New-onset refractory status epilepticus

Gaspard, Nicolas; Foreman, Brandon; Alvarez, Vincent; Kang, Christian Cabrera C.C.; Probasco, John C.; Jongeling, Amy C.; Meyers, Emma; Espinera, Alyssa R.; Haas, Kevin F.; Schmitt, Sarah; Gerard, Elizabeth E.; Gofton, Teneille; Kaplan, Peter W.; Lee, Jong W.; Legros, Benjamin; Szaflarski, Jerzy P.; Westover, Brandon; LaRoche, Suzette M.; Hirsch, Lawrence J.

doi:10.1212/wnl.0000000000001940

Cited by 380 publications

(358 citation statements)

References 31 publications

(43 reference statements)

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“…More remote seems the possibility of drug intoxication for the presented patients: no one had a history of drug abuse or psychiatric comorbidities; no one had possible exposure to possible environmental toxic substances. Given that, we agree with previous literature that as for the actual knowledge (and its limits) it is advisable to rapidly treat these patients with immune modulatory agents, beyond just the standard treatment of SE (7, 9–11). …”

Section: Discussionsupporting

confidence: 89%

“…New-onset RSE (NORSE) is a rare but challenging condition, characterized by the occurrence of a prolonged period of refractory seizures with no identifiable cause in otherwise healthy individuals (4–6). Gaspard et al (7) reported a large retrospective case-series of 130 NORSE cases evaluated between 2008 and 2013. In some patients, an autoimmune or paraneoplastic etiology was identified but more than half of the cases remained cryptogenic.…”

Section: Introductionmentioning

confidence: 99%

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New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features

et al. 2017

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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.

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Section: Discussionsupporting

confidence: 89%

Section: Introductionmentioning

confidence: 99%

New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features

et al. 2017

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“…[25*] Autoantibodies targeting this and other neuronal antigens have been infrequently identified in adults or children with chronic epilepsy (0–9%) or status epilepticus (< 3%), [26–31,32*,33] and were found at higher frequency (15–37%) when the seizures were refractory to antiepileptic treatment. [34,35] However, with the exception of glutamic acid decarboxylase 65(GAD65) antibodies, the significance of antibodies in chronic epilepsy is unclear because they are usually identified only in serum and may disappear over time despite seizure recurrence. [31] Careful consideration should be given to the interpretation of antibodies against proteins interacting with voltage-gated potassium channels (VGKC complex).…”

Section: Introductionmentioning

confidence: 99%

“…[140] A multicenter study on patients with NORSE for whom the underlying etiology could not be determined during the first 48 hours of presentation, found that in 40% the cause was autoimmune and in the other 60% the cause remained unknown. [34] In recent reports, the use of immunotherapy improved the outcome of 42–75% of patients with NORSE but approximately 30% developed chronic epilepsy. [141,142]…”

Section: Introductionmentioning

confidence: 99%

Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis

Spatola

Dalmau

2017

Current Opinion in Neurology

147

137

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Purpose of review To assess the seizure manifestations and risk of epilepsy in encephalitis associated to antibodies against neuronal cell-surface (AE) or myelin-associated antigens, and to review several chronic epileptic disorders including, Rasmussen’s encephalitis (RE), fever-induced refractory epileptic syndromes (FIRES), and new-onset refractory status epilepticus (NORSE). Recent findings Seizures are a frequent manifestation of AE. Some AE may associate with characteristic features: faciobrachial dystonic seizures (anti-LGI1 encephalitis), EEG extreme delta brush (anti-NMDAR), or multifocal FLAIR-MRI abnormalities (anti-GABAAR). In anti-LGI1 encephalitis, cortical, limbic, and basal ganglia dysfunction results in different types of seizures. AE or myelin-antibody associated syndromes are often immunotherapy-responsive and appear to have a low risk for chronic epilepsy. In contrast patients with seizures related to GAD65-antibodies (an intracellular antigen) frequently develop epilepsy and have suboptimal response to treatment (including surgery). RE or FIRES may occur with autoantibodies of unclear significance and rarely respond to immunotherapy. A study of patients with NORSE showed that 30% developed chronic epilepsy. Summary Although seizures are frequent in all types of AE, the risk for chronic epilepsy is dependent on the antigen: lower if located on the cell-surface, and higher if intracellular. For other disorders (RE, FIRES, NORSE) the prognosis remains poor.

