ArticleNeurology(R) neuroimmunology & neuroinflammation2024
Progressive Encephalomyelitis With Rigidity and Myoclonus With Glycine Receptor and GAD65 Antibodies: Case Report and Potential Mechanisms.
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Article in Neurology(R) neuroimmunology & neuroinflammation, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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8 citing papers in PubMed.
- Stiff Person Syndrome: Bridging Neuroimmunology and Rehabilitation for Improved Patient Outcomes.Archives of internal medicine research · 2026Article
- Acute onset of progressive encephalomyelitis with rigidity and myoclonus complicated by paroxysmal respiratory failure: a case report.BMC neurology · 2026Article
- Individualized repetitive intravenous immunoglobulin therapy for a 79-year-old patient with GlyR antibody-positive PERM: a case report and literature review.Frontiers in immunology · 2026Review
- Neuroimmune mechanisms of the cerebellum-brainstem network in autoimmune encephalitis and related neuroimmune disorders: antibody targets, selective vulnerability, and translational opportunities.Frontiers in immunology · 2026Review
- Neuronal pSTAT1 hallmarks synaptic pathology in autoimmune encephalitis against intracellular antigens.Acta neuropathologica · 2025Article
- Chimeric antigen receptor T-cell therapy for stiff-person syndrome: bridging innovation and clinical challenges in neuroimmunology.Therapeutic advances in neurological disorders · 2025Review
- Clinical features of progressive encephalomyelitis with rigidity and myoclonus: Case Report.Frontiers in immunology · 2025Article
- Stiff-person spectrum disorder induced by shingles: a case report.BMJ neurology open · 2025Article
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Authors and funding
12 authors.
Michael WinklehnerFrom the Division of Neuropathology and Neurochemistry (M.W., E.G., R.H.), Department of Neurology, Medical University of Vienna; Department of Neurology (M.W.), Johannes Kepler University Linz, Kepler University Hospital, Linz, Austria; Section of Translational Neuroimmunology (J.W., D.B., A.G., C.G.), Department of Neurology, Jena University Hospital, Germany; Comprehensive Center for Clinical Neurosciences and Mental Health (E.G., R.H.), Medical University of Vienna, Austria; Institute of Clinical Neurobiology (V.R., C.V.), University Hospital, Julius-Maximilians-University of Würzburg, Germany; Department of Neuroimmunology (J.B.), Center for Brain Research; and Center for Medical Physics and Biomedical Engineering (A.S.S., P.J.), Medical University of Vienna, Austria.
Jonathan WickelFrom the Division of Neuropathology and Neurochemistry (M.W., E.G., R.H.), Department of Neurology, Medical University of Vienna; Department of Neurology (M.W.), Johannes Kepler University Linz, Kepler University Hospital, Linz, Austria; Section of Translational Neuroimmunology (J.W., D.B., A.G., C.G.), Department of Neurology, Jena University Hospital, Germany; Comprehensive Center for Clinical Neurosciences and Mental Health (E.G., R.H.), Medical University of Vienna, Austria; Institute of Clinical Neurobiology (V.R., C.V.), University Hospital, Julius-Maximilians-University of Würzburg, Germany; Department of Neuroimmunology (J.B.), Center for Brain Research; and Center for Medical Physics and Biomedical Engineering (A.S.S., P.J.), Medical University of Vienna, Austria.ORCID 0000-0002-5854-3759
Ellen GelpiFrom the Division of Neuropathology and Neurochemistry (M.W., E.G., R.H.), Department of Neurology, Medical University of Vienna; Department of Neurology (M.W.), Johannes Kepler University Linz, Kepler University Hospital, Linz, Austria; Section of Translational Neuroimmunology (J.W., D.B., A.G., C.G.), Department of Neurology, Jena University Hospital, Germany; Comprehensive Center for Clinical Neurosciences and Mental Health (E.G., R.H.), Medical University of Vienna, Austria; Institute of Clinical Neurobiology (V.R., C.V.), University Hospital, Julius-Maximilians-University of Würzburg, Germany; Department of Neuroimmunology (J.B.), Center for Brain Research; and Center for Medical Physics and Biomedical Engineering (A.S.S., P.J.), Medical University of Vienna, Austria.
