Neurology: Case of the Month

Test Yourself — Patient 55

Subacute Sclerosing Panencephalitis (SSPE)

1. Which of the following statements about the epidemiology of SSPE is FALSE:
   • [ A ] Most patients who develop SSPE had measles before two years of age.
   • [ B ] Some patients may give no history of measles infection.
   • [ C ] SSPE is seen more frequently in females than males.
   • [ D ] There is little compelling evidence that measles vaccination causes SSPE.
2. All of the following are TRUE statements concerning subacute sclerosing panencephalitis (SSPE), EXCEPT:
   • [ A ] The first stage of SSPE is most often characterized by personality changes and difficulty in school or work.
   • [ B ] SSPE is caused by a persistent CNS infection by the rubella virus.
   • [ C ] The demyelination seen in SSPE usually begins in the occipital region and extends frontally.
   • [ D ] Most patients with SSPE live less than three years following diagnosis.
3. The EEG in SSPE classically shows:
   • [ A ] Periodic high-voltage generalized slow and sharp wave complexes occurring every 5-10 sec.
   • [ B ] Periodic generalized sharp waves occurring every second.
   • [ C ] Periodic lateralized spike and wave discharges.
   • [ D ] Generalized asynchronous slow waves.
4. Which of the following statements is NOT TRUE in regards to patients with SSPE:
   • [ A ] Immunoglobulin levels in the CSF are elevated.
   • [ B ] Oligoclonal banding is uncommon in the CSF.
   • [ C ] Cowdry A inclusion bodies may be seen in the nuclei of neurons.
   • [ D ] EEG changes are associated with myoclonic activity.
5. Which of the following is NOT one of the criteria used to make the diagnosis of SSPE:
   • [ A ] Characteristic EEG changes.
   • [ B ] Elevated CSF measles antibody titers.
   • [ C ] Clinical history of progressive cognitive decline and myoclonus.
   • [ D ] MRI changes with increased signal in the white matter on T2-weighted images.

 

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