/Landau-Kleffner syndrome

Landau-Kleffner syndrome

Rare Disease

MONDO:0009509

Also Known As

LKSLandau-Kleffner syndromeacquired epileptic aphasiaFESDRolandic epilepsy, intellectual disability, and speech dyspraxia, autosomal dominantRolandic epilepsy, mental retardation, and speech dyspraxia, autosomal dominantacquired aphasia with convulsive disorderacquired epileptiform aphasiaaphasia, acquired, with epilepsybenign epilepsy of childhood with centrotemporal spikescontinuous Spike and waves during slow-Wave sleep syndromeepilepsy, focal, with speech disorder and with or without impaired intellectual developmentepilepsy, focal, with speech disorder and with or without intellectual disabilityepilepsy, focal, with speech disorder and with or without mental retardation

Definition

A rare form of epileptic encephalopathy with spike-wave activation in sleep (EE-SWAS) characterized by various combinations of acquired cognitive, language, behavioral, and motor deficits associated with marked spike- and- wave activation in sleep. In Landau-Kleffner syndrome, receptive language is mainly affected, with an acquired auditory verbal agnosia.