Neurology: Case of the Month

Test Yourself — Patient 9

Lissencephaly

  1. Hypsarrhythmia refers to which of the following:
    • [ A ] An inability to perform rapid alternating movements of the lower extremities.
    • [ B ] A high voltage, chaotic, multifocal spike EEG pattern.
    • [ C ] A normal EEG pattern seen in children with benign myoclonic seizures.
    • [ D ] A brain malformation specific pattern.
  2. Classic or type 1 lissencephaly is seen in which of the following conditions:
    • [ A ] Miller-Dieker lissencephaly
    • [ B ] Isolated lissencephaly sequence
    • [ C ] X-linked lissencephaly
    • [ D ] All of the above
  3. Chromosome abnormalities seen in Miller-Dieker lissencephaly include:
    • [ A ] Deletions of the short arm of the 17th chromosome
    • [ B ] Microdeletions of the LIS-1 gene only detectable by fluorescent in situ hybridization (FISH)
    • [ C ] Both A and B
    • [ D ] None of the above
  4. With regards to Miller-Dieker lissencephaly or isolated lissencephaly sequence, which statement is TRUE:
    • [ A ] These patients have a normal life span.
    • [ B ] Most patients have seizures at some point in their life.
    • [ C ] Normal intellectual development is common.
    • [ D ] Patients may be able to care for themselves.
  5. In addition to lissencephaly syndromes due to deletions of the LIS-1 gene (Miller-Dieker lissencephaly and isolated lissencephaly), other genetic disorders associated with lissencephaly include:
    • [ A ] Brain malformations associated with Walker-Warburg syndrome
    • [ B ] X-linked brain malformation
    • [ C ] Polymicrogyria
    • [ D ] A and B

 

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