that the true seat of prosopalgia, or tic douloureux, is in the
Gasserian ganglion. Although pathological proof is still wanting,
the disease is in all probability a form of degeneration of the cells of
this ganglion, similar to that which occurs in the dorsal spinal ganglia
in tabes, and to the degeneration of the ganglion cells of the anterior
horns in chronic atrophic affections, such as amyotrophic lateral scle-
rosis and progressive muscular atrophy. It is probable that in sen-
sory cases the disease gradually invades all portions of the ganglion,
and as one cell after another drops out the instability of the ganglion
is increased, and with this the tendency to violent sensory discharges.
This theory seems to me more probable than that which attributes
the affection to a thalamic or cortical origin. As the central sensory
neurons have their cells of origin in the Gasserian ganglion, and
possibly in the thalamus, the discharging lesions which give rise
to the violent paroxysms of pain have their true seat and source
in one or the other of these cell masses, but most probably in the
former.

Pathological Anatomy. — In some cases forms of low grade
neuritis have been discoA'eied in branches of the nerve affected. The
vessels which supjoly the nerve with blood are sometimes affected
with chronic inflammatory processes, but in a large number of cases
branches of the nerve which have been removed by operation have
been examined without discovering any noteworthy alterations in
the ner\'e. In three cases in which Dana examined four superior
maxillary nerves, striking e\'idences of arterial disease were found,
and in a fourth case no bloodvessel was present in the specimen.
This led him to the view that an obliterating arteritis was a factor
in the disease.



TIC DOULOUEEUX. 871

Diagnosis.— The diagnosis of prosopalgia is usually readily made.
The only point of importance is to distinguish bet-s^-een that form
which has been considered in the present section, which is due to
a chronic degenerative process of the Gasserian ganglion, and the
forms which are due to focal irritative lesions, such as neuritis,
exostosis, cicatrices, tumors, meningitis, etc., located somewhere in
the course of the trigeminal sensory apparatus.

Prognosis.— The prognosis of prosopalgia is almost invariably
bad. Periods of relief from suffering are obtained either by treat-
ment or without reference to treatment. Unfortunately in the vast
majority of cases the disease gradually grows worse, both as to the
frequency with which paroxysms occur and as to their severity.
On the whole, those patients do best who early submit to operation,
probably because even the temporary relief which they obtain— a
relief which often extends over months, or it may be years — allows
time for recuperation.

Treatment. — A large number of medicinal remedies have been
used for the relief of prosopalgia. Those which appear to afford
most relief can be classified under the two heads of constructives
and anodyne sedatives. Ifearly all sufferers from prosopalgia re-
ceive some benefit from rest and the use of tonic and building-up
remedies like cod liver oil, preparations of iron, arsenic, manganese,
and the metallic tonics generally. Strychnine sulphate in increasing
doses has proved of great benefit when the treatment has been con-
tinued over a number of weeks or months, and nitroglycerin is of
particular value in the aged or prematurely senile, acting through
its well known effect upon the vessels. Preparations of aconite, and
especially the alkaloid aconitine, are also useful both in relieving the
pain and in changing the character of the disease, and among other
remedies for the relief of the pain are morphine, codeine, plienacetin,
antipyrin, antifebrin, and ammonol. Morphine or codeine, if given
in sufficient doses, will usually give relief, but opium preparations
should not be given too freely, as the patient may acquire a drug
habit. Hot whiskey and quinine will sometimes temper the violence
of an attack. All constitutional conditions, like anemia, rheumatism,
gout, diabetes, and syphilis, which predispose to the neuralgic attacks
should receive attention, and should be treated with the drugs which
are known to be beneficial in these affections. In special instances
the salicylic preparations will prove of great value, and the best
of these are the sodium salicylates and oil of gaultheria. Prom ten
to fifteen grains of the former, or from ten to twenty drops of the
latter, should be given three or four times daily. When, as is too
frequently the case, tic douloureux does not yield to other treatment
and continues to render the life of the patient almost unendurable,
resort should be had to surgical treatment. Even when a permanent
cure is not obtained, the few months or few years of relief afforded