Dirk BrämerFrom the Division of Neuropathology and Neurochemistry (M.W., E.G., R.H.), Department of Neurology, Medical University of Vienna; Department of Neurology (M.W.), Johannes Kepler University Linz, Kepler University Hospital, Linz, Austria; Section of Translational Neuroimmunology (J.W., D.B., A.G., C.G.), Department of Neurology, Jena University Hospital, Germany; Comprehensive Center for Clinical Neurosciences and Mental Health (E.G., R.H.), Medical University of Vienna, Austria; Institute of Clinical Neurobiology (V.R., C.V.), University Hospital, Julius-Maximilians-University of Würzburg, Germany; Department of Neuroimmunology (J.B.), Center for Brain Research; and Center for Medical Physics and Biomedical Engineering (A.S.S., P.J.), Medical University of Vienna, Austria.ORCID 0009-0008-3633-6047
Vera RauschenbergerFrom the Division of Neuropathology and Neurochemistry (M.W., E.G., R.H.), Department of Neurology, Medical University of Vienna; Department of Neurology (M.W.), Johannes Kepler University Linz, Kepler University Hospital, Linz, Austria; Section of Translational Neuroimmunology (J.W., D.B., A.G., C.G.), Department of Neurology, Jena University Hospital, Germany; Comprehensive Center for Clinical Neurosciences and Mental Health (E.G., R.H.), Medical University of Vienna, Austria; Institute of Clinical Neurobiology (V.R., C.V.), University Hospital, Julius-Maximilians-University of Würzburg, Germany; Department of Neuroimmunology (J.B.), Center for Brain Research; and Center for Medical Physics and Biomedical Engineering (A.S.S., P.J.), Medical University of Vienna, Austria.
Albrecht GüntherFrom the Division of Neuropathology and Neurochemistry (M.W., E.G., R.H.), Department of Neurology, Medical University of Vienna; Department of Neurology (M.W.), Johannes Kepler University Linz, Kepler University Hospital, Linz, Austria; Section of Translational Neuroimmunology (J.W., D.B., A.G., C.G.), Department of Neurology, Jena University Hospital, Germany; Comprehensive Center for Clinical Neurosciences and Mental Health (E.G., R.H.), Medical University of Vienna, Austria; Institute of Clinical Neurobiology (V.R., C.V.), University Hospital, Julius-Maximilians-University of Würzburg, Germany; Department of Neuroimmunology (J.B.), Center for Brain Research; and Center for Medical Physics and Biomedical Engineering (A.S.S., P.J.), Medical University of Vienna, Austria.
Jan BauerFrom the Division of Neuropathology and Neurochemistry (M.W., E.G., R.H.), Department of Neurology, Medical University of Vienna; Department of Neurology (M.W.), Johannes Kepler University Linz, Kepler University Hospital, Linz, Austria; Section of Translational Neuroimmunology (J.W., D.B., A.G., C.G.), Department of Neurology, Jena University Hospital, Germany; Comprehensive Center for Clinical Neurosciences and Mental Health (E.G., R.H.), Medical University of Vienna, Austria; Institute of Clinical Neurobiology (V.R., C.V.), University Hospital, Julius-Maximilians-University of Würzburg, Germany; Department of Neuroimmunology (J.B.), Center for Brain Research; and Center for Medical Physics and Biomedical Engineering (A.S.S., P.J.), Medical University of Vienna, Austria.
Anika Simonovska SerraFrom the Division of Neuropathology and Neurochemistry (M.W., E.G., R.H.), Department of Neurology, Medical University of Vienna; Department of Neurology (M.W.), Johannes Kepler University Linz, Kepler University Hospital, Linz, Austria; Section of Translational Neuroimmunology (J.W., D.B., A.G., C.G.), Department of Neurology, Jena University Hospital, Germany; Comprehensive Center for Clinical Neurosciences and Mental Health (E.G., R.H.), Medical University of Vienna, Austria; Institute of Clinical Neurobiology (V.R., C.V.), University Hospital, Julius-Maximilians-University of Würzburg, Germany; Department of Neuroimmunology (J.B.), Center for Brain Research; and Center for Medical Physics and Biomedical Engineering (A.S.S., P.J.), Medical University of Vienna, Austria.
