散发性成人杆状体肌病的研究进展OA

Sporadic late-onset nemaline myopathy(SLONM)is a rare,acquired,and treatable myopathy with a sub-acute or chronic progressive clinical course,characterized by asymmetric muscle atrophy and weakness in the axial and limb muscles,with or without involvement of respiratory and cardiac muscles.The only definitive diagnostic method at present is the identification of rods accumulation in muscle fibers by muscle biopsy pathology.This article provides a re-view of the clinical manifestations,diagnostic evaluations,muscle pathology,and advances in the treatment of SLONM.