872 NERVOUS DISEASES.

enable the patient's general health to be built up, and give him the
opportunity of enjoying life during the period of respite. The most
important surgical measures are stretching, resection, or avulsion of
the extracranial portions of the nerve, section of the nerve trunks
in front of the Gasserian ganglion, and operation on this ganglion.
For the various methods of operation surgical works should be
consulted. The choice of operation must depend to some extent on
the severity but especially on the diffusion of the pain. When this
is confined to a single accessible portion of the nerve, neurectomy
should be tried, or neurectomy and avulsion. When the neuralgia
affects two or more of the great divisions of the nerve, the Gasserian
or pre-Gasserian operation deserves full consideration, although even
in some of these cases separate operations on different nerve trunks
seem to afford the most relief. In the light of what has been said of
the pathology of prosopalgia, it may be thought that no operation
except that on the ganglion has a rational basis ; but this does not
seem to me to be the correct view to take. While the disease is
probably a degenerative and irritative process, attacking the cell
bodies of this ganglion, it must be remembered that the nerve trunks
themselves are composed of the peripherally distributed axis cylinder
processes of these cells, and that their section may reflexly inhibit
the discharging process which causes the pain or may prevent the
transmission of the painful impulses. In many cases experience has
taught that neurectomy and avulsion afford relief for a considerable
period, but, on the whole, the extirpation of the Gasserian ganglion
has afforded the most permanent relief.

MIG-RAINE.

Definition, Synonyms, and Varieties Migraine is a period-
ical paroxysmal affection, usually hereditary, and chiefly character-
ized by severe attacks of pain confined to one side or to one portion
of one side of the head, and commonly associated with either nausea
or vomiting or both. The synonyms for migraine are megrim, sick
headache, neuralgic sick headache, paroxysmal headache, and hemicrania.
Migraine in the first place presents itself in what might be called its
most typical form, in which all the phenomena are of great severity,
the pain extending over a laige part of one side of the face and
head, and in some cases radiating to all parts of the head, although
the focus of greatest severity is usually in the tem^^oral or the supra-
orbital region of one side. Special varieties are determined chiefly
by the limitations of the painful areas ; but the only one of 'these
that will be separately considered is the migrainoid supraorbital
neuralgia.

Symptomatology. — MiscelJaneous Symptoms. Not infrequently
the patient has decided prodromes, usually in the form of general
malaise, discomfort, or depression, which may last for hours or days.



