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Muscle-eye-brain disease with bilateral multicystic leucodystrophy

ORPHA:370997· ICD-10 G71.0

Definition

A rare congenital muscular alpha-dystroglycanopathy with brain and eye anomalies disease characterized by a severe muscle-eye-brain disease-like phenotype associated with intellectual disability, muscular dystrophy, macrocephaly and extended bilateral multicystic white matter disease.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Infancy