and then at its elimination from the body, constitute a rational
treatment. Sulphur baths and the internal administration of sulphur
may be employed with a view of converting the lead eliminated by the
skin and mucous membranes into an insoluble sulphide, and thus
preventing its resorption. Repeated laxative doses of castor oil will
remove unabsorbed lead from the intestinal canal. The plan of
treatment at present in general favor consists in the combined use of
potassium iodide and magnesium sulphate. From five to twenty grains of
the iodide are to be given in not less than six or eight fluidounces
of water three times daily, the stomach being empty: two hours after
each dose one or two drachms of the magnesium sulphate are to be
taken; after this dose an ordinary meal.
This treatment is designed to dissolve the lead deposited in the
tissues, and cause its elimination by the mucus of the alimentary
canal in part, and to a slight extent also by the urine. The magnesium
sulphate tends to remove such lead as finds its way into the
alimentary canal thence with all possible rapidity. These measures,
together with the removal of the patient from further exposure, exert
of themselves a favorable influence upon the malnutrition and anæmia.
Quinine, strychnine, iron, cod-liver oil may be advantageously
administered as the toxic effects of the lead pass away.
The colic demands the administration of opium or its derivatives to
relieve the pain, which is commonly excruciating. For this purpose the
hypodermic injection of morphia is our most efficient remedy. Alum in
doses of twenty to thirty grains every four or six hours is useful in
lead colic.
For the relief of the local paralytic affections local as well as
general treatment is necessary. Massage is of great use, especially
when combined with passive movements. Galvanism, one large electrode
being applied to the cervical vertebræ, the other to the extensor
surface of the affected limbs, is followed by excellent results.
Labile currents of fifteen or twenty cells should be thus applied, the
poles being changed several times at each sitting. As the nutrition of
the muscles improves faradic currents may be occasionally substituted.
Persevering treatment is necessary to obtain the best results.
For the present relief of the arthralgia gentle frictions with or
without anodyne liniments must be employed. The hypodermic use of
morphine {692} may become necessary in cases in which the pain is
urgent. It is, however, here as elsewhere, to be if possible avoided.
The tendency to recurrent joint-pains rapidly disappears as the poison
is eliminated from the organism.
For the relief of the severe cerebral symptoms which are described
under the term Encephalopathy special treatment is of little avail.
All observers agree in recommending an expectant plan. The measures of
treatment directed against the general condition, as above described,
must be steadily continued. The influence of chronic lead-poisoning
upon pregnancy is very deleterious. Constantine Paul[14] and others
have shown that the early death of the fœtus very constantly occurs.
The prompt removal of women who have become pregnant, from all
exposure to lead, and energetic medicinal treatment, are needed to
obviate the danger of abortion.
[Footnote 14: _Arch. gén. de Méd._, vol. xv., 1860.]
{693}
PROGRESSIVE UNILATERAL FACIAL ATROPHY.
BY CHARLES K. MILLS, A.M., M.D.
DEFINITION.—Progressive unilateral facial atrophy is a disease
characterized by progressive wasting of the skin, connective tissue,
fat, bone, and more rarely muscles of one side of the face.
SYNONYMS.—Progressive facial hemiatrophy, Neurotic atrophy of the face
(Virchow), Facial trophoneurosis (Romberg), Prosopodismorphia
(Bergson), Atrophy of the connective tissue of the face or Laminar
aplasia (Lande).
HISTORY AND LITERATURE.—This disease has been known to the medical
profession since 1825, when, according to Eulenburg, it was first
described by Parry. The _Index Catalogue_ contains references to
thirty books and articles on the subject in various languages.
Eulenburg gives a bibliography which contains thirteen references not
given in the _Index Catalogue_. One personal case, which will be
detailed later, has fallen under observation. In all, I have collected
from fifty to sixty cases. The number of reported cases is slowly
increasing; nevertheless, the disease must still be regarded as rare.
