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Continuous spikes and waves during sleep(CSWS; CSWSS)

MedGen UID:
1790601
•Concept ID:
C5552731
•
Disease or Syndrome
Synonyms: CONTINUOUS SPIKE AND WAVES DURING SLOW-WAVE SLEEP SYNDROME; Continuous spike-wave during slow sleep syndrome
 
Monarch Initiative: MONDO:0019123
OMIM®: 245570

Definition

Continuous spikes and waves during sleep (CSWS) is a rare epileptic encephalopathy of childhood characterized by seizures, an electroencephalographic (EEG) pattern of electrical status epilepticus in sleep (ESES) and neurocognitive regression in at least 2 domains of development. [from MONDO]

Professional guidelines

PubMed

Long-term response to high-dose diazepam treatment in continuous spikes and waves during sleep.
Sánchez Fernández I, Peters JM, An S, Bergin AM, Takeoka M, Rotenberg A, Kothare SV, Riviello JJ Jr, Loddenkemper T
Pediatr Neurol 2013 Sep;49(3):163-170.e4. doi: 10.1016/j.pediatrneurol.2013.04.027. PMID: 23953953Free PMC Article
Landau-Kleffner syndrome and CSWS syndrome: treatment with intravenous immunoglobulins.
Arts WF, Aarsen FK, Scheltens-de Boer M, Catsman-Berrevoets CE
Epilepsia 2009 Aug;50 Suppl 7:55-8. doi: 10.1111/j.1528-1167.2009.02221.x. PMID: 19682053
When to start drug treatment for childhood epilepsy: the clinical-epidemiological evidence.
Arts WF, Geerts AT
Eur J Paediatr Neurol 2009 Mar;13(2):93-101. Epub 2008 Jun 24 doi: 10.1016/j.ejpn.2008.02.010. PMID: 18567515

Recent clinical studies

Etiology

Clinical spectrum and treatment outcome of 95 children with continuous spikes and waves during sleep (CSWS).
Sonnek B, Döring JH, Mütze U, Schubert-Bast S, Bast T, Balke D, Reuner G, Schuler E, Klabunde-Cherwon A, Hoffmann GF, Kölker S, Syrbe S
Eur J Paediatr Neurol 2021 Jan;30:121-127. Epub 2020 Oct 24 doi: 10.1016/j.ejpn.2020.10.010. PMID: 33132036
Levels of Melatonin in Continuous Spikes and Waves During Sleep.
Ayça S, Aksoy HU, Taştan İ, Polat M
J Child Neurol 2019 May;34(6):309-312. Epub 2019 Feb 14 doi: 10.1177/0883073819828580. PMID: 30761941
Early identification of epileptic encephalopathy with continuous spikes-and-waves during sleep: A case-control study.
Desprairies C, Dozières-Puyravel B, Ilea A, Bellavoine V, Nasser H, Delanöe C, Auvin S
Eur J Paediatr Neurol 2018 Sep;22(5):837-844. Epub 2018 Apr 27 doi: 10.1016/j.ejpn.2018.04.009. PMID: 29739701
Current understanding and neurobiology of epileptic encephalopathies.
Auvin S, Cilio MR, Vezzani A
Neurobiol Dis 2016 Aug;92(Pt A):72-89. Epub 2016 Mar 16 doi: 10.1016/j.nbd.2016.03.007. PMID: 26992889
Continuous spikes and waves during sleep in children with shunted hydrocephalus.
Veggiotti P, Beccaria F, Papalia G, Termine C, Piazza F, Lanzi G
Childs Nerv Syst 1998 Apr-May;14(4-5):188-94. doi: 10.1007/s003810050209. PMID: 9660121

