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inclusion_body_myositis

inclusion body myositis (IBM)

introduction

3 main forms

sporadic (sIBM)

  • mixed autoimmune and degenerative processes
  • incidence increases with age and symptoms usually begin after 50 years of age
  • 50 cases per million in those aged over 50 years (compared with 15 cases per million in all ages)
  • the most common acquired muscle disorder seen in people over 50
  • 20% have onset before age 50yrs
  • weakness comes on slowly (over months or years)
  • slightly more common in men than women
  • may become unable to perform daily living activities and most require assistive devices within 5 to 10 years of symptom onset
  • may cause potentially fatal dysphagia
  • no effective treatment exists

familial IBM

  • occurs in siblings but not passed to children

hereditary (hIBM)

  • heterogeneous group of very rare hereditary conditions
  • IBM2:
    • mainly Iranian Jew ethnicity
    • if the quadriceps are spared but the hamstrings and iliopsoas are severely affected in a person between ages of 20-40, it is very likely IBM2 will be at the top of the differential diagnosis.

Dx

  • muscle biopsy:
    • inclusion bodies, rimmed vacuoles and accumulation of aberrant proteins similar to those found in senile plaques of Alzheimer's disease
inclusion_body_myositis.txt · Last modified: 2013/09/27 06:53 by 127.0.0.1

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