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A case of sporadic late‐onset nemaline myopathy without monoclonal gammopathy of unknown significance/human immunodeficiency virus successfully treated with intravenous gamma globulin
Author(s) -
Fukazawa Ryosuke,
Cho Masanori,
Hidaka Yukihiro,
Takezawa Hidesato,
Ogasawara Masashi,
Nishino Ichizo,
Fujii Akihiro
Publication year - 2021
Publication title -
clinical and experimental neuroimmunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.297
H-Index - 15
ISSN - 1759-1961
DOI - 10.1111/cen3.12620
Subject(s) - nemaline myopathy , medicine , monoclonal gammopathy of undetermined significance , bence jones protein , pathology , gamma globulin , immunology , antibody , myopathy , monoclonal , monoclonal antibody , immunoglobulin light chain
Background Sporadic 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 presentation A 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). Conclusions IVIg may be effective in SLONM without monoclonal gammopathy of unknown significance or HIV infection by improving symptoms and suppressing the progression.