Neurology: Case of the Month

Test Yourself — Patient 6

Inclusion Body Myositis

1. The following statement is true about sporadic Inclusion Body Myositis (sIBM):
   • [ A ] occurs more often in females
   • [ B ] usually presents before the age of 50
   • [ C ] distal muscle weakness is rare
   • [ D ] none of the above are true
2. The following muscle biopsy feature is not seen in hereditary Inclusion Body Myositis (hIBM):
   • [ A ] rimmed vacuoles
   • [ B ] atrophic denervated fibers
   • [ C ] eosinophilic inclusions
   • [ D ] mild to moderate inflammation
3. The following EMG findings would typically be seen in a patient with sporadic Inclusion Body Myositis (sIBM):
   • [ A ] myopathic units
   • [ B ] fibrillations and positive sharp waves
   • [ C ] polyphasics of normal and longer durations and increased amplitudes
   • [ D ] all of the above
4. All of the following statements are true regarding sporadic IBM (sIBM) EXCEPT:
   • [ A ] the muscle pathology of rimmed vacuoles and eosinophilic inclusions is specific
   • [ B ] steroids are generally ineffective in the treatment of sIBM
   • [ C ] the etiology is thought to be via an immune-mediated mechanism
   • [ D ] dysphagia is a common symptom
5. All of the following are helpful in differentiating sporadic IBM (sIBM) from polymyositis EXCEPT:
   • [ A ] the presence of lymphocytes on muscle biopsy
   • [ B ] the response to immunosuppressant therapy
   • [ C ] distal weakness in the upper extremities
   • [ D ] the presence of beta-amyloid, phosphorylated tau, and ubiquitin on muscle biopsy

 

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