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 P. Epileptic encephalopathy with continuous spike-waves during slow-wave sleep including Landau-Kleffner syndrome. Handb Clin Neurol. 2013;111:635-40. doi: 10.1016/B978-0-444-52891-9.00066-X.
[PMID: 23622211] [DOI: 10.1016/B978-0-444-52891-9.00066-X]
2. a, b Nickels K, Wirrell E. Electrical status epilepticus in sleep. Semin Pediatr Neurol. 2008 Jun;15(2):50-60. doi: 10.1016/j.spen.2008.03.002.
[PMID: 18555191] [DOI: 10.1016/j.spen.2008.03.002]
3. a, b Hughes JR. A review of the relationships between Landau-Kleffner syndrome, electrical status epilepticus during sleep, and continuous spike-waves during sleep. Epilepsy Behav. 2011 Feb;20(2):247-53. doi: 10.1016/j.yebeh.2010.10.015. Epub 2011 Jan 15.
[PMID: 21242107] [DOI: 10.1016/j.yebeh.2010.10.015]
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 AS, Bojko A, Lado F, Moshé SL. The spectrum of neuropsychiatric abnormalities associated with electrical status epilepticus in sleep. Brain Dev. 2000 Aug;22(5):279-95. doi: 10.1016/s0387-7604(00)00127-3.
[PMID: 10891635] [DOI: 10.1016/s0387-7604(00)00127-3]
6. a Syntax error [pubmed plugin]
7. a Mukhin KI, Muronov MB, Kholin AA, Glukhova LI, Piliia SV, Volkova EI, Golovteev AL, Pylaeva OA, Petrukhin AS. [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. Electrical status epilepticus during slow sleep: one case with sensory aphasia. Clin Electroencephalogr. 1988 Apr;19(2):111-3. doi: 10.1177/155005948801900213.
[PMID: 3396207] [DOI: 10.1177/155005948801900213]
10. a Fröscher W. Sleep and prolonged epileptic activity (status epilepticus). Epilepsy Res Suppl. 1991;2:165-76.
[PMID: 1760086]
11. a Ortega JJ, Belda VJ, Tripiana JL, Gil M, Salas R, Orenga JV, Serrano AL. [Electrical status epilepticus during sleep]. Rev Neurol. 1996 Dec;24(136):1551-3.
[PMID: 9064176]
12. a Guerrini R, Genton P, Bureau M, Parmeggiani A, Salas-Puig X, Santucci M, Bonanni P, Ambrosetto G, Dravet C. Multilobar polymicrogyria, intractable drop attack seizures, and sleep-related electrical status epilepticus. Neurology. 1998 Aug;51(2):504-12. doi: 10.1212/wnl.51.2.504.
[PMID: 9710026] [DOI: 10.1212/wnl.51.2.504]
13. a Kobayashi K, Nishibayashi N, Ohtsuka Y, Oka E, Ohtahara S. Epilepsy with electrical status epilepticus during slow sleep and secondary bilateral synchrony. Epilepsia. 1994 Sep-Oct;35(5):1097-103. doi: 10.1111/j.1528-1157.1994.tb02561.x.
[PMID: 7925158] [DOI: 10.1111/j.1528-1157.1994.tb02561.x]
14. a Okuyaz C, Aydin K, Gücüyener K, Serdaroğlu A. Treatment of electrical status epilepticus during slow-wave sleep with high-dose corticosteroid. Pediatr Neurol. 2005 Jan;32(1):64-7. doi: 10.1016/j.pediatrneurol.2004.06.020.
[PMID: 15607609] [DOI: 10.1016/j.pediatrneurol.2004.06.020]
15. a Nobutoki T, Sugai K, Fukumizu M, Hanaoka S, Sasaki M. [Continuous midazolam infusion for refractory nonconvulsive status epilepticus in children]. No To Hattatsu. 2005 Sep;37(5):369-73.
[PMID: 16164241]
16. a Wang S, Weng W, Fan P, Lee W. Levetiracetam in continuous spike waves during slow-wave sleep syndrome. Pediatr Neurol. 2008 Aug;39(2):85-90. doi: 10.1016/j.pediatrneurol.2008.04.007.
[PMID: 18639750] [DOI: 10.1016/j.pediatrneurol.2008.04.007]
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