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Leiden Dystrophy

Leiden dystrophy (synonymous with Leiden - Möbius type of progressive muscular dystrophy) - a form of progressive muscular dystrophy.

It differs, on the one hand, from the spinal form of Aran's muscular atrophy — Dyushena, on the other hand, from pseudohypertrophy (see Muscular Atrophy).

Pathological changes observed in the muscles themselves, which have the same character as in other forms of muscular dystrophy. The disease is hereditary and familial in nature. There are also sporadic cases. The type of inheritance cannot be considered finally established. As with pseudohypertrophic forms, the disease begins with the proximal parts of the lower limbs and the muscles of the pelvic girdle, but, unlike the first, it is not accompanied by false or true muscle hypertrophy.

The disease in most cases develops in childhood (8-10 years), gradually, in perfectly healthy children. The atrophic process spreads symmetrically. A striking, first-ever, duck-like gait is striking, depending on the atrophy of the gluteal muscles. Such children easily fall, hardly climb the stairs. Typical for them is lifting from a horizontal position, indicating weakness of the extensors of the back, hips, and shins. The lordosis observed in these cases also applies to the early symptoms of the disease. The shoulder girdle and upper limbs are affected later. In this case, atrophy also begins with the proximal sections. Electrostimulation of the muscles decreases. There are no fibrillary twitches. Tendon jerks decrease as the strength of the atrophied muscles decreases. On the part of the nervous system, no special changes are noted. On the part of the psyche, various degrees of inborn inferiority are often noted, usually not progressing with the development of the muscular process. The disease develops very slowly, gradually capturing all new muscle groups. Long process stops (up to 10 years and more) are noted.

Treatment - see myopathy .