Carswell,[1] according to Lasègue,[2] first arranged scientifically
the various lesions which might arise in consequence of a retardation
and arrest, or even inverted action, in nutrition, and at the same
time that he assigned their causes pointed out the several forms of
atrophy. Aran[3] has written a contribution on the subject, furnishing
valuable material for classifying atrophies. Romberg called general
attention to the disease in his clinical researches, published in
1846. He described the disease under the name of a new form of atrophy
of the face, and took the position that it was a primary
trophoneurosis. Of contributions by American writers, a paper by
Bannister[4] is the most complete and valuable. This not only contains
two fully and carefully reported cases, but also a thorough discussion
of special symptoms and the pathology of the disease. The chapter of
Eulenburg[5] contains an excellent sketch of the disease, and is
especially valuable for the discussion of the various theories as to
its nature.
[Footnote 1: _Illustrations of the Elementary Forms of Disease_,
1836.]
[Footnote 2: _Archives générales de Médecine_, May, 1852, p. 71.]
[Footnote 3: _Ibid._, Sept. and Oct., 1850.]
[Footnote 4: _Journal of Nervous and Mental Disease_, vol. iii., No.
4, Oct., 1876, p. 539.]
[Footnote 5: _Ziemssen's Cyclopædia of the Practice of Medicine_, vol.
xiv., 1877.]
ETIOLOGY.—The disease is of much more frequent occurrence among
females than males. Out of 25 cases which I have been able to
classify, {694} 9 were in men and 16 in women. It is an affection of
comparatively early life. The following were the ages of 25 patients:
under ten years, 7; from ten to twenty years, 11; from twenty to
thirty years, 6; over thirty years, 1.
Graefe[6] reports a case due to syphilis, in which there was also
paralysis of the left oculo-motor externus and trigeminus. In one of
Bannister's cases the patient had suffered repeatedly from slight
frost-bites. Among the causes which seem to be thoroughly established
traumatism holds the first place. The affection could be traced to
injury in a fair number of the published cases. A case is reported by
Maragliano[7] of a child who had a fall in which she struck the left
half of her face, and in consequence of which a circumscribed swelling
appeared near the external angle of the left eye and remained for a
few days. Shortly after this the left half of the face began to grow
smaller than the right. Schuchardt[8] speaks of a fall on the head
which left a cicatrix on the right parietal bone below the coronary
suture, the injury being followed by facial atrophy. Panas[9] reports
a case in which the wasting of the face followed a fracture of the
lower jaw. In one of two cases reported by Bannister,[10] a man, aged
forty-two, is stated to have been thrown from a stage-coach, falling
on his head, and not losing consciousness, but receiving a scalp wound
over the coronal suture. Subsequent to this time he had cerebral
symptoms, physical and mental, and unilateral facial atrophy
developed.
[Footnote 6: Quoted by Rosenthal.]
[Footnote 7: _Note di Clinica Medica_, Genoa, 1881, translated by
Joseph Workman, M.D.—_Alienist and Neurologist_, vol. ii. No. 2,
April, 1881, p. 146.]
[Footnote 8: Quoted by Eulenburg.]
[Footnote 9: _Bull. Soc. de Chir. de Par._, 1869, 1870, 2d S. ex.,
198, and _Gaz. des Hôpitaux_, 1869.]
[Footnote 10: _Journ. Nerv. and Ment. Dis._, Chicago, 1876, iii. pp.
539-560.]
SYMPTOMATOLOGY.—Before discussing the symptomatology I will give brief
notes of the following case, which has not been before reported, and
which was observed by me while in charge of the Nervous Dispensary of
the Hospital of the University of Pennsylvania several years since.
C——, aged seventeen, white, a mill-operative, had a good family
history. Three years before coming under observation a white spot
appeared in the skin over the right malar prominence, and since that
time this side of the face had gradually atrophied. When this patient
was first examined the skin had a mottled appearance. Close
examination showed that the skin, connective tissue, and bone were
decidedly wasted, and there seemed also to be some loss of substance
in the muscles of the right side of the face. The mouth, however,
could be closed properly, the lips showing no puckering or drawing to
one side. The right half arches of the palate were atrophied. The
uvula was drawn slightly but positively to the left. Careful
examination of the membrane of the drum of the right ear showed it to
be atrophied. An ophthalmoscopic examination of both eyes was made,
but no changes were discovered. No changes were noted in the hair of
the patient on the affected side. He was able to perform all movements
with the facial muscles, but the creases and contours of the face in
repose and in motion were less marked on the right side than on the
left. The skin of the right side of the face seemed to be bound
closely to the bone. The muscles on the same side responded well to
{695} the faradic and galvanic current. The patient had at times a
peculiar sensation of stinging in the atrophied half of the face. He
was treated with electricity, local massage, and tonics, and did not
improve, but did not get worse while under observation, a period of
several months.
