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Camptocormia due to inclusion body myositis

Brent P Goodman1, Teerin Liewluck, Brian A Crum

  • 1Departments of Neurology, Mayo Clinic, Scottsdale, AZ, USA. goodman.brent@mayo.edu

Journal of Clinical Neuromuscular Disease
|November 23, 2012
PubMed

Insights

Inclusion body myositis, typically affecting limbs, can present as axial myopathy with progressive truncal weakness. This case expands the known clinical spectrum of this common elderly inflammatory myopathy.

Area of Science:

  • Neurology
  • Rheumatology
  • Pathology

Background:

  • Inclusion body myositis is the most frequent idiopathic inflammatory myopathy in older adults.
  • It characteristically causes proximal and distal limb weakness, predominantly affecting forearm flexors and quadriceps.
  • Axial muscle involvement is considered uncommon.

Purpose of the Study:

  • To report a rare case of inclusion body myositis presenting primarily with axial muscle weakness.
  • To highlight the expanded clinical presentation of inclusion body myositis.
  • To contribute to the understanding of idiopathic inflammatory myopathies.

Main Methods:

  • Case report of an 80-year-old male with progressive truncal weakness.
  • Clinical and neurological examination.
  • Magnetic resonance imaging (MRI) of paraspinal muscles.
  • Needle electromyography (EMG).
  • Thoracic paraspinal muscle biopsy.

Main Results:

  • The patient exhibited significant truncal weakness and stooped posture without limb weakness.
  • MRI revealed paraspinal muscle atrophy and fatty infiltration.
  • EMG confirmed an axial myopathy.
  • Muscle biopsy demonstrated features consistent with inclusion body myositis.

Conclusions:

  • This case demonstrates that inclusion body myositis can manifest with prominent axial muscle involvement.
  • The findings broaden the recognized clinical spectrum of inclusion body myositis.
  • It underscores the importance of considering inclusion body myositis in elderly patients with unexplained axial weakness.

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