Abstract

AbstractBackgroundSporadic late‐onset nemaline myopathy (SLONM) is an acquired form of nemaline myopathy developed in adulthood. Due to its rarity, standard treatment has not been established albeit some reports suggest treatment efficacy.Case presentationA 75‐year‐old woman developed progressive muscle weakness in her extremities 3 years previously. Muscle biopsy revealed nemaline bodies, which led to the diagnosis of SLONM. M protein, human immunodeficiency virus (HIV) antigen/antibody, and urine Bence‐Jones protein were all negative. Muscle strength gradually improved after receiving two courses of intravenous immunoglobulin (IVIg).ConclusionsIVIg may be effective in SLONM without monoclonal gammopathy of unknown significance or HIV infection by improving symptoms and suppressing the progression.

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