Acquired epileptiform opercular syndrome: a second case report, review of the literature, and comparison to the Landau-Kleffner syndrome.
A 5-year-old girl developed recurrent prolonged episodes of severe oral apraxia, dysarthria, and drooling, similar to the opercular syndrome in children. Each episode lasted several weeks to > 6 months and was associated with exacerbation of epileptiform activity in her EEG. Electrographic status epilepticus during slow wave sleep (ESES) was recorded during three of the exacerbations. The EEG improved markedly when clinical symptoms subsided. Antiepileptic drugs (AEDs) were not effective, although there was some improvement when they were combined with a ketogenic diet. A similar case was described by Roulet et al. We believe that this is a distinct epileptic syndrome, equivalent to the Landau-Kleffner syndrome (LKS).
Shafrir
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Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA.
Prensky
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AL
D000377:Agnosia / Q000175:diagnosis*; D000927:Anticonvulsants / Q000627:therapeutic use; D001037:Aphasia / Q000175:diagnosis*; D001072:Apraxias / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004041:Dietary Fats / Q000008:administration & dosage; D004569:Electroencephalography; D004750:Enteral Nutrition; D004827:Epilepsy / Q000175:diagnosis* / Q000188:drug therapy; D005260:Female; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000175:diagnosis*; D008297:Male; D012798:Sialorrhea / Q000175:diagnosis; D013226:Status Epilepticus / Q000175:diagnosis; D013577:Syndrome
D000927:Anticonvulsants; D004041:Dietary Fats