A system of practical medicine. By American authors. Vol. 5

by Unknown

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. 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 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.