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Бічний аміотрофічний склероз, тип 4

ORPHA:357043· ICD-10 G12.2· Amyotrophic lateral sclerosis type 4

Визначення(English summary)

A rare, genetic motor neuron disease characterized by late childhood- or adolescent-onset of slowly progressive, severe, distal limb muscle weakness and wasting, in association with pyramidal signs, normal sensation, and absence of bulbar involvement, leading to degeneration of motor neurons in the brain and spinal cord.

Поширеність
<1 / 1 000 000
Успадкування
Autosomal dominant
Вік початку
Adolescent, Childhood