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Results of an international Delphi consensus in epilepsy with myoclonic atonic seizures/ Doose syndrome

Joshi, C; Nickels, K; Demarest, S; Eltze, C; Cross, JH; Wirrell, E; (2021) Results of an international Delphi consensus in epilepsy with myoclonic atonic seizures/ Doose syndrome. Seizure , 85 pp. 12-18. 10.1016/j.seizure.2020.11.017.

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International Doose syndrome Delphi final.pdf - Accepted version
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Table with Doose Delphi results- August 7.pdf - Accepted version
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Cross_1-s2.0-S1059131120303812-mmc1.pdf - Accepted version
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Cross_1-s2.0-S1059131120303812-mmc2.pdf - Accepted version
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1-s2.0-S1059131120303812-mmc3.pdf - Accepted version
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1-s2.0-S1059131120303812-mmc4.pdf - Accepted version
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Abstract

OBJECTIVE: To establish a standard framework for early phenotypic diagnosis, investigations, expected findings from investigations, evolution, effective therapies and prognosis in the syndrome of Epilepsy with myoclonic atonic seizures (EMAS) / Doose syndrome. METHODS: A core study group (CSG) interested in EMAS was convened. CSG then identified and nominated 15 experts in the field of EMAS. This expert panel (EP) from English speaking nations was invited to participate in anonymous questionnaires. A literature review was provided to them (supplement 1). Three rounds of questionnaires were sent to identify areas of consensus, strength of consensus and areas of contention. RESULTS: Strong consensus was obtained regarding the clinical phenotype of EMAS: myoclonic atonic seizure was identified among others as a mandatory seizure type with typical onset of afebrile seizures between one and six years. A new term "stormy phase" (SP) was designated to delineate a characteristic phenotypic evolution in EMAS patients associated with seizure worsening. Strong consensus regarding the existence and time of onset of the SP, mandatory investigations to be performed early and later in the clinical course of EMAS, first and second tier treatment and prognostic factors for poor outcome were identified. Areas of lack of consensus included some seizure types that are necessary to diagnose EMAS, interictal EEG findings that prognosticate the course of EMAS, overall duration of SP, time to complete remission, and best approach to treat drug resistant EMAS. SIGNIFICANCE: Expert consensus on core diagnostic criteria of EMAS necessary for natural history studies, phenotype-genotype correlations, and clinical trials including comparative studies was demonstrated. Areas of disagreements (especially prognostic features; treatment options) need further research.

Type: Article
Title: Results of an international Delphi consensus in epilepsy with myoclonic atonic seizures/ Doose syndrome
Location: England
DOI: 10.1016/j.seizure.2020.11.017
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Consensus, Delphi, Doose, EMAS, Stormy phase
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Neurosciences Dept
URI: https://discovery.ucl.ac.uk/id/eprint/10120227
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