Neurology: Case of the Month

Test Yourself — Patient 57

Opsoclonus-Myoclonus Syndrome; Neuroblastoma

  1. Which of the following is NOT a typical presentation of neuroblastoma.
    • [ A ] Horner's syndrome
    • [ B ] Myelopathy
    • [ C ] Hypotensive episodes
    • [ D ] Pain
  2. Which of the following is a typical characteristic of the opsoclonus-myoclonus syndrome?
    • [ A ] Disconjugate, randomly directed, rapid movements of the eyes
    • [ B ] Correlated bursts of EEG and EMG activity
    • [ C ] Unilateral myoclonus
    • [ D ] Persistence of opsoclonus during sleep.
  3. Which of the following statements about the opsoclonus-myoclonus syndrome is TRUE?
    • [ A ] The presence of CSF pleocytosis rules out a paraneoplastic syndrome.
    • [ B ] Thorough MR imaging of the head and body in an infant with opsoclonus- myoclonus syndrome rules out occult tumor.
    • [ C ] Cerebellar ataxia rarely occurs in association with opsoclonus and myoclonus in paraneoplastic disease.
    • [ D ] Opsoclonus-myoclonus syndrome as the presenting sign of neuroblastoma suggests an unfavorable prognosis.
    • [ E ] Screens for paraneoplastic anti-neuronal nuclear antibodies are of greater value in adults than in children presenting with the opsoclonus-myoclonus syndrome.
  4. Which of the following suggests a favorable biologic factor in neuroblastoma?
    • [ A ] Diploid DNA index
    • [ B ] Elevated levels of TrkA expression
    • [ C ] High N-myc gene copy number
    • [ D ] High mitotic-karyorrhectic index
  5. Which of the following statements about treatment of neuroblastoma is TRUE?
    • [ A ] The most important factor to consider in treating patients with neuroblastoma is tumor location.
    • [ B ] Low risk patients should receive radiation therapy only.
    • [ C ] Some cases of neuroblastoma have been found to spontaneously remit.
    • [ D ] Surgical intervention is only used when other modalities of treatment have failed.

 

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