At the Hospital of the University of Pennsylvania I also observed one
well-marked case of unilateral atrophy of the tongue without atrophy
of the face, of which, unfortunately, I have not preserved the notes.
The patient was a middle-aged man with a syphilitic history. The
atrophy in the reported cases has occurred much more frequently upon
the left than upon the right side of the face.
Changes in the skin are among the most constant and striking
phenomena. By some dermatologists the disease is regarded as a form of
morphœa, the keloid of Addison. Morphœa as described by Duhring[11]
occurs in the form of patches, rounded, ovoid, or irregular in
outline, small or large, tough or leathery, smooth or shiny, and may
also manifest itself in the form of atrophic pit-like depressions in
the skin. The skin alterations differ somewhat in character;
frequently one of the first signs of the disease is the appearance of
a white spot on the cheek. This was the first noticeable phenomenon
observed in the above case. After a time this spot or patch may change
in color, becoming of a yellowish or brownish hue. A number of spots
appearing and undergoing change, the skin after a time assumes a
mottled appearance. In some of the reported cases a yellowish, or
yellowish-gray, or brownish appearance is recorded as having been
present. In these the observations were probably made after the
affection had existed for some time, and in them the white spots or
patches were probably at first present. In one of Romberg's cases,
first recorded when the patient was nine and a half years of age, he
noted the deposit of a yellowish-gray pigment which began at the
median line and extended to the angle of the jaw across the lower half
of the face and neck, divided here and there by portions of healthy
skin. Discoloration, varying in intensity, also existed in irregular
patches on the upper part of the face and on the forehead. The
discolored tissues were glossy and greasy-looking. The skin adhering
to the bone often gives the patient an aged appearance. Pruritus has
been several times observed.
[Footnote 11: _Medical News_, vol. xlv. No. 4, July 26, 1884, p. 85.]
Changes take place in the hair. Sometimes it simply changes color; at
other times it falls out or ceases to grow. The beard or hair of the
head will thus be gray or white upon one side, or in one or two
locations of one side and not of the other; or the hair may be absent
or simply thinned out. Peculiar limitations in the extent to which the
hair is affected are sometimes observed. In one of Romberg's cases,
for example, complete absence of the eyelashes from the inner angle of
the eye to the middle of the lids was noted; the hair was very thin,
and in some parts altogether wanting, and the left eyebrow was almost
entirely devoid of hair. The changes which take place in the color of
the hair in some severe cases of chronic trigeminal neuralgia will be
recalled in this connection. I have known of several instances in
which a few locks of hair were turned gray or white on the neuralgic
side. Anstie, author of the work on Neuralgia, was a sufferer from
supraorbital neuralgia, and the eyebrow of the same side contained a
white tuft. Facts of this kind {696} serve to corroborate and
emphasize the view that unilateral facial atrophy is a
tropho-neurosis.
Anidrosis, or absence of perspiration, on the atrophied side of the
face is a nearly constant phenomenon. In one of Bannister's cases, a
printer, attention was first called to the patient by the fact that in
working a hand printing-press in the hot weather he perspired only on
the right side of his face. The left side remained perfectly dry, no
matter how warm the temperature or hard the labor. This hemidrosis, or
unilateral sweating, was confined only to the right side of the
forehead, the right cheek, side of the nose, and lip. On the chin it
encroached a little on the left. Under the chin and in the throat
there was a little excess of perspiration on the right side; on other
portions of the body no difference was noted. Nicati[12] considers a
combination of symptoms, several of which were present in a recorded
case—namely, lowered temperature, stoppage of perspiration, and
wasting of the side of the face—as indicating a paralysis of the
sympathetic nerve in what he calls its second or more advanced stage.
