Fatal Status Epilepticus in Dravet Syndrome

Overview

Journal Brain Sci

Publisher MDPI

Date 2020 Nov 26

PMID 33238377

Citations 4

Authors

Paola De Liso

Virginia Pironi

Massimo Mastrangelo

Domenica Battaglia

Dana Craiu

Marina Trivisano

Nicola Specchio

Rima Nabbout

Federico Vigevano

Affiliations

Soon will be listed here.

Abstract

Dravet Syndrome (DS) is burdened by high epilepsy-related premature mortality due to status epilepticus (SE). We surveyed centres within Europe through the Dravet Italia Onlus and EpiCARE network (European Reference Network for Rare and Complex Epilepsies). We collated responses on seven DS SCN1A+ patients who died following refractory SE (mean age 6.9 year, range 1.3-23.4 year); six were on valproate, clobazam, and stiripentol. All patients had previous SE. Fatal SE was always triggered by fever: either respiratory infection or one case of hexavalent vaccination. SE lasted between 80 min and 9 h and all patients received IV benzodiazepines. Four patients died during or within hours of SE; in three patients, SE was followed by coma with death occurring after 13-60 days. Our survey supports the hypothesis that unresponsive fever is a core characteristic feature of acute encephalopathy. We highlight the need for management protocols for prolonged seizures and SE in DS.

Citing Articles

The Charlotte Project: Recommendations for patient-reported outcomes and clinical parameters in Dravet syndrome through a qualitative and Delphi consensus study.

Aledo-Serrano A, Mingorance A, Villanueva V, Garcia-Penas J, Gil-Nagel A, Boronat S Front Neurol. 2022; 13:975034.

PMID: 36119672 PMC: 9481303. DOI: 10.3389/fneur.2022.975034.

Different Outcomes of Acute Encephalopathy after Status Epilepticus in Patients with Dravet Syndrome. How to Avoid Them? Comment on De Liso et al. Fatal Status Epilepticus in Dravet Syndrome. 2020, , 889.

Dravet C Brain Sci. 2021; 11(6).

PMID: 34208473 PMC: 8235089. DOI: 10.3390/brainsci11060792.

Reply to Dravet, C. Different Outcomes of Acute Encephalopathy after Status Epilepticus in Patients with Dravet Syndrome. How to Avoid Them? Comment on "De Liso et al. Fatal Status Epilepticus in Dravet Syndrome. . 2020, , 889".

De Liso P, Pironi V, Mastrangelo M, Battaglia D, Craiu D, Trivisano M Brain Sci. 2021; 11(6).

PMID: 34207311 PMC: 8234181. DOI: 10.3390/brainsci11060811.

Fenfluramine in the successful treatment of super-refractory status epilepticus in a patient with Dravet syndrome.

Millett D, Pach S Epilepsy Behav Rep. 2021; 16:100461.

PMID: 34179745 PMC: 8214086. DOI: 10.1016/j.ebr.2021.100461.

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