New-onset refractory status epilepticus with claustrum damage: definition of the clinical and neuroimaging features

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00843989%3A_____%2F17%3AE0106347" target="_blank" >RIV/00843989:_____/17:E0106347 - isvavai.cz</a>

Result on the web

<a href="http://dx.doi.org/10.3389/fneur.2017.00111" target="_blank" >http://dx.doi.org/10.3389/fneur.2017.00111</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.3389/fneur.2017.00111" target="_blank" >10.3389/fneur.2017.00111</a>

Result language

angličtina

Original language name

New-onset refractory status epilepticus with claustrum damage: definition of the clinical and neuroimaging features

Original language description

New-onset refractory status epilepticus (NORSE) is a rare but challenging condition occurring in a previously healthy patient, often with no identifiable cause. We describe the electro-clinical features and outcomes in a group of patients with NORSE who all demonstrated a typical magnetic resonance imaging (MRI) sign characterized by bilateral lesions of the claustrum. The group includes 31 patients (12 personal and 19 previously published cases; 17 females; mean age of 25 years). Fever preceded status epilepticus (SE) in 28 patients, by a mean of 6 days. SE was refractory/super-refractory in 74% of the patients, requiring third-line agents and a median of 15 days staying in an intensive care unit. Focal motor and tonic-clonic seizures were observed in 90%, complex partial seizures in 14%, and myoclonic seizures in 14% of the cases. All patients showed T2/FLAIR hyperintense foci in bilateral claustrum, appearing on average 10 days after SE onset. Other limbic (hippocampus, insular) alterations were present in 53% of patients. Within the personal cases, extensive search for known autoantibodies was inconclusive, though 7 of 11 patients had cerebrospinal fluid lymphocytic pleocytosis and 3 cases had oligoclonal bands. Two subjects died during the acute phase, one in the chronic phase (probable sudden unexplained death in epilepsy), and one developed a persistent vegetative state. Among survivors, 80% developed drug-resistant epilepsy. Febrile illness-related SE associated with bilateral claustrum hyperintensity on MRI represents a condition with defined clinical features and a presumed but unidentified autoimmune etiology. A better characterization of de novo SE is mandatory for the search of specific etiologies.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

—

OECD FORD branch

30210 - Clinical neurology

Project

—

Continuities

V - Vyzkumna aktivita podporovana z jinych verejnych zdroju

Publication year

2017

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Frontiers in neurology

ISSN

1664-2295

e-ISSN

—

Volume of the periodical

8

Issue of the periodical within the volume

article 111

Country of publishing house

CH - SWITZERLAND

Number of pages

9

Pages from-to

1-9

UT code for WoS article

000397396700001

EID of the result in the Scopus database

2-s2.0-85018641904