In true facial hemiatrophy, however, other phenomena of such paralysis
are wanting. Brunner[13] gives a case in which perspiration, tears,
mucous secretions, and temperature were all diminished on the affected
side, and in which were also present exophthalmos and dilatation of
the pupil. The cervical sympathetic in this case was irritated by a
tumor. I agree with Bannister, however, that while we cannot exclude
the possibility of the participation of the sympathetic in hemiatrophy
of the face, the evidence is not sufficiently positive. Mechanical
interference with the sweat-glands by pressure or otherwise has been
suggested. The true view to take would seem to be that the
perspiratory disorder is due to the same central or peripheral
neurotic affection which gives rise primarily to the atrophy.
Eulenburg says that the secretion of the sebaceous follicles is in
most cases diminished or stopped on the atrophied side, while the
sweat-glands often act in a normal manner; but his experience does not
seem to correspond with that of the majority of other observers.
[Footnote 12: _Sur Paralysie du Nerf sympathetique-cervical_,
Lausanne, 1873, quoted by Bannister.]
[Footnote 13: _St. Petersburger Med. Zeitschr._, ii., 1871, quoted by
Bannister.]
Seguin, Dreschfeld, and others have found no notable differences in
surface temperature between the two sides of the face. In one of
Bannister's cases the temperature was tested in both ears, and a
difference of six-tenths of a degree Fahrenheit in favor of the ear of
the unatrophied side was found, that of the left ear being 97.6°, and
that of the right 98.2°. This examination was repeated on another
occasion with nearly the same result.
Weakness of pulsation in the carotid of the affected side has been
observed. Sometimes the power to blush is lost on the affected side,
but this may return. In one instance the cheek, having been reddened
by electrical stimulation, so remained for an hour or more.
Paræsthesiæ, such as pain, numbness, and stinging sensations, are not
uncommon. A diminution of the general tactile sensibility of the skin
of the affected side has been observed by Tanturri[14] and by
Vulpian,[15] and perhaps by others, but anæsthesia is not commonly
present.
[Footnote 14: _Il Morgagni_, 1872, quoted by Bannister.]
[Footnote 15: _L'Appareil vaso-moteur_, ii. p. 430.]
Eulenburg and Guttmann found atrophy of the muscles supplied by the
motor branch of the fifth nerve—namely, the masseter and temporal.
{697} The changes which take place in the muscles of the affected side
are, so far as can be judged, not of the character of fatty
degeneration. Probably a general and uniform shrinkage of calibre of
muscular fibres and bundles occurs. Certainly, this would seem to be
true of the muscles supplied by the portio dura. The muscles on the
atrophied side respond to both the faradic and galvanic currents,
reactions of degeneration not being present. The absence of the
reactions of degeneration in the facial muscles shows that normal
muscular fibres remain. In one reported case the facial muscles seemed
at first to react more readily than in normal conditions. The muscles
of the upper lip sometimes appear to have wasted, preventing full
closure of the mouth on this side. Fibrillary twitchings have been
observed only very rarely. Voluntary control over the muscles of the
affected side is not impaired.
In one of Bannister's cases the first upper molar of the left side
sometimes ached, and the gum and bone were so wasted as to expose its
roots for a considerable distance. Falling out of the teeth has been
observed in a few other cases.
Atrophy of the tongue is associated with the facial wasting in a
certain percentage of cases, and the uvula and soft palate are also
sometimes wasted. On the other hand, atrophy of the tongue has been
observed as an isolated phenomena.
Atrophy of the bones of the face is frequently present, and has been
determined by careful measurements. It will show to a greater or
lesser extent according to the age of the patient. When the disease
arises after the bony development has been fully acquired, there will
be little external evidence of osseous involvement; when it begins
during the time of active growth of the bone, as it does not
infrequently, the arrest in the skeleton will be very apparent. Thus
in a number of cases beginning in early childhood bone atrophy has
been a very marked feature.
Eulenburg says that the functions of taste, smell, hearing, and seeing
are not interfered with in any of the reported cases; but Bannister
reports impairment of the sense of taste in both of his cases. In one
the whole left half of the tongue was involved, in the other only the
left posterior third. In the first of these, in which the whole left
half of the tongue was involved, both the glosso-pharyngeal and the
chorda tympani nerves were affected. According to Bannister, the
significance of this observation is that either the glosso-pharyngeal
and fifth nerves are both involved, or that the taste-fibres for the
base as well as tip of the tongue are derived from the last-named
nerve. In this case, however, which was due to a severe injury, the
headache, mental confusion, loss of hearing, etc. more probably
indicated a widespread intracranial lesion, affecting to some degree
the nucleus of the glosso-pharyngeal. In the other case, which was
apparently a typical one of unilateral facial atrophy in its early
stage, no history of traumatism was present, and the sense of taste,
which was carefully tested several times, was seriously impaired over
the left posterior third of the tongue.
