Immunoglobulin-responsive refractory epilepsy – 3 cases with a similar EEG pattern

Dionisio, Sasha; Brown, Helen; Lander, Cecilie M.; Airey, Caroline; Lehn, Alexander; Nooruddin, Hozefa; Blum, Stefan

doi:10.1016/j.seizure.2013.02.013

Cited by 6 publications

(17 citation statements)

References 37 publications

(38 reference statements)

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“…All of the studies were retrospective in nature, with 8 case series 4,11,15,18,19,[21][22][23] and 13 case reports. [5][6][7][8][9][10][12][13][14]16,17,20,24 Across all studies, 87 pediatric patients were documented as having received IVIG for RSE (mean: 4.1 patients/study; range: 1-30 patients/study). The mean age of the patients was 7.8 years (median: 8.1; range: 2-17.5 years).…”

Section: Resultsmentioning

confidence: 99%

“…Seizures were classified as GRSE in 20 patients, 5,10,13,15,20,22 FRSE in 54 patients, 4,6,9,12,[14][15][16][17][18][19]21,22 NCRSE in 1 patient, 24 and RSE not specified in 12 patients. 7,8,11,15,23 The etiology of RSE was quite varied, and included feverinduced refractory epilepsy syndrome (FIRES) not otherwise specified in 56 patients, 4,5,11,14,15,22,23 acute encephalitis with refractory repetitive partial seizures in 17 patients, 12,18,19 N-methyl-D-aspartate encephalitis in 2 patients, 8,9 antigamma aminobutyric acid encephalitis in 1 patient, 16 antivoltage-gated potassium channel encephalitis in 1 patient, 20 antithyroperoxidase/antinuclear antibody (ANA) encephalitis in 1 patient, 6 ANA/antiribonuclear protein/Sjogren's syndrome antibody encephalitis in 1 patient, 7 ANA encephalitis in 1 patient, 17 antismooth muscle antibody (ASMA) encephalitis in 1 patient, 21 ANA/ASMA encephalitis in 1 patient, 21 and Lennox-Gestaut's syndrome in 1 patient. 24 Four patients had nonspecific meningoencephalitis or unknown syndromes.…”

Section: Resultsmentioning

confidence: 99%

“…Eleven of the 21 studies included in this review had descriptions of their IVIG regimen. 4,6,[9][10][11][12]15,17,18,20,21 The remaining studies failed to record IVIG dosing and frequency. The exact regimens recorded varied significantly between studies with dosing ranging from 0.4 mg/kg/d to 2 g/kg/d.…”

Section: Intravenous Immunoglobulin Treatment Characteristicsmentioning

confidence: 99%

“…Duration of treatment prior to the administration of IVIG was documented in six articles, 6,10,12,13,17,20 ranging from 1 to 30 days (mean ¼ 11.6 days; median ¼ 10). The remaining articles failed to mention the duration of therapy prior to IVIG.…”

Section: Intravenous Immunoglobulin Treatment Characteristicsmentioning

confidence: 99%

“…The remaining articles failed to mention the duration of therapy prior to IVIG. The number of AEDs administered prior to IVIG was documented in 8 studies, [4][5][6]9,10,18,20,22 with the total number ranging from 3 to 10 (mean ¼ 5.9, median ¼ 5). Finally, numerous other immunotherapies were administered in these patients and are detailed in ►Table 2.…”

Section: Intravenous Immunoglobulin Treatment Characteristicsmentioning

confidence: 99%

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Intravenous Immunoglobulins for Refractory Status Epilepticus: A Scoping Systematic Review of the Pediatric Literature

et al. 2017

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Our goal was to perform a scoping systematic review of the literature on the use of intravenous immunoglobulins (IVIGs) for refractory status epilepticus (RSE) in pediatric patients. Articles from MEDLINE, BIOSIS, EMBASE, Global Health, Healthstar, Scopus, Cochrane Library, the International Clinical Trials Registry Platform, ClinicalTrials.gov (inception to June 2016), reference lists of relevant articles, and gray literature were searched. The strength of evidence was adjudicated using both the Oxford and Grading of Recommendation Assessment Development and Education (GRADE) methodology by two independent reviewers. We identified 21 original articles. Eighty-seven pediatric patients were described as having received IVIG therapy for RSE. The mean age was 7.8 years (range: 2–17.5 years). Seizure response occurred in 14 of the 87 patients (16.1%), with 3 (3.4%) and 11 (12.6%) displaying partial and complete responses, respectively. Seventy-three of the 87 patients (83.9%) failed to display any seizure response to IVIG therapy. No complications related to IVIG therapy were recorded. The majority of patients had moderate to severe neurological deficits upon follow-up. Oxford level 4, GRADE D evidence exists to suggest little to no impact on seizure control in pediatric autoimmune RSE. Routine use of IVIG for pediatric RSE cannot be recommended at this time and should be considered experimental.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Intravenous Immunoglobulin Treatment Characteristicsmentioning

