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limb-girdle muscular dystrophy type 2N; muscular dystrophy-dystroglycanopathy limb-girdle type C2 (LGMD2N)

Pathology: - muscular dystrophy - muscle biopsy shows dystrophic changes, inflammation & severely decreased alpha-dystroglycan Genetics: - autosomal recessive - associated with defects in POMT2 Clinical manifestations: - muscular dystrophy with onset after ambulation - mild muscle weakness - cognition is normal Laboratory: - increased serum creatine kinase

Database Correlations

OMIM 613158

References

OMIM :accession 613158