Neurology: Case of the Month

Test Yourself — Patient 78

Paraneoplastic cerebellar ataxia and encephalopathy, associated with anti-neuronal nuclear antibodies (ANNA-1). [in association with presynaptic neuromuscular transmission defect (subclinical Lambert-Eaton myasthenic syndrome) and small-cell lung carcinoma]

1. Which of the following antibodies has NOT been frequently associated with paraneoplastic neurologic disease?
   • [ A ] anti-Ri
   • [ B ] anti-GQ1b
   • [ C ] anti-P/Q type voltage-gated calcium channel
   • [ D ] anti-amphiphysin
   • [ E ] anti-recoverin
2. Which of the following is the most effective treatment for paraneoplastic encephalomyelitis (PEM)?
   • [ A ] Plasma exchange
   • [ B ] Intravenous methylprednisolone
   • [ C ] Cyclophosphamide
   • [ D ] Intravenous immunoglobulin
   • [ E ] None of the above
3. Which of the following factors have NOT been associated with early mortality in ANNA-1 (anti-Hu) associated paraneoplastic encephalomyelitis?
   • [ A ] Female gender
   • [ B ] Age > 60
   • [ C ] Failure to treat the underlying tumor
   • [ D ] Advanced disability at diagnosis
   • [ E ] All of the above
4. Which of the following statements are TRUE?
   • [ A ] In nearly all paraneoplastic syndromes, an individual patient will exhibit a single paraneoplastic antibody detectable in the serum.
   • [ B ] In paraneoplastic syndromes associated with small-cell lung cancer, tumors are nearly always widely disseminated at the time of diagnosis.
   • [ C ] Passive transfer of disease features to an animal model has been successfully accomplished with sera from patients with the Lambert-Eaton myasthenic syndrome.
   • [ D ] Both A and C are true.
   • [ E ] None of the above are true.
5. In ANNA-1 associated paraneoplastic disease, negative CT or MR imaging of the chest, together with negative bronchoscopic evaluation, is adequate to exclude an underlying small-cell carcinoma.
   • [ A ] True
   • [ B ] False

 

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