confidence: 99%

Section: Intravenous Immunoglobulin Treatment Characteristicsmentioning

confidence: 99%

Section: Intravenous Immunoglobulin Treatment Characteristicsmentioning

confidence: 99%

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Intravenous Immunoglobulins for Refractory Status Epilepticus: A Scoping Systematic Review of the Pediatric Literature

et al. 2017

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Therapeutic Options for Patients with Refractory Status Epilepticus in Palliative Settings or with a Limitation of Life-Sustaining Therapies: A Systematic Review

et al. 2020

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Background Refractory status epilepticus (RSE) represents a serious medical condition requiring early and targeted therapy. Given the increasing number of elderly or multimorbid patients with a limitation of life-sustaining therapy (LOT) or within a palliative care setting (PCS), guidelines-oriented therapy escalation options for RSE have to be omitted frequently. Objectives This systematic review sought to summarize the evidence for fourth-line antiseizure drugs (ASDs) and other minimally or non-invasive therapeutic options beyond guideline recommendations in patients with RSE to elaborate on possible treatment options for patients undergoing LOT or in a PCS. Methods A systematic review of the literature in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, focusing on fourth-line ASDs or other minimally or non-invasive therapeutic options was performed in February and June 2020 using the MEDLINE, EMBASE and Cochrane databases. The search terminology was constructed using the name of the specific ASD or therapy option and the term 'status epilepticus' with the use of Boolean operators, e.g. "(brivaracetam) AND (status epilepticus)". The respective Medical Subject Headings (MeSH) and Emtree terms were used, if available. ResultsThere is currently no level 1, grade A evidence for the use of ASDs in RSE. The best evidence was found for the use of lacosamide and topiramate (level 3, grade C), followed by brivaracetam, perampanel (each level 4, grade D) and stiripentol, oxcarbazepine and zonisamide (each level 5, grade D). Regarding non-medicinal options, there is little evidence for the use of the ketogenic diet (level 4, grade D) and magnesium sulfate (level 5, grade D) in RSE. The broad use of immunomodulatory or immunosuppressive treatment options in the absence of a presumed autoimmune etiology cannot be recommended; however, if an autoimmune etiology is assumed, steroid pulse, intravenous immunoglobulins and plasma exchange/plasmapheresis should be considered (level 4, grade D). Even if several studies suggested that the use of neurosteroids (level 5, grade D) is beneficial in RSE, the current data situation indicates that there is formal evidence against it. Conclusions RSE in patients undergoing LOT or in a PCS represents a challenge for modern clinicians and epileptologists. The evidence for the use of ASDs in RSE beyond that in current guidelines is low, but several effective and well-tolerated options are available that should be considered in this patient population. More so than in any other population, advance care planning, advance directives, and medical ethical aspects have to be considered carefully before and during therapy.

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Inmunoglobulina G intravenosa como tratamiento adyuvante en epilepsia infantil farmacorresistente

Gonzalez-Castillo

Gómez

et al. 2020

Neurología

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Immunoglobulin-responsive refractory epilepsy – 3 cases with a similar EEG pattern

Cited by 6 publications

References 37 publications

Intravenous Immunoglobulins for Refractory Status Epilepticus: A Scoping Systematic Review of the Pediatric Literature

Intravenous Immunoglobulins for Refractory Status Epilepticus: A Scoping Systematic Review of the Pediatric Literature

Therapeutic Options for Patients with Refractory Status Epilepticus in Palliative Settings or with a Limitation of Life-Sustaining Therapies: A Systematic Review

Inmunoglobulina G intravenosa como tratamiento adyuvante en epilepsia infantil farmacorresistente

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