Chapter 44 Myoclonic epilepsies: syndromes and their treatment

摘要: Publisher Summary This chapter discusses some of the better known myoclonic epilepsies and their treatment. Early referred to as early infantile epileptic encephalopathy with suppression-burst or Ohtahara syndrome is characterized by a triad myoclonic, focal, later tonic seizures. Their clinical manifestations include onset in infancy, usually within first three months, 60% before ten days age. Erratic fragmentory myoclonus face limbs, asynchronous asymmetric, are noted massive jerks appearing later. The main seizures spasms (similar West syndrome) other ictal events being partial myoclonic. electroencephalography (EEG) pattern complex bursts high amplitude spikes sharp slow waves both during waking sleeping states. Multiple etiologies responsible for but majority cases associated structural brain damage severe bilateral injury. differentiated from seizure type erratic myoclonias, focal clusters spasms, rather than primarily. Children epilepsy infancy (SMEI), also “Dravet syndrome,” have year life development normal prior onset.