Hemiatrophia

HEMIATROPHIA (from Greek hemi- half and atrophy), a process of arrested development affecting one half of an organ, body part, or the entire body. In peripheral paralysis of one of the paired mixed nerves innervating a specific organ or body part, atrophy of tissues develops on the side of the paralyzed nerve, in addition to paralysis. Thus, with peripheral paralysis of one of the hypoglossal nerves, hemiatrophy of the tongue- N. linguae- develops, and when protruded, the tongue deviates to the side of the paralyzed nerve (see illustration in article Hypoglossus nerve); this phenomenon may be congenital or accompany various organic diseases, such as syringomyelia, tabes dorsalis, multiple sclerosis, and all cases of injury to one of the hypoglossal nerves (tumors, wounds); it is also observed in hemiatrophy of the face. Hemiatrophia depends on damage to the autonomic nervous system, peripheral or central; it can affect not only muscles but also other tissues, both soft and dense, resulting in reduction of organ size, thinning of tissues, and disturbance of autonomic functions. The pathological anatomy of hemiatrophia caused by damage to the central autonomic nervous system is little studied, but there can be little doubt about the dependence of hemiatrophia precisely on the autonomic nervous system. Hemiatrophia cruciata et hemihypertrophia cruciata. Crossed hemiatrophy or hemihypertrophy are extremely rare. The number of cases published to date does not reach ten. The peculiarity of this form is that the atrophic or hypertrophic process is localized on the face on one side, and on the trunk and limbs (sometimes only on the upper extremities) on the opposite side. These crossed forms apparently depend on diseases of the autonomic nervous system, and there is more evidence to consider them, like non-crossed forms, as diseases of central origin due to damage to trophic pathways. Crossed hemiatrophia was first published in 1896 by Luntz. In 1903, Volhard described a case of hemiatrophia of the face with pigmentary abnormalities on the skin of the opposite half of the body. Crossed hemihypertrophy was first published by A. M. Kozhevnikov in 1922. The etiology and pathogenesis of these diseases are completely unknown. Only pathological-anatomical study of the corresponding cases will shed light on these unique and extremely interesting pathological forms. Hemiatrophia faciei progressive, Romberg's disease, is characterized by gradual atrophy of one half of the face. A rare disease, first described by Parry in 1837. In 1846, Romberg included it among trophoneuroses, and since then the disease bears his name. Hemiatrophia fac. progr. most often develops in young subjects aged 10-20 years, and sometimes in early childhood; after 30 years it is rare. In some cases, the development of the disease was preceded by facial or skull injuries, in others-infectious diseases (diphtheria, typhus, erysipelas, etc.). Recently, Mankovsky published a case that developed after epidemic encephalitis. Often, the onset of the disease is preceded by trigeminal neuralgia. The left half of the face is affected significantly more often than the right (according to Klingman, 75%); this confirms Stir's theory that in right-handed persons atrophic processes develop on the left, and hypertrophic on the right, while in left-handed persons these relationships are reversed. The disease usually does not begin with the entire half of the face, but with a separate area, with the orbit, lower jaw, or cheek. The essence of the disease comes down to gradually progressive atrophy of all tissues. First, the skin atrophies; sometimes it loses its pigment, in other cases it takes on an abnormal color. Sometimes the facial and scalp hairs lose their pigment, sometimes loss of eyebrows, eyelashes, mustaches is observed. Next, atrophy of subcutaneous tissue and bones develops; sometimes the facial muscles, masticatory muscles, and tongue muscles undergo atrophy; the muscle atrophy is not degenerative, and motor function suffers relatively little. The secretion of sebaceous glands is reduced, while sweating is sometimes increased. Sensitivity in most cases is objectively unchanged, but sometimes neuralgic pains occur. Occasionally the pathological process involves the eyeball, larynx, and pharynx. The face becomes asymmetrical: the affected side is significantly smaller than the healthy side, bones are reduced, soft tissues are thinned, the eyeball is sunken, the cheek skin is wrinkled, furrowed. Often the atrophy is not limited to the face, but extends to the neck, upper extremity, or even the entire corresponding half of the body (hemiatrophia totalis). Sometimes the atrophy spreads to the opposite side. Progressing gradually over several years, the process subsequently stops. Combination with other nervous diseases (neuralgia, epilepsy, migraine, mental disorders, etc.) is not uncommon. Combination of hemiatrophia with scleroderma is particularly frequent (Oppenheim). Sometimes hemiatrophia is a symptom of another nervous disease-cerebral palsy, syringomyelia, tabes, syphilis, brain tumor and echinococcus. The cause of the disease lies in the disruption of the functions of the autonomic nervous system, of peripheral or central origin. According to Muller, the cause lies in chronic irritation of trophic fibers in the cervical sympathetic nerve, in its centers, or in the fibers going to the periphery. The prognosis of the disease, in terms of recovery, is unfavorable; it does not pose a danger to life. Therapy gives little comfort. Electotherapy is useless in most cases. In one case, Oppenheim's section of the cervical sympathetic nerve gave improvement, in another improvement followed after removal in the region of ganglion suprem of a hardened gland. There are as yet no convincing data on the effect of Lericsh's operation. Gershuni proposed subcutaneous injections of paraffin for cosmetic purposes.

A. Kozhevnikov.

Cerebral hemiatrophia of Bielschowsky type is characterized by early development of epileptic seizures and gradually occurring spastic phenomena, to which hypoplasia of skeletal muscles is added. Athetoid movements may also occur. Apparently, the disease is congenital in nature. Patho-anatomically, selective necrosis of the third layer of the cortex and nucl. caudati is found.