LEIDEN DYSTROPHY

From Big Medical Encyclopedia

LEIDEN DYSTROPHY ( E. V. Leyden , it is mute. therapist, 1832 — 1910; synonym: children's form of the progressing muscular dystrophy, muscular dystrophy of Leyden — Möbius, Dyushenn's syndrome — Leyden, a tazovo-humeral form of muscular dystrophy) — the special form of the progressing muscular dystrophy with preferential damage of muscles of pelvic and humeral belts developing at children's age.

The detailed description of the progressing muscular dystrophy is made by G. Dyushenn in 1861. In 1876 E. Leyden observed a family form of muscular dystrophy with preferential damage of muscles of pelvic and humeral belts and the beginning of a disease in children's age. In 1879 P. J. Mobius showed that the described G. Dyushennom and E. Leyden of a form treat one disease. Further detailed studying of a disease is carried out by V. Erb and V. K. Roth. V. Erb subdivided a tazovo-humeral form of the progressing muscular dystrophy into the options beginning at children's, youthful or mature age. Most of authors consider L. as children's option of a tazovo-humeral form of muscular dystrophy of Erba — the Company (see. Myopathy ).

The disease is inherited on autosomal recessively type, begins at the age of 3 — 5 years with the progressing muscular weakness and atrophies covering in the beginning muscles of a pelvic girdle, then humeral. Less often defeat extends from muscles of a shoulder girdle to muscles of pelvic.

Morfol, changes of the affected muscles are specific to primary miodistrofichesky process — thinning, vacuolation, hyaline dystrophy of muscle fibers, increase in number of kernels in a sarcolemma and an arrangement their chain, substitution of atrophied muscle fibers fatty and connecting tissue.

Clinic

Klin, manifestations correspond to symptomatology of a tazovo-humeral form of the progressing muscular dystrophy of Erba — the Company: gradual increase of muscular weakness, an atrophy of muscles of proximal departments of extremities, a moderate pseudo-hypertrophy of gastrocnemius muscles, emergence of characteristic receptions of «miopat» at a rising, other typical symptoms (alate shovels, «free shoulder girdles», «wasp waist», etc.).

Treatment

Treatment complex, includes substances, corrective metabolism and blood supply of muscles, neuromuscular conductivity (ATP, vitamins, anabolic hormones, drugs of potassium, Bupatolum, Halidorum, Nicospanum, prozerin, Galantaminum, etc.), and also to lay down. gymnastics and massage, oxygenobarotherapy, electrostimulation of muscles.

The forecast

Progressing of process, as a rule, slow, full loss of ability to movement comes in 20 — 30 years after the beginning of a disease. Dependence of speed of progressing on age is established, the disease develops in Krom: the earlier process begins, the quicker there are heavy motive frustration.



Bibliography: Leyden E. V. Klinik der Rtickenmarks-Krankheiten, Bd 2, S. 525, B., 1875.


L. O. Badalyan.

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