CSWS-related autistic regression versus autistic regression without CSWS.
Continuous spike-waves during slow-wave sleep (CSWS) and Landau-Kleffner syndrome (LKS) are two clinical epileptic syndromes that are associated with the electroencephalography (EEG) pattern of electrical status epilepticus during slow wave sleep (ESES). Autistic regression occurs in approximately 30% of children with autism and is associated with an epileptiform EEG in approximately 20%. The behavioral phenotypes of CSWS, LKS, and autistic regression overlap. However, the differences in age of regression, degree and type of regression, and frequency of epilepsy and EEG abnormalities suggest that these are distinct phenotypes. CSWS with autistic regression is rare, as is autistic regression associated with ESES. The pathophysiology and as such the treatment implications for children with CSWS and autistic regression are distinct from those with autistic regression without CSWS.
Tuchman
Roberto
R
Miami Children's Hospital, Miami Children's Hospital Dan Marino Center, 2900 South Commerce Parkway, Weston, Florida, U.S.A. tuchman@att.net
D001321:Autistic Disorder / Q000175:diagnosis* / Q000453:epidemiology; D002648:Child; D002653:Child Behavior Disorders / Q000175:diagnosis / Q000453:epidemiology; D002675:Child, Preschool; D003072:Cognition Disorders / Q000175:diagnosis / Q000453:epidemiology; D015897:Comorbidity; D004569:Electroencephalography / Q000706:statistics & numerical data; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000175:diagnosis* / Q000453:epidemiology; D009483:Neuropsychological Tests; D012008:Recurrence; D012890:Sleep / Q000502:physiology*; D013226:Status Epilepticus / Q000175:diagnosis* / Q000453:epidemiology