Continuous focal spikes during REM sleep in a case of acquired aphasia (Landau-Kleffner syndrome).
We report a girl 3 years and 6 months old with onset of aphasia at age 3 years and 3 months. There was no evidence of brain damage and there were no seizures. The neuropsychological evaluation showed that the girl tended to be right-handed, that aphasia was global and that other higher cortical functions seemed to be preserved. Isolated spikes and spikes-and-wave were recorded during wake over the right temporal region with rare independent contralateral abnormalities. During polysomnography (PSG), the physiological patterns of sleep were preserved and right temporal epileptiform discharges were significantly increased in all sleep stages. Maximal activation was obtained at sleep onset and during rapid eye movement (REM) sleep periods, when focal abnormalities became continuous and spread contralaterally. Repeat PSGs showed that the activation profile retained this particular trait, although subclinical discharges tended to increase during slow wave sleep (SWS). This pattern of subclinical temporal status epilepticus during REM sleep differs from the characteristic activation profile found in the syndrome of continuous spikes-and-waves during SWS. However, this profile was transient and all epileptiform changes disappeared during clinical recovery at 18 months of follow-up.
D000927:Anticonvulsants / Q000627:therapeutic use; D001037:Aphasia / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology*; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D004833:Epilepsy, Temporal Lobe / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D009483:Neuropsychological Tests; D017286:Polysomnography / Q000295:instrumentation*; D012815:Signal Processing, Computer-Assisted / Q000295:instrumentation*; D012894:Sleep Stages / Q000502:physiology*; D012895:Sleep, REM / Q000187:drug effects / Q000502:physiology; D013577:Syndrome; D013702:Temporal Lobe / Q000187:drug effects / Q000503:physiopathology
D000927:Anticonvulsants