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“…N ew-onset refractory status epilepticus (NORSE) is a clinical condition characterized by prolonged pharmacoresistant seizures in an individual without active epilepsy or other pre-existing relevant neurologic disorders. 1,2 Other terms such as acute encephalitis with refractory, repetitive partial seizures; devastating epileptic encephalopathy in school-aged children; and febrile infection-related epilepsy syndrome describe the same disease entity. Although the absence of a clear acute or active toxic, metabolic, or structural cause is necessary for the diagnosis, the neuropathology of NORSE includes patients with viral infections (such as herpes simplex virus 1) and autoimmune syndromes such as autoimmune limbic encephalitis (ALE).…”

mentioning

confidence: 99%

FDG-PET and MRI in the Evolution of New-Onset Refractory Status Epilepticus

Strohm

Steriade²,

et al. 2019

AJNR Am J Neuroradiol

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BACKGROUND AND PURPOSE: New-onset refractory status epilepticus is a clinical condition characterized by acute and prolonged pharmacoresistant seizures without a pre-existing relevant neurologic disorder, prior epilepsy, or clear structural, toxic, or metabolic cause. New-onset refractory status epilepticus is often associated with antineuronal antibodies and may respond to early immunosuppressive therapy, reflecting an inflammatory element of the condition. FDG-PET is a useful diagnostic tool in inflammatory and noninflammatory encephalitis. We report here FDG-PET findings in new-onset refractory status epilepticus and their correlation to disease activity, other imaging findings, and outcomes. MATERIALS AND METHODS:Twelve patients who met the criteria for new-onset refractory status epilepticus and who had FDG-PET and MR imaging scans and electroencephalography at a single academic medical center between 2008 and 2017 were retrospectively identified. Images were independently reviewed by 2 radiologists specialized in nuclear imaging. Clinical characteristics and outcome measures were collected through chart review. RESULTS:Twelve patients underwent 21 FDG-PET scans and 50 MR imaging scans. Nine (75%) patients were positive for autoantibodies. All patients had identifiable abnormalities on the initial FDG-PET in the form of hypermetabolism (83%) and/or hypometabolism (42%). Eight (67%) had medial temporal involvement. All patients (n ϭ 3) with N-methyl-D-aspartic acid receptor antibodies had profound bilateral occipital hypometabolism. Initial MR imaging findings were normal in 6 (50%) patients. Most patients had some degree of persistent hyper-(73%) or hypometabolism (45%) after immunosuppressive therapy. FDG-PET hypometabolism was predictive of poor outcome (mRS 4 -6) at hospital discharge (P ϭ .028). CONCLUSIONS:Both FDG-PET hypometabolism and hypermetabolism are seen in the setting of new-onset refractory status epilepticus and may represent markers of disease activity. ABBREVIATIONS: ALE ϭ autoimmune limbic encephalitis; EEG ϭ electroencephalography; GABA-B ϭ ␥-aminobutyric acid B; LGI1 ϭ leucine-rich glioma inactivated 1; NMDA ϭ N-methyl-D-aspartate; NMDA-R ϭ N-methyl-D-aspartate receptor; NORSE ϭ new-onset refractory status epilepticus; VGKC ϭ voltage-gated potassium channel

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New-onset refractory status epilepticus

Cited by 380 publications

References 31 publications

New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features

New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features

Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis

FDG-PET and MRI in the Evolution of New-Onset Refractory Status Epilepticus

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