Severe hypotonia-psychomotor developmental delay-strabismus-cardiac septal defect syndrome

Orpha code: 467176OMIM code: 616816

Definition

Severe hypotonia-psychomotor developmental delay-strabismus-cardiac septal defect syndrome is a rare, genetic, non-dystrophic congenital myopathy disorder characterized by a neonatal-onset of severe generalized hypotonia associated with mild psychomotor delay, congenital strabismus with abducens nerve palsy, and atrial and/or ventricular septal defects. Cryptorchidism is commonly reported in male patients and muscle biopsy typically reveals increased variability in muscle fiber size.

Disease data
Classification

Disease

ORPHA code
467176
OMIM code
616816
ICD10 code
G71.2
ICD11 code
-

No additional description.

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