POMT1-related limb-girdle muscular dystrophy R11

Orpha code: 86812OMIM code: 609308

Definition

A form of limb-girdle muscular dystrophy characterized by the onset of slowly progressive proximal muscle weakness during childhood (with fatigue and difficulty running and climbing stairs) and developmental delay. Mild intellectual deficit and microcephaly, without any obvious structural brain abnormality, are found in all patients. Mild pseudohypertrophy and joint contractures of the ankles have also been reported.

Disease data
Classification

Disease

Synonyms
Autosomal recessive limb-girdle muscular dystrophy type 2K
Dystrofia obręczowo-kończynowa- niepełnosprawność intelektualna
LGMD2K
LGMD type 2K
LGMD2K
Limb-girdle muscular dystrophy type 2K
Limb-girdle muscular dystrophy-intellectual disability syndrome
POMT1-related LGMD R11
ORPHA code
86812
OMIM code
609308
ICD10 code
G71.0
ICD11 code
8C70.41

No additional description.

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