vitalwiki

Congenital muscular dystrophy with cerebellar involvement

ORPHA:370959· ICD-10 G71.2

Definition

A rare, congenital muscular dystrophy due to dystroglycanopathy characterized by proximal muscle weakness with a tendency for muscle hypertrophy and pseudohypertrophy, variable cognitive impairment, microcephaly, cerebellar hypoplasia with or without cysts, and other structural brain anomalies.

Inheritance
Autosomal recessive
Age of onset
Infancy, Neonatal