Fatal Status Epilepticus in Dravet Syndrome Article Swipe
Paola De Liso
,
Virginia Pironi
,
Massimo Mastrangelo
,
Domenica Battaglia
,
Dana Craiu
,
Marina Trivisano
,
Nicola Specchio
,
Rima Nabbout
,
Federico Vigevano
·
YOU?
·
· 2020
· Open Access
·
· DOI: https://doi.org/10.3390/brainsci10110889
YOU?
·
· 2020
· Open Access
·
· DOI: https://doi.org/10.3390/brainsci10110889
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.
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Metadata
- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.3390/brainsci10110889
- https://www.mdpi.com/2076-3425/10/11/889/pdf?version=1606111228
- OA Status
- gold
- Cited By
- 16
- References
- 26
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W3108026517
All OpenAlex metadata
Raw OpenAlex JSON
- OpenAlex ID
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https://openalex.org/W3108026517Canonical identifier for this work in OpenAlex
- DOI
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https://doi.org/10.3390/brainsci10110889Digital Object Identifier
- Title
-
Fatal Status Epilepticus in Dravet SyndromeWork title
- Type
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articleOpenAlex work type
- Language
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enPrimary language
- Publication year
-
2020Year of publication
- Publication date
-
2020-11-23Full publication date if available
- Authors
-
Paola De Liso, Virginia Pironi, Massimo Mastrangelo, Domenica Battaglia, Dana Craiu, Marina Trivisano, Nicola Specchio, Rima Nabbout, Federico VigevanoList of authors in order
- Landing page
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https://doi.org/10.3390/brainsci10110889Publisher landing page
- PDF URL
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https://www.mdpi.com/2076-3425/10/11/889/pdf?version=1606111228Direct link to full text PDF
- Open access
-
YesWhether a free full text is available
- OA status
-
goldOpen access status per OpenAlex
- OA URL
-
https://www.mdpi.com/2076-3425/10/11/889/pdf?version=1606111228Direct OA link when available
- Concepts
-
Dravet syndrome, Status epilepticus, Clobazam, Medicine, Coma (optics), Pediatrics, Epilepsy, Refractory (planetary science), Encephalopathy, Anesthesia, Internal medicine, Psychiatry, Astrobiology, Physics, OpticsTop concepts (fields/topics) attached by OpenAlex
- Cited by
-
16Total citation count in OpenAlex
- Citations by year (recent)
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2025: 4, 2023: 3, 2022: 4, 2021: 4, 2020: 1Per-year citation counts (last 5 years)
- References (count)
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26Number of works referenced by this work
- Related works (count)
-
10Other works algorithmically related by OpenAlex
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