AI Article Synopsis

  • Miyoshi myopathy (MM) is a rare genetic disorder due to mutations in the dysferlin gene, linked to muscle degeneration and recognized as the same disease as Lower Girdle Muscular Dystrophy R2 (LGMD2B).
  • A 44-year-old male patient started experiencing symptoms at 19, including difficulties with stairs and toe-standing, leading to observable muscle atrophy and walking issues over the years.
  • The diagnosis was confirmed through genetic testing, revealing a mutation in the DYSF gene, and highlights the critical role of genetic diagnostics and the need for further research in muscular dystrophies.*

Article Abstract

Miyoshi myopathy (MM) is an autosomal recessive dysferlinopathy caused by a mutation in the dysferlin (DYSF) gene on chromosome 2p. Recent findings indicate that MM and Lower Girdle Muscular Dystrophy R2 (LGMD2B) are the same disease. We present the case of a 44-year-old male who first experienced symptoms of MM at the age of 19, initially noticing difficulty climbing stairs and standing on his toes. By the age of 29, he had developed significant calf muscle atrophy and weakness, which led to difficulties with walking. Electromyography and nerve conduction studies showed axonal damage and myogenic features. Genetic testing ruled out Charcot-Marie-Tooth disease but identified a pathogenic variant in the DYSF gene. Laboratory tests revealed elevated creatine kinase levels. Photographs of the patient's lower limbs showed significant calf muscle atrophy. Based on clinical, laboratory, and electrophysiological findings, he was diagnosed with MM. This case highlights the importance of genetic testing in diagnosing muscular dystrophies and underscores the need for continued research into gene and cell therapies. To the best of our knowledge, this is one of the first studies reporting a case of MM in Poland.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11457810PMC
http://dx.doi.org/10.7759/cureus.68869DOI Listing

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