The Landau-Kleffner Syndrome

Authors

  • Phillip L. Pearl M.D.,

    1. Department of Neurology, Children's National Medical Center, and George Washington University School of Medicine, Washington, D.C.;
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  • Enrique J. Carrazana M.D.,

    1. Department of Neurology, Children's National Medical Center, and George Washington University School of Medicine, Washington, D.C.;
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  • Gregory L. Holmes M.D.

    1. Department of Neurology, Children's National Medical Center, and George Washington University School of Medicine, Washington, D.C.;
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Address correspondence to P.L. Pearl, M.D., Children's National Medical Center, 111 Michigan Avenue NW, Dept. of Neurology, Washington, D.C. 20010; E-mail: ppearl@cnmc.org

Abstract

Landau-Kleffner syndrome (LKS), or acquired epileptiform aphasia, is an epilepsy syndrome involving progressive neuropsychological impairment related to the appearance of paroxysmal electroencephalograph (EEG) activity. LKS appears to share a common pathophysiologic mechanism with continuous spike-wave of sleep (CSWS), acquired epileptic opercular syndrome (AEOS), and even benign childhood epilepsy with centrotemporal spikes (BECTS), with differentiating factors including age of onset, area of primary epileptogenicity, and severity of clinical presentation. This article covers the clinical, diagnostic, therapeutic, and prognostic features of LKS. In a child with autistic spectrum disorder, the presence of a fluctuating clinical course or regression should raise suspicion for the presence of associated epilepsy.

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