Sporadic late-onset nemaline myopathy (SLONM) is a rare subacute adult-onset myopathy pathologically characterized by the presence of nemaline rods in muscle biopsy [1-3]. While the pathogenesis of SLONM remains unclear, exclusive muscle involvement may suggest an autoimmunity-related association [2]. Approximately half of SLONM patients have concomitant monoclonal gammopathy of undetermined significant (MGUS) although the causal relationship between MGUS and SLONM has not been elucidated [1]. In comparison to other non-heritable adult-onset nemaline myopathies, including SLONM without MGUS and HIV-associated nemaline myopathy (HIV-NM), SLONM associated with MGUS (SLONM-MGUS) is clinically distinctive [1,2].

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