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Distal hereditary motor neuropathy type 7

ORPHA:139589· ICD-10 G12.2

Definition

A rare, slowly progressive genetic peripheral neuropathy characterized by distal atrophy and weakness affecting the upper limbs (with a predilection for the thenar eminence) and subsequently the lower limbs, associated with uni- or bilateral vocal cord paresis leading to hoarse voice and breathing difficulties, and facial weakness.

Inheritance
Autosomal dominant
Age of onset
Adolescent, Adult, Childhood