Diagnosis

Continuous Spikes and Waves During Sleep (CSWS), Severe Epileptic Encephalopathy, and Choreoathetosis due to Mutations in FRRS1L.
Mir A, Amer F, Ali M, Alotaibi W, Alotaibi M, Hedaithy A, Aldurayhim F, Hussain F, Bashir S, Housawi Y
Clin EEG Neurosci 2023 Sep;54(5):526-533. Epub 2022 Jul 11 doi: 10.1177/15500594221112508. PMID: 35815844
Clinical spectrum and treatment outcome of 95 children with continuous spikes and waves during sleep (CSWS).
Sonnek B, Döring JH, Mütze U, Schubert-Bast S, Bast T, Balke D, Reuner G, Schuler E, Klabunde-Cherwon A, Hoffmann GF, Kölker S, Syrbe S
Eur J Paediatr Neurol 2021 Jan;30:121-127. Epub 2020 Oct 24 doi: 10.1016/j.ejpn.2020.10.010. PMID: 33132036
Early identification of epileptic encephalopathy with continuous spikes-and-waves during sleep: A case-control study.
Desprairies C, Dozières-Puyravel B, Ilea A, Bellavoine V, Nasser H, Delanöe C, Auvin S
Eur J Paediatr Neurol 2018 Sep;22(5):837-844. Epub 2018 Apr 27 doi: 10.1016/j.ejpn.2018.04.009. PMID: 29739701
Epileptic encephalopathy with continuous spikes and waves during sleep.
Striano P, Capovilla G
Curr Neurol Neurosci Rep 2013 Jul;13(7):360. doi: 10.1007/s11910-013-0360-5. PMID: 23666433
Continuous spikes and waves during sleep in children with shunted hydrocephalus.
Veggiotti P, Beccaria F, Papalia G, Termine C, Piazza F, Lanzi G
Childs Nerv Syst 1998 Apr-May;14(4-5):188-94. doi: 10.1007/s003810050209. PMID: 9660121

Therapy

Serum inflammatory mediators correlate with disease activity in electrical status epilepticus in sleep (ESES) syndrome.
van den Munckhof B, de Vries EE, Braun KP, Boss HM, Willemsen MA, van Royen-Kerkhof A, de Jager W, Jansen FE
Epilepsia 2016 Feb;57(2):e45-50. Epub 2015 Dec 14 doi: 10.1111/epi.13274. PMID: 26666401
Treatment for continuous spikes and waves during sleep (CSWS): survey on treatment choices in North America.
Sánchez Fernández I, Chapman K, Peters JM, Klehm J, Jackson MC, Berg AT, Loddenkemper T
Epilepsia 2014 Jul;55(7):1099-108. Epub 2014 Jun 10 doi: 10.1111/epi.12678. PMID: 24917485
Epileptic encephalopathy with continuous spikes and waves during sleep.
Striano P, Capovilla G
Curr Neurol Neurosci Rep 2013 Jul;13(7):360. doi: 10.1007/s11910-013-0360-5. PMID: 23666433
A revisited strategy for antiepileptic drug development in children: designing an initial exploratory step.
Chiron C, Kassai B, Dulac O, Pons G, Nabbout R
CNS Drugs 2013 Mar;27(3):185-95. doi: 10.1007/s40263-012-0035-9. PMID: 23345029
Clinical staging and electroencephalographic evolution of continuous spikes and waves during sleep.
Fernández IS, Peters JM, Hadjiloizou S, Prabhu SP, Zarowski M, Stannard KM, Takeoka M, Rotenberg A, Kothare SV, Loddenkemper T
Epilepsia 2012 Jul;53(7):1185-95. Epub 2012 May 11 doi: 10.1111/j.1528-1167.2012.03507.x. PMID: 22578248

