Key result
A 60-year-old male with proximal muscle weakness was diagnosed with sporadic late-onset nemaline myopathy based on the presence of numerous nemaline rods in myofibers.
Why the study?
Sporadic late-onset nemaline rod myopathy is a rare adult-onset myopathy that can lead to treatment failure and unfavorable outcomes without a correct diagnosis.
Case Report (n=1)
Highlights a rare case of sporadic late-onset nemaline rod myopathy presenting with proximal muscle weakness in an adult, emphasizing the need for correct diagnosis to avoid treatment failure.
Answers are grounded in this paper and cite their sources.
May aid recognition of rare adult-onset myopathy; leaves open need for larger cohorts on management.
Sporadic late-onset nemaline rod myopathy is a rare, acquired, sub-acute, adult-onset myopathy characterized by proximal muscle weakness and nemaline rods in the myofibers. In contrast to its congenital form, the prevalence in adult population is comparatively rare. Herein, we report a case of 60-year-old male who presented with insidious onset proximal muscle weakness with myopathic pattern on electromyography. Histopathological examination showed type 1 and 2 muscle fiber atrophy with minimal chronic inflammatory cell infiltrate. Modified Gomori trichrome staining revealed granular deposits in the myofibers. Ultrastructure examination showed numerous nemaline rods in the myofibers; hence, the diagnosis of sporadic late-onset nemaline myopathy was rendered. Its association with monoclonal protein has been reported in the past. One should be aware of this rare entity as without correct diagnosis, there can be treatment failure and unfavorable outcome.
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Narwal et al. (2024) conducted a case report in Sporadic late-onset nemaline rod myopathy (n=1). A 60-year-old male with proximal muscle weakness was diagnosed with sporadic late-onset nemaline myopathy based on the presence of numerous nemaline rods in myofibers.
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