Childhood epilepsy with neuropsychological regression and continuous spike waves during sleep: epilepsy surgery in a young adult.
We describe the case of a man with a history of complex partial seizures and severe language, cognitive and behavioural regression during early childhood (3.5 years), who underwent epilepsy surgery at the age of 25 years. His early epilepsy had clinical and electroencephalogram features of the syndromes of epilepsy with continuous spike waves during sleep and acquired epileptic aphasia (Landau-Kleffner syndrome), which we considered initially to be of idiopathic origin. Seizures recurred at 19 years and presurgical investigations at 25 years showed a lateral frontal epileptic focus with spread to Broca's area and the frontal orbital regions. Histopathology revealed a focal cortical dysplasia, not visible on magnetic resonance imaging. The prolonged but reversible early regression and the residual neuropsychological disorders during adulthood were probably the result of an active left frontal epilepsy, which interfered with language and behaviour during development. Our findings raise the question of the role of focal cortical dysplasia as an aetiology in the syndromes of epilepsy with continuous spike waves during sleep and acquired epileptic aphasia.
Roulet Perez
E
E
Department of Pediatrics, University Hospital, Lausanne, Switzerland.
Seeck
M
M
Mayer
E
E
Despland
P A
PA
de Tribolet
N
N
Deonna
T
T
D000328:Adult; D001037:Aphasia / Q000175:diagnosis; D018450:Disease Progression; D004569:Electroencephalography; D017029:Epilepsy, Complex Partial / Q000175:diagnosis* / Q000601:surgery*; D005625:Frontal Lobe / Q000002:abnormalities / Q000098:blood supply / Q000601:surgery; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000175:diagnosis / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests; D012008:Recurrence; D012043:Regression, Psychology; D012890:Sleep / Q000502:physiology*; D015899:Tomography, Emission-Computed, Single-Photon