Treatment options for atypical forms of Landau-Kleffner syndrome (LKS) are
not well delineated. Many patients with typical LKS fail to respond to anti
epileptic drug treatment, but some benefit from multiple subpial transectio
ns (MSTs). The authors report seven patients with autism or autistic epilep
tiform regression who responded in varying degrees to MSTs after failed med
ical management. These patients derived from an original cohort of 36 child
ren (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 abnormaliti
es, who were evaluated with,ideo-electroencephalogram (EEG) monitoring over
a 2-year period. Fifteen patients had clinical seizures (11 of the 19 chil
dren with autistic epileptiform regression and four of 12 autistic children
), Epilepsy was refractory to medication in seven, Surgical treatment vario
usly involved MSTs of the left neocortex in temporal, parietal, and frontal
regions, often including regions within the classic perisylvian language a
reas. 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 tempora
ry in most. Autistic epileptiform regression resembles LKS in that both may
respond to MST. MST is used to treat epilepsy in eloquent regions. The res
ponsiveness of autistic epileptiform regression to MST buttresses the argum
ent that autistic epileptiform regression is a form of focal epilepsy. (C)
1999 by Elsevier Science Inc. All rights reserved.