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Abstract
Treatment options for atypical forms of Landau-Kleffner syndrome (LKS) are not well
delineated. Many patients with typical LKS fail to respond to antiepileptic drug treatment,
but some benefit from multiple subpial transections (MSTs). The authors report seven
patients with autism or autistic epileptiform regression who responded in varying
degrees to MSTs after failed medical management. These patients derived from an original
cohort of 36 children (29 males, seven females, ranging from 2 years, 3 months to
11 years, 3 months, mean age = 5 years, 8 months) with a history of language delay
or regression, as well as varying degrees of social and behavioral abnormalities,
who were evaluated with video-electroencephalogram (EEG) monitoring over a 2-year
period. Fifteen patients had clinical seizures (11 of the 19 children with autistic
epileptiform regression and four of 12 autistic children). Epilepsy was refractory
to medication in seven. Surgical treatment variously involved MSTs of the left neocortex
in temporal, parietal, and frontal regions, often including regions within the classic
perisylvian language areas. One patient also had a left temporal lobectomy. In all
seven patients, seizure control or EEG improved after MSTs. Language, social, and
overall behavior improved to a moderate degree, although improvements were temporary
in most. Autistic epileptiform regression resembles LKS in that both may respond to
MST. MST is used to treat epilepsy in eloquent regions. The responsiveness of autistic
epileptiform regression to MST buttresses the argument that autistic epileptiform
regression is a form of focal epilepsy.