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 syndr...

Full description

Saved in:
Bibliographic Details
Published in:European journal of paediatric neurology 1998, Vol.2 (6), p.303-311
Main Authors: Perez, Eliane Roulet, Seeck, Margitta, Mayer, Eugène, Despland, Paul-André, Tribolet, Nicolas De, Deonna, Thierry
Format: Article
Language:English
Subjects:
Acquired epileptic aphasia
Adult
Aphasia - diagnosis
Continuous spike waves during sleep
Disease Progression
Electroencephalography
Epilepsy, Complex Partial - diagnosis
Epilepsy, Complex Partial - surgery
Epileptic regression
Focal cortical dysplasia
Frontal Lobe - abnormalities
Frontal Lobe - blood supply
Frontal Lobe - surgery
Humans
Landau-Kleffner syndrome
Landau-Kleffner Syndrome - diagnosis
Landau-Kleffner Syndrome - surgery
Magnetic Resonance Imaging
Male
Neuropsychological Tests
Partial epilepsy
Recurrence
Regression (Psychology)
Sleep - physiology
Tomography, Emission-Computed, Single-Photon
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:1090-3798
1532-2130
DOI:10.1016/S1090-3798(98)80005-6