Leiden dystrophy

Leiden dystrophy (synonym Leiden - Mobius type progressive muscular dystrophy) is one of the forms of progressive muscular dystrophy.
Different, on the one hand, from the spinal cord forms of muscular atrophy Haran-DuChene, on the other - from pseudohypertrophy (see muscular Atrophy).
Pathological changes observed in the muscles, which have the same character as in other forms of muscular dystrophy. The disease is a hereditary-family character. Observed and sporadic cases. The type of inheritance cannot be regarded as finally established. As with pseudohypertrophic forms, the disease begins with proximal lower limbs and muscles of the pelvic girdle, but, unlike the first, it is not accompanied by false or true muscle hypertrophy.
The disease in the majority of cases it develops in children (8-10 years), gradually, perfectly healthy before children. Atrophic process is distributed symmetrically. Striking first of all changed, "duck" gait dependent atrophy gluteus. These children easily fall hardly get up the stairs. Typical of them is lifting from the horizontal position, showing the weakness of the extensor back, hips and legs. Observed in these cases, lordosis applies also to the early symptoms of the disease. Shoulder girdle and upper extremities are affected later. This atrophy also begins with proximal. Elektrovozoremontnij muscles decreases. Fibrillar twitching not. Tendon reflexes reduced correspondingly lower forces wasting of the muscles. From the nervous system little change is observed. On the part of the psyche often have a different degree of innate inferiority, usually not progressing with the development of muscle in the process. The disease develops very slowly, gradually seizing more muscle groups. Marked long stop of process (up to 10 years and more).
Treatment - see Myopathy.