MIGRAINE. 873

Photophobia, visual spectra, tinnitus, vertigo, and gastric disorder
may be other prodromal signs. The attacks frequently show a great
tendency to periodicity, a fact to which special attention has been
called by Liveing and Sinkler. The latter has reported a number
of instances indicating the tendency of the disease to recur on fixed
days. Salius relates the case of an Italian monk who for three
years and seven months had an attack of violent hemicrania every
Monday, the attacks lasting from twenty-eight to thirty hours (Tis-
sot). Usually beginning in one side of the head, the pain augments
in violence, sometimes slowly, sometimes rapidly, until finally the
patient is compelled to take a recumbent position. It is often of a
throbbing character, the patient complaining that the head feels as
if it would burst. It is increased by noise, light, jarring movements,
and anything which causes emotional excitement. The appearance
of the patient differs in different cases. Frequently the face is
pale ; both the head and the limbs feel cold to the patient. In other
instances the opposite appearance may be present, the face being
flushed or even turgid, and in still other cases alternations of pallor
and of flushing or turgescence may occur. In rare cases one side
of the head and face may be pallid and the other flushed. These
dififerences in vascularity and in temperature are among the clini-
cal reasons which have led to the belief that the disease should be
subdivided into an angiospastic and an angioparalytic variety. The
pupils may be either contracted .or dilated, myosis usually accom-
panying pallor of the face, and mydriasis flushing. Forms of oculo-
motor paresis are comparatively common, and have already been
referred to when discussing affections of the third nerve, page 844.
Some would have us regard the cases of migraine with these oculo-
motor symptoms as a special type, which has been designated ophthal-
moplegic migraine, but no reason for such classification exists greater
than might exist for describing an auditory form or other forms
based upon the frequent presence of certain special symptoms. The
mental condition of the patient varies somewhat with temperament.
Frequently great apprehension is felt, and nearly always a feeling
of depression. The ideas may become confused, and the patient
may have hallucinations of sight or hearing. K"ausea and vomiting
are frequent accompaniments of the headache, and have given it one
of its commonest names, sick headache. Sickness of stomach comes
on early in some, in others not until the paroxysm has persisted for
a number of hours. An attack of copious vomiting, either sponta-
neous or brought on by the use of emetics, often marks the climax
of the attack, the patient afterwards sinking into a sleep, and the
pain gradually disappearing. The pulse is usually small and tense,
and is more likely to be lessened than to be increased in frequency.
The duration of an attack is from eight to twenty-four hours, al-
though it may last two or more days. While the affection, as has



874 NEEVOU9 DISEASES.

been stated, exhibits a considerable tendency to periodicity, in many
cases the paroxysms recur somewhat irregularly, varying from one
week to two or three months. In children the symptoms are some-
what less pronounced.

Visual Spectra (Ophthalmic Migraine). Reference has already been
made in several places in preceding sections to the spectra of migraine
and the forms of fugacious hemianopsia with phosphenes and other
visual phenomena sometimes present preceding or during an attack
of migraine (pages 757 and 773). It has been stated that the central
field is nearly always free from visual spectra, and also that various
disturbances of the mechanism of cerebral speech occur. The dis-
turbances of vision are of such dominant importance in some in-
stances that it has been suggested that ophthalmic migraine should be
regarded as a special form of this disease (Charcot). This so-called
ophthalmic migraine may present itself in a simple form in which
only headache and visual phenomena are present : Gowers refers to
one curious case in which visual disturbances exactly such as pre-
cede attacks of migraine occurred frequently during many years as
an isolated symptom, but at no time was there any headache or pain.
In other cases various symptoms of cerebral disturbance are com-
bined with the headache, and visual spectra. The patient, for in-
stance, may suffer from transient and incomplete aphasia, and this
may or may not be associated with hemiparesis or monoparesis or
with paresthesia like numbness and tingling confined to one side of
the body or of the face or to the limbs of one side. In rare instances
transient aphasia is the only evidence of an attack which is funda-
mentally the same as that of migraine, a fact which should be re-
membered, as the absence of headache and of the other phenomena
of the disorder may lead to the belief that the patient is suffering
from a real but limited apoplexy. Charcot has directed attention
to the occurrence of ophthalmic migraine as a prodrome of general
paralysis of the insane. In other rare instances migraine does not
set in until after the disease has been initiated as indicated by other
physical or mental symptoms. In most cases the migraine cannot be
regarded as having any direct connection with the general paralysis.
It is a concomitant and forerunner only in the sense that among the
lesions productive of the disorder happen to be some which are so
situated and of such character as to give rise to ophthalmic phe-
nomena. Much space could be filled with a description of special
types of visual phenomena recorded by different authorities as oc-
curring in migraine. Gowers in particular has elaborately discussed
this subject. Among such recorded visual spectra are the zigzag
" fortification lines ;" luminous objects, or objects encircled by lumi-
nous rings or fringes of the most brilliant colors ; visions of moving



Online LibraryCharles K. (Charles Karsner) MillsThe nervous system and its diseases. A practical treatise on neurology for the use of physicians and students → online text (page 97 of 121)