Muscle pathology and diseases are a high-yield topic for neurology examinations as many of these disorders have characteristic or pathognomonic findings that allow for easy test question making! For this topic, pathology slide interpretation will be a great skill to have! Luckily this chapter has dozens of high-quality images with which to practice.

Author: Brian Hanrahan MD

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Acquired Myopathies

Idiopathic inflammatory myopathies

Polymyositis

  • A female-predominant disease seen in one’s adult years that presents with subacute symmetric proximal weakness and pain.
  • Can be associated with rheumatoid arthritis, HIV, or underlying malignancy.
  • Muscle biopsy shows endomysial and perivascular monocytic inflammation and necrosis with regeneration.

Dermatomyositis

  • A female-predominant disease which presents with subacute proximal weakness and pain.
  • Dermatologic manifestations include a heliotrope rash on eyelids and an erythematous rash of the face or neck.
  • Can be associated with connective tissue disease, malignancy, and interstitial lung disease.
  • Muscle biopsy shows perifascicular inflammation and atrophy with sparing of the central fascicle.

Inclusion body myositis (IBM)

  • A male-predominant slowly progressive idiopathic inflammatory condition of patients over the age of 50.

  • Clinical features include asymmetric weakness of the finger flexors and the quadriceps muscles.

Treatment

  • Polymyositis and dermatomyositis are responsive to immunosuppressive therapies (steroids, methotrexate, azathioprine, mycophenolate, etc.) while IBM is not.


 

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