Myopathies with finger flexor weakness: Not only inclusion-body myositis

Muscle Nerve. 2020 Oct;62(4):445-454. doi: 10.1002/mus.26914. Epub 2020 Jun 1.

Abstract

Muscle disorders are characterized by differential involvement of various muscle groups. Among these, weakness predominantly affecting finger flexors is an uncommon pattern, most frequently found in sporadic inclusion-body myositis. This finding is particularly significant when the full range of histopathological findings of inclusion-body myositis is not found on muscle biopsy. Prominent finger flexor weakness, however, is also observed in other myopathies. It occurs commonly in myotonic dystrophy types 1 and 2. In addition, individual reports and small case series have documented finger flexor weakness in sarcoid and amyloid myopathy, and in inherited myopathies caused by ACTA1, CRYAB, DMD, DYSF, FLNC, GAA, GNE, HNRNPDL, LAMA2, MYH7, and VCP mutations. Therefore, the finding of finger flexor weakness requires consideration of clinical, myopathological, genetic, electrodiagnostic, and sometimes muscle imaging findings to establish a diagnosis.

Keywords: distal myopathy; finger flexor weakness; inclusion-body myositis; myopathy with rimmed vacuoles; myotonic dystrophy.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Amyloidosis / pathology
  • Amyloidosis / physiopathology
  • Distal Myopathies / pathology
  • Distal Myopathies / physiopathology
  • Fingers / physiopathology*
  • Glycogen Storage Disease Type II / pathology
  • Glycogen Storage Disease Type II / physiopathology
  • Humans
  • Muscle Weakness / physiopathology*
  • Muscle, Skeletal / pathology
  • Muscle, Skeletal / physiopathology*
  • Muscular Diseases / pathology
  • Muscular Diseases / physiopathology*
  • Muscular Dystrophies / pathology
  • Muscular Dystrophies / physiopathology
  • Muscular Dystrophies, Limb-Girdle / pathology
  • Muscular Dystrophies, Limb-Girdle / physiopathology
  • Muscular Dystrophy, Duchenne / pathology
  • Muscular Dystrophy, Duchenne / physiopathology
  • Myositis, Inclusion Body / pathology
  • Myositis, Inclusion Body / physiopathology
  • Myotonic Dystrophy / pathology
  • Myotonic Dystrophy / physiopathology
  • Sarcoidosis / pathology
  • Sarcoidosis / physiopathology

Supplementary concepts

  • Dysferlinopathy
  • Filaminopathy, autosomal dominant