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Autoimmune encephalitis With mGluR1 antibodies presenting with Epilepsy, but without cerebellar signs

dc.contributor.authorVinke, Anita M.
dc.contributor.authorZong, Shenghua
dc.contributor.authorJanssen, Josien H.
dc.contributor.authorHoffmann, Carolin Correia
dc.contributor.authorMané-Damas, Marina
dc.contributor.authorDamoiseaux, Jan G.M.C.
dc.contributor.authorde Vries, J.M.
dc.contributor.authorPröpper, Dirk
dc.contributor.authorMolenaar, Peter
dc.contributor.authorLosen, Mario
dc.contributor.authorMartinez Martinez, Pilar
dc.contributor.authorRouhl, Rob P.W.
dc.date.accessioned2022-12-06T10:12:38Z
dc.date.available2022-12-06T10:12:38Z
dc.date.issued2022
dc.description.abstractObjective To describe the unique case history of a patient with mGluR1 antibodies, with mainly limbic and without cerebellar symptoms. Methods A 50-year-old woman initially presented with focal seizures with epigastric rising and deja-vu sensations, next to cognitive complaints, and musical auditory hallucinations. MRI, EEG, and neuronal autoantibody tests were performed. Results EEG findings showed slow and sharp activity (sharp waves and sharp-wave-slow-wave complex) in the left temporal lobe. A test for autoantibodies was negative initially. Because of persistent symptoms, serum and CSF were tested 4 years later and found positive for mGluR1 antibodies. Treatment started with monthly IV immunoglobulins and azathioprine that was replaced by mycophenolate mofetil later. Especially cognitive symptoms and hallucinations did not respond well to the treatment. During treatment, mGluR1 antibodies remained present in CSF. Discussion Whereas cerebellar symptoms are present in 97% of mGluR1-positive cases, our patient presented without ataxia. Therefore, we suggest that the clinical presentation of patients with mGluR1 antibodies is probably more diverse than previously described. Testing for mGluR1 antibodies should be considered in patients with limbic encephalitis and epilepsy, especially when negative for more common antibodies.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.doi10.1212/NXI.0000000000001171pt_PT
dc.identifier.issn2332-7812
dc.identifier.urihttp://hdl.handle.net/10400.1/18579
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherLippincott, Williams & Wilkinspt_PT
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/pt_PT
dc.subjectNMDA-Receptor Encephalitispt_PT
dc.subjectPrognostic-Factorspt_PT
dc.titleAutoimmune encephalitis With mGluR1 antibodies presenting with Epilepsy, but without cerebellar signspt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.issue4pt_PT
oaire.citation.startPagee1171pt_PT
oaire.citation.titleNeurology - Neuroimmunology Neuroinflammationpt_PT
oaire.citation.volume9pt_PT
person.familyNameHoffmann
person.givenNameCarolin
person.identifier.ciencia-id211F-792C-1B73
person.identifier.orcid0000-0002-2528-7541
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT
relation.isAuthorOfPublication50a4d3cc-b575-4d58-8df8-a51bed2914bb
relation.isAuthorOfPublication.latestForDiscovery50a4d3cc-b575-4d58-8df8-a51bed2914bb

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