Bannister discusses the probable cause of impairment of taste in each
case, as follows: “The atrophy seemed to involve the region supplied
by the second division of the fifth nerve as much as, or more than,
the other divisions; and on this is situated the spheno-palatine
ganglion which gives off the Vidian nerve, the upper division of
which, the superior {698} superficial petrosal nerve, is supposed by
Schiff to contain the taste-fibres, which return from the lingual
nerve through the chorda tympani. The usual theory of this disease is
that it depends upon an affection of the trophic fibres contained in
the fifth nerve or one or more of its divisions. Accepting this
theory, Baerwinkel,[16] noticing a case of unilateral atrophy of the
face affecting only the second division of the fifth, and, as he held,
indicating trouble with the spheno-palatine ganglion, its trophic
centre, mentions the fact that there was no impairment of taste in the
anterior portions of the tongue as rather against the hypothesis of
Schiff as to the course of the taste-fibres. At the same time he gives
two other observations of lesions of the trifacial and the seventh
nerve that favor it. In our patient I should have expected a priori to
have found the taste affected in the anterior portion of the tongue,
if anywhere, but the reverse was the fact. It is difficult to suppose
an accidental lesion of the glosso-pharyngeal coexisting with the one
of the trigeminus that produces the atrophy when there are no more
signs of nervous disorder than there were in this case. On the other
hand, the taste-fibres of the glosso-pharyngeal are not generally
supposed to have such connections with the fifth nerve as to be
involved in its disease, while those of the chorda tympani, of the
connections of which with the trigeminus there is much more
physiological evidence, escape. The observation is a contradictory
one, and I cannot at present explain it.”
[Footnote 16: _Deutsch. Archiv f. klin. Med._, xvii. 1, 1875.]
So far as I know, ophthalmoscopic changes have never been noted. In a
few cases enophthalmos, or sinking in of the eye, doubtless due to the
disappearance of orbital fat as the disease advances, has been
recorded.
Defects of hearing, and even partial deafness, have been reported in a
very limited number of cases, making it questionable whether this
symptom is a coincidence or a complication. In the case reported by me
careful examination of the membrane of the tympanum by W. Ziegler of
the ear department of the Hospital of the University of Pennsylvania
showed atrophy of this membrane.
Eulenburg speaks of one case in which the external atrophy had reached
as far as the larynx, and in which the pronunciation of the letter _r_
was somewhat impeded. Beverly Robinson,[17] reports a case accompanied
with slight paralysis of the adductors of the vocal cords. The
laryngeal paralysis in this case improved under treatment, which led
Robinson to believe that the relation between the two affections might
have been only one of coincidence.
[Footnote 17: _Am. Journ. Med. Sci._, October, 1878, p. 437.]
Flascher[18] has described a unique case of bilateral facial atrophy
occurring in a woman twenty-three years old. When an infant she fell
and injured her forehead, and soon afterward had an attack of measles
without eruption. Shortly afterward the atrophy appeared. Tactile
sensibility was diminished and the secretion of sweat absent. The
masseter, temporal, and facial muscles and bones were atrophied.
External strabismus of the left eye was present; the pupil of this eye
was dilated, irregular, and non-reactile; sight was diminished, the
optic disc atrophied. This case would appear to differ somewhat from
true facial hemiatrophy. It was probably a peripheral nerve affection
following measles.
[Footnote 18: _Berliner klin. Wochenschr._, 1880, No. 31.]
{699} Mitchell[19] has reported a case of absence of adipose matter in
the upper half of the body. I had an opportunity of examining this
patient, who was exhibited at a meeting of the Philadelphia
Neurological Society, and noticed that both sides of the face
presented a striking similarity to the affected side in cases of
unilateral atrophy. The muscles were apparently not affected. Mitchell
suggests the possibility of separate centres capable of restraining
deposits of adipose tissue.
[Footnote 19: _Am. Journ. Med. Sci._, July, 1885.]