ArticleEuropean journal of neurology2026
Concurrent Sporadic Late-Onset Nemaline Myopathy and an Excessive Glycogen Accumulation Associated With Monoclonal Gammopathy.
Article in European journal of neurology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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2 citing papers in PubMed.
- Review
- The Role of Muscle Biopsy in the Era of Modern Genomic Medicine-A Review.Journal of clinical medicine · 2026Review
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5 authors.
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Abstract
backgroundMonoclonal gammopathy-associated myopathies (MGAMs) include light chain (AL) amyloid myopathy, sporadic late-onset nemaline myopathy (SLONM), and vacuolar myopathy with monoclonal gammopathy and stiffness (VAMMGAS). These subtypes usually occur separately, although rare overlap has been described. We report a patient with monoclonal gammopathy and concurrent SLONM and excessive glycogen accumulation, resembling VAMMGAS but with distinct features.
methodsCase report with clinical, electrophysiological, pathological, and therapeutic characterization of a patient with IgG-kappa monoclonal gammopathy and coexisting SLONM and glycogen accumulation within muscle fibers.
resultsA 69-year-old man developed subacute progressive axial and limb weakness, head drop, dysphagia, and weight loss. Examination showed proximal/distal weakness, neck extensor weakness, and lumbar hyperlordosis. EMG revealed myopathic motor unit potentials without electrical myotonia or complex repetitive discharges. Serum studies identified IgG-kappa monoclonal protein with elevated kappa light chain and ratio. Muscle biopsy demonstrated nemaline rods (1% of fibers) and scattered fibers with non-rimmed vacuoles (0.3% of fibers) containing PAS-positive, diastase-labile material consistent with concurrent SLONM and glycogen storage myopathy-like pathology. Genetic testing for congenital nemaline and glycogen storage myopathies was negative. Plasma-cell directed therapy with daratumumab, lenalidomide, and dexamethasone led to rapid functional improvement and M-protein reduction. Further gains followed autologous stem cell transplantation.
conclusionThis case expands the MGAM spectrum, highlighting co-occurrence of SLONM and an excessive glycogen accumulation responsive to immunotherapy and transplantation. These findings suggest a spectrum of related disease processes. Recognition of this combined pathology is clinically important, as affected patients may benefit from targeted immunotherapy or stem cell transplantation.
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