Congenital myopathy with reduced type 2 muscle fibers

Orpha code: 544602OMIM code: 618414

Definition

A rare congenital myopathy characterized by neonatal onset of severe muscle weakness with selective atrophy/hypotrophy or absence of type II myofibers. Patients present at birth with hypotonia and respiratory failure, as well as mild facial and severe axial and proximal upper and lower limb weakness with areflexia and mild contractures. Eye movements and cardiac function are normal.

Disease data
Classification

Disease

Synonyms
Congenital myopathy with fast-twitch fiber atrophy
Congenital myopathy with reduced type II muscle fibers
Congenital myopathy with type 2 muscle fiber atrophy
Congenital myopathy with type II fiber atrophy
Congenital myopathy with fast-twitch fiber atrophy
Congenital myopathy with reduced type II muscle fibers
Congenital myopathy with type 2 muscle fiber atrophy
Congenital myopathy with type II fiber atrophy
ORPHA code
544602
OMIM code
618414
ICD10 code
G71.2
ICD11 code
-

No additional description.

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