Encephalopathy with electrical status epilepticus during slow sleep or ESES syndrome including the acquired aphasia

Encephalopathy with electrical status epilepticus during sleep or ESES is an age-dependent and self-limited syndrome whose distinctive features include a characteristic age of onset (with a peak around 4–5 years), heterogeneous seizures types (mostly partial motor or unilateral seizures during sleep...

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Bibliographic Details
Published in:Clinical neurophysiology 2000-09, Vol.111, p.S94-S102
Main Authors: Tassinari, C.A, Rubboli, G, Volpi, L, Meletti, S, d'Orsi, G, Franca, M, Sabetta, A.R, Riguzzi, P, Gardella, E, Zaniboni, A, Michelucci, R
Format: Article
Language:English
Subjects:
Acquired aphasia
Aphasia - physiopathology
Aphasia - psychology
Brain Diseases - physiopathology
Electrical status epilepticus during slow sleep
Electroencephalography
Encephalopathy
Humans
Neuropsychological Tests
Sleep - physiology
Status Epilepticus - physiopathology
Status Epilepticus - psychology
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Summary:Encephalopathy with electrical status epilepticus during sleep or ESES is an age-dependent and self-limited syndrome whose distinctive features include a characteristic age of onset (with a peak around 4–5 years), heterogeneous seizures types (mostly partial motor or unilateral seizures during sleep and absences or falls while awake), a typical EEG pattern (with continuous and diffuse paroxysms occupying at least 85% of slow wave sleep) and a variable neuropsychological regression consisting of IQ decrease, reduction of language (as in acquired aphasia or Landau–Kleffner syndrome), disturbance of behaviour (psychotic states) and motor impairment (in the form of ataxia, dyspraxia, dystonia or unilateral deficit). Despite the long-term favourable outcome of epilepsy and status epilepticus during sleep (SES), the prognosis is guarded because of the persistence of severe neuropsychological and/or motor deficits in approximately half of the patients. No specific treatment has been advocated for this syndrome, but valproate sodium, benzodiazepines and ACTH have been shown to control the seizures and the SES pattern in many cases, although often only temporarily. Subpial transection is proposed in some instances as in non-regressive acquired aphasia. Recent data support the concept that ESES syndrome may include a large subset of developmental or acquired regressive conditions of infancy.
ISSN:1388-2457
1872-8952
DOI:10.1016/S1388-2457(00)00408-9