Evidence map›Paper›PMID 29272336›Full record

ArticleBrain : a journal of neurology2018

The importance of early immunotherapy in patients with faciobrachial dystonic seizures.

Julia Thompson, Mian Bi, Andrew G Murchison, Mateusz Makuch, Christian G Bien, Kon Chu, Pue Farooque, Jeffrey M Gelfand, Michael D Geschwind, Lawrence J Hirsch and 7 more

Open access · hybridAbstract readVideo-Audio Media
PubMed Full text at PMC Publisher
In one paragraph

Article in Brain : a journal of neurology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 202 papers, 7 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
202citing papers in PubMed, 7 pooled it
19.7field-weighted citation impact, top 1% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

202 citing papers in PubMed, 7 syntheses or guidelines pooled it, 390 citations in OpenAlex.

  1. Evaluation of the clinical assessment scale for autoimmune encephalitis (CASE) in a retrospective cohort and a systematic review.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2024
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  8. The safety and efficacy of intravenous immunoglobulin in autoimmune encephalitis.Annals of clinical and translational neurology · 2022
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  12. Suspected encephalitis in adults.Practical neurology · 2026
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142 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

17 authors at 7 institutions in 5 countries.

Julia ThompsonOxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, OX3 9DS, UK.
Mian BiDementia Research Unit, University of New South Wales, Kensington, Sydney, New South Wales, NSW 2052, Australia.
Andrew G MurchisonOxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, OX3 9DS, UK.
Mateusz MakuchOxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, OX3 9DS, UK.
Christian G BienKrankenhaus Mara, Epilepsy Center Bethel, Bielefeld D-33617, Germany.
Kon ChuDepartment of Neurology, Comprehensive Epilepsy Center, Biomedical Research Institute, Seoul National University Hospital, Seoul, 110-744, South Korea.
Pue FarooqueComprehensive Epilepsy Center, Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA.
Jeffrey M GelfandUCSF Department of Neurology, 675 Nelson Rising Lane, San Francisco, CA 94143, USA.
Michael D GeschwindUCSF Department of Neurology, 675 Nelson Rising Lane, San Francisco, CA 94143, USA.
Lawrence J HirschComprehensive Epilepsy Center, Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA.
Ernest SomervilleComprehensive Epilepsy Service, Prince of Wales Clinical School, University of New South Wales, Sydney, New South Wales, NSW 2052, Australia.
Bethan LangOxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, OX3 9DS, UK.
Angela VincentOxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, OX3 9DS, UK.
Maria I LeiteOxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, OX3 9DS, UK.
Patrick WatersOxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, OX3 9DS, UK.
Sarosh R IraniOxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, OX3 9DS, UK.
Faciobrachial Dystonic Seizures Study Group
University of Oxford · GBUniversity of California, San Francisco · USUNSW Sydney · AUYale University · USEvangelisches Krankenhaus Bielefeld · DEJohn Radcliffe Hospital · GBSeoul National University Hospital · KR

Funding

Yale Clinical and Translational Science Award (U Component)UL1TR001863 · NCATS · YALE UNIVERSITY · PI John H. Krystal, LUCILA OHNO-MACHADO · 2016 to 2026
$102.9M
Predicting progression of human prion diseaseR01AG031189 · NIA · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI GESCHWIND, MICHAEL D · 2008 to 2017
$5.7M
NCATS NIH HHS UL1 TR001863NIA NIH HHS L30 AG024892NIA NIH HHS R01 AG031189Wellcome Trust 104079/Z/14/Z
6 · The paper itself

Abstract

Faciobrachial dystonic seizures and limbic encephalitis closely associate with antibodies to leucine-rich glioma-inactivated 1 (LGI1). Here, we describe 103 consecutive patients with faciobrachial dystonic seizures and LGI1 antibodies to understand clinical, therapeutic and serological differences between those with and without cognitive impairment, and to determine whether cessation of faciobrachial dystonic seizures can prevent cognitive impairment. The 22/103 patients without cognitive impairment typically had normal brain MRI, EEGs and serum sodium levels (P < 0.0001). Overall, cessation of faciobrachial dystonic seizures with antiepileptic drugs alone occurred in only 9/89 (10%) patients. By contrast, 51% showed cessation of faciobrachial dystonic seizures 30 days after addition of immunotherapy (P < 0.0001), with earlier cessation in cognitively normal patients (P = 0.038). Indeed, expedited immunotherapy (P = 0.031) and normal cognition (P = 0.0014) also predicted reduced disability at 24 months. Furthermore, of 80 patients with faciobrachial dystonic seizures as their initial feature, 56% developed cognitive impairment after 90 days of active faciobrachial dystonic seizures. Whereas only one patient developed cognitive impairment after cessation of faciobrachial dystonic seizures (P < 0.0001). All patients had IgG4-LGI1 antibodies, but those with cognitive impairment had higher proportions of complement-fixing IgG1 antibodies (P = 0.03). Both subclasses caused LGI1-ADAM22 complex internalization, a potential non-inflammatory epileptogenic mechanism. In summary, faciobrachial dystonic seizures show striking time-sensitive responses to immunotherapy, and their cessation can prevent the development of cognitive impairment.awx323media15681705685001.

Indexed as

ADAM ProteinsAdultAgedAged, 80 and overAnalysis of VarianceAntibodiesAnticonvulsantsCognition DisordersFemaleFlow CytometryFollow-Up StudiesGreen Fluorescent ProteinsHEK293 CellsHumansImmunotherapyIntracellular Signaling Peptides and ProteinsADAM22 protein, humanADAM ProteinsAntibodiesAnticonvulsantsenhanced green fluorescent proteinGreen Fluorescent ProteinsIntracellular Signaling Peptides and ProteinsLGI1 protein, humanNerve Tissue ProteinsProteinscognitive impairmentfaciobrachial dystonic seizuresimmunotherapyleucine-rich glioma-inactivated 1seizures

Identifiers

PMID29272336
PMCPMC5837230
OpenAlexW2783144549

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.