Epileptic encephalopathy with continuous spike-waves during sleep

Epileptic encephalopathy with continuous spike-waves during sleep (ECSWS)
Epileptic encephalopathy with continuous spike-waves during slow-wave sleep (CSWS)

  • a spectrum of epileptic conditions best defined by the association of cognitive or behavioral impairment acquired during childhood and not related to another factor other than the presence of abundant interictal epileptiform discharges (IED) during sleep[1]
  • characterised by infrequent nocturnal focal seizures, atypical absences related to secondary bilateral synchrony, negative myoclonia, and atonic and rare generalized tonic-clonic seizures.
  • Landau-Kleffner syndrome (LKS) is a particular presentation where acquired aphasia is the core symptom
  • LKS and CSWS are said to “describe the clinical epileptic syndromes seen with ESES. There is an overlap between the two syndromes in that patients with CSWS have more global regression and more problematic epilepsy than those with LKS, who had only acquired auditory agnosia and fewer seizures[2]
  • The EEG patterns CSWS and electrical status epilepticus during sleep (ESES) are likely equivalent terms. CSWS is used by some authors, and ESES by others[3]
  • The term “epilepsy with continuous spike and wave during slow sleep” was adopted by the Commission on Classification and Terminology of the International League Against Epilepsy in 1989[4]
  • transition from childhood epilepsy with centro-temporal spikes (CECTS) into LKS and CSWS has been shown to occur particularly when there are atypical signs, high discharge rates, prolonged duration of ESES, cognitive dysfunction, and poor responsiveness to medications[5].

Electrical status epilepticus during sleep

Patry et al. in 1971[6] decribed six children in whom sleep induces, night after night and sometimes over a period of many years, a dramatic modification of the EEG, very characteristic of petit mal status but without any clinical accompaniment nor changes in normal sleep patterns. Lasting throughout the whole night, it subsides upon awakening. They proposed to call the phenomenon an “electrical status epilepticus.”

ESES can be divided into symptomatic and idiopathic forms[3][7]. Symptomatic forms are associated with local structural lesions on MRI in whom ther are hemiclonic, secondarily generalized, and automotor seizures with continuous regional or diffuse epileptiform activity and also marked cognitive disturbances.

The idiopathic form was characterized by early normal development, mostly “rolandic” attacks, atypical absences, negative myoclonus, exclusively diffuse epileptiform activity, cortical subatrophy on MRI, and some cognitive disturbances.

  • acquired aphasia[8]; sensory aphasia followed later by motor aphasia[9], usually severe and called “epileptic aphasia”[10]
  • cognitive function impairment
  • neuropsychological impairment (behaviour problems)
  • motor impairment (ataxia, dyspraxia, dystonia, or some unilateral deficit)
  • all types of generalized seizures may occur
  • improvement in seizures usually is associated with improvement in cognition followiing AED institution
  • continuous, diffuse, bilateral spike-waves during slow-wave sleep
  • in CSWS EEG foci is predominantly in the frontotemporal or frontocentral areas, whereas in LKS the EEG foci is in the posterior temporal areas[2]
  • in ESES nearly continuous, irregular generalized spike–wave complexes, seen in at least 85% of the sleep record and with minimal abnormalities during wakefulness[11]
  • the bilateral complexes in ESES represent secondary bilateral synchrony[12][13]
  • prednisolone[14], benzodiazepines[15], and also levetiracetam[16] have been found to be effective in patients with CSWS.

1. a Van Bogaert Patrick. Handb Clin Neurol. 2013;111:635-40. PMID : 23622211
2. a, b Nickels Katherine et al. Semin Pediatr Neurol. 2008 Jun;15(2):50-60. PMID : 18555191
3. a, b Hughes John R. Epilepsy Behav. 2011 Feb;20(2):247-53. PMID : 21242107
4. a Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia 1989;30:389–99.
5. a Galanopoulou A S et al. Brain Dev. 2000 Aug;22(5):279-95. PMID : 10891635
6. a Syntax error [pubmed plugin]
7. a Mukhin K Iu, Muronov M B, Kholin A A, Glukhova L Iu, Piliia S V, Volkova E Iu, Golovteev A L, Pylaeva O A, Petrukhin A S. [Epilepsy with electrical status epilepticus during slow sleep: diagnostic criteria and approaches to therapy]. Zh Nevrol Psikhiatr Im S S Korsakova. 2006;106(4):4-9. PMID : 16737153
8. a Billard, Catherine & Autret, A & Laffont, F & Giovanni, Elysa & Lucas, B & Santini, J & Dulac, O & Plouin, Perrine. (1982). Acquired aphasia in epileptic children–four cases with electrical infraclinic status epilepticus during sleep (author's transl). Revue d'électroencéphalographie et de neurophysiologie clinique. 11. 457-67.
9. a DeMarco P. Clin Electroencephalogr. 1988 Apr;19(2):111-3. PMID : 3396207
10. a Fröscher W. Epilepsy Res Suppl. 1991;2:165-76. PMID : 1760086
11. a Ortega J J et al. Rev Neurol. 1996 Dec;24(136):1551-3. PMID : 9064176
12. a Guerrini R et al. Neurology. 1998 Aug;51(2):504-12. PMID : 9710026
13. a Kobayashi K et al. Epilepsia. 1994 Sep-Oct;35(5):1097-103. PMID : 7925158
14. a Okuyaz Cetin et al. Pediatr Neurol. 2005 Jan;32(1):64-7. PMID : 15607609
15. a Nobutoki Tatsuro et al. No To Hattatsu. 2005 Sep;37(5):369-73. PMID : 16164241
16. a Wang Shi-Bing et al. Pediatr Neurol. 2008 Aug;39(2):85-90. PMID : 18639750
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