Clinical and neuropathologic findings in a case of severe myoclonic epilepsy of infancy

Epilepsia. May-Jun 1990;31(3):287-91. doi: 10.1111/j.1528-1157.1990.tb05378.x.


The autopsy of a 19-month-old boy with severe myoclonic epilepsy of infancy (SMEI) and sudden unexpected death (SUD) revealed several developmental brain abnormalities. The most striking features were microdysgenesis of cerebellum and cerebral cortex and threefold spinal cord channels with surrounding ectopic tissue. Hippocampus and brainstem were normal.

Publication types

  • Case Reports

MeSH terms

  • Central Nervous System / metabolism
  • Central Nervous System / pathology*
  • Electroencephalography
  • Epilepsies, Myoclonic / metabolism
  • Epilepsies, Myoclonic / pathology
  • Epilepsies, Myoclonic / physiopathology*
  • Humans
  • Immunohistochemistry
  • Infant
  • Male