Prognosis

Population-based study of rare epilepsy incidence in a US urban population.
Barbour K, Tian N, Yozawitz EG, Wolf S, McGoldrick PE, Sands TT, Nelson A, Basma N, Grinspan ZM
Epilepsia 2024 Aug;65(8):2341-2353. Epub 2024 May 25 doi: 10.1111/epi.18029. PMID: 38795333Free PMC Article
Long-term response to high-dose diazepam treatment in continuous spikes and waves during sleep.
Sánchez Fernández I, Peters JM, An S, Bergin AM, Takeoka M, Rotenberg A, Kothare SV, Riviello JJ Jr, Loddenkemper T
Pediatr Neurol 2013 Sep;49(3):163-170.e4. doi: 10.1016/j.pediatrneurol.2013.04.027. PMID: 23953953Free PMC Article
Epileptic encephalopathy with continuous spikes and waves during sleep.
Striano P, Capovilla G
Curr Neurol Neurosci Rep 2013 Jul;13(7):360. doi: 10.1007/s11910-013-0360-5. PMID: 23666433
Clinical staging and electroencephalographic evolution of continuous spikes and waves during sleep.
Fernández IS, Peters JM, Hadjiloizou S, Prabhu SP, Zarowski M, Stannard KM, Takeoka M, Rotenberg A, Kothare SV, Loddenkemper T
Epilepsia 2012 Jul;53(7):1185-95. Epub 2012 May 11 doi: 10.1111/j.1528-1167.2012.03507.x. PMID: 22578248
When to start drug treatment for childhood epilepsy: the clinical-epidemiological evidence.
Arts WF, Geerts AT
Eur J Paediatr Neurol 2009 Mar;13(2):93-101. Epub 2008 Jun 24 doi: 10.1016/j.ejpn.2008.02.010. PMID: 18567515

Clinical prediction guides

Clinical spectrum and treatment outcome of 95 children with continuous spikes and waves during sleep (CSWS).
Sonnek B, Döring JH, Mütze U, Schubert-Bast S, Bast T, Balke D, Reuner G, Schuler E, Klabunde-Cherwon A, Hoffmann GF, Kölker S, Syrbe S
Eur J Paediatr Neurol 2021 Jan;30:121-127. Epub 2020 Oct 24 doi: 10.1016/j.ejpn.2020.10.010. PMID: 33132036
Long-term response to high-dose diazepam treatment in continuous spikes and waves during sleep.
Sánchez Fernández I, Peters JM, An S, Bergin AM, Takeoka M, Rotenberg A, Kothare SV, Riviello JJ Jr, Loddenkemper T
Pediatr Neurol 2013 Sep;49(3):163-170.e4. doi: 10.1016/j.pediatrneurol.2013.04.027. PMID: 23953953Free PMC Article
A revisited strategy for antiepileptic drug development in children: designing an initial exploratory step.
Chiron C, Kassai B, Dulac O, Pons G, Nabbout R
CNS Drugs 2013 Mar;27(3):185-95. doi: 10.1007/s40263-012-0035-9. PMID: 23345029
Metabolic evidence for remote inhibition in epilepsies with continuous spike-waves during sleep.
De Tiège X, Ligot N, Goldman S, Poznanski N, de Saint Martin A, Van Bogaert P
Neuroimage 2008 Apr 1;40(2):802-810. Epub 2007 Dec 8 doi: 10.1016/j.neuroimage.2007.11.043. PMID: 18201907
Regional cerebral glucose metabolism in epilepsies with continuous spikes and waves during sleep.
De Tiège X, Goldman S, Laureys S, Verheulpen D, Chiron C, Wetzburger C, Paquier P, Chaigne D, Poznanski N, Jambaqué I, Hirsch E, Dulac O, Van Bogaert P
Neurology 2004 Sep 14;63(5):853-7. doi: 10.1212/01.wnl.0000137015.04054.2c. PMID: 15365136

Recent systematic reviews

Treatment of electrical status epilepticus in sleep: A pooled analysis of 575 cases.
van den Munckhof B, van Dee V, Sagi L, Caraballo RH, Veggiotti P, Liukkonen E, Loddenkemper T, Sánchez Fernández I, Buzatu M, Bulteau C, Braun KP, Jansen FE
Epilepsia 2015 Nov;56(11):1738-46. Epub 2015 Sep 4 doi: 10.1111/epi.13128. PMID: 26337159

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