Philipp JaukFrom the Division of Neuropathology and Neurochemistry (M.W., E.G., R.H.), Department of Neurology, Medical University of Vienna; Department of Neurology (M.W.), Johannes Kepler University Linz, Kepler University Hospital, Linz, Austria; Section of Translational Neuroimmunology (J.W., D.B., A.G., C.G.), Department of Neurology, Jena University Hospital, Germany; Comprehensive Center for Clinical Neurosciences and Mental Health (E.G., R.H.), Medical University of Vienna, Austria; Institute of Clinical Neurobiology (V.R., C.V.), University Hospital, Julius-Maximilians-University of Würzburg, Germany; Department of Neuroimmunology (J.B.), Center for Brain Research; and Center for Medical Physics and Biomedical Engineering (A.S.S., P.J.), Medical University of Vienna, Austria.
Carmen VillmannFrom the Division of Neuropathology and Neurochemistry (M.W., E.G., R.H.), Department of Neurology, Medical University of Vienna; Department of Neurology (M.W.), Johannes Kepler University Linz, Kepler University Hospital, Linz, Austria; Section of Translational Neuroimmunology (J.W., D.B., A.G., C.G.), Department of Neurology, Jena University Hospital, Germany; Comprehensive Center for Clinical Neurosciences and Mental Health (E.G., R.H.), Medical University of Vienna, Austria; Institute of Clinical Neurobiology (V.R., C.V.), University Hospital, Julius-Maximilians-University of Würzburg, Germany; Department of Neuroimmunology (J.B.), Center for Brain Research; and Center for Medical Physics and Biomedical Engineering (A.S.S., P.J.), Medical University of Vienna, Austria.ORCID 0000-0003-1498-6950
Romana HöftbergerFrom the Division of Neuropathology and Neurochemistry (M.W., E.G., R.H.), Department of Neurology, Medical University of Vienna; Department of Neurology (M.W.), Johannes Kepler University Linz, Kepler University Hospital, Linz, Austria; Section of Translational Neuroimmunology (J.W., D.B., A.G., C.G.), Department of Neurology, Jena University Hospital, Germany; Comprehensive Center for Clinical Neurosciences and Mental Health (E.G., R.H.), Medical University of Vienna, Austria; Institute of Clinical Neurobiology (V.R., C.V.), University Hospital, Julius-Maximilians-University of Würzburg, Germany; Department of Neuroimmunology (J.B.), Center for Brain Research; and Center for Medical Physics and Biomedical Engineering (A.S.S., P.J.), Medical University of Vienna, Austria.ORCID 0000-0002-5769-1100
Christian GeisFrom the Division of Neuropathology and Neurochemistry (M.W., E.G., R.H.), Department of Neurology, Medical University of Vienna; Department of Neurology (M.W.), Johannes Kepler University Linz, Kepler University Hospital, Linz, Austria; Section of Translational Neuroimmunology (J.W., D.B., A.G., C.G.), Department of Neurology, Jena University Hospital, Germany; Comprehensive Center for Clinical Neurosciences and Mental Health (E.G., R.H.), Medical University of Vienna, Austria; Institute of Clinical Neurobiology (V.R., C.V.), University Hospital, Julius-Maximilians-University of Würzburg, Germany; Department of Neuroimmunology (J.B.), Center for Brain Research; and Center for Medical Physics and Biomedical Engineering (A.S.S., P.J.), Medical University of Vienna, Austria.ORCID 0000-0002-9859-581X
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesProgressive encephalomyelitis with rigidity and myoclonus (PERM) is a severe form of stiff-person spectrum disorder that can be associated with antibodies against surface antigens (glycine receptor (GlyR), dipeptidyl-peptidase-like-protein-6) and intracellular antigens (glutamate decarboxylase (GAD65), amphiphysin).
methodsWe report clinico-pathologic findings of a PERM patient with coexisting GlyR and GAD65 antibodies.
resultsA 75-year-old man presented with myoclonus and pain of the legs, subsequently developed severe motor symptoms, hyperekplexia, a pronounced startle reflex, hallucinations, dysautonomia, and died 10 months after onset despite extensive immunotherapy, symptomatic treatment, and continuous intensive care support. Immunotherapy comprised corticosteroids, IVIG, plasmapheresis, immunoadsorption, cyclophosphamide, and bortezomib. Intensive care treatment and permanent isoflurane sedation was required for more than 20 weeks. CNS tissue revealed neuronal loss, astrogliosis and microgliosis, representing a pallido-nigro-dentato-bulbar-spinal degeneration pattern, specifically along GlyR and GAD expression sites. Neurons showed pSTAT1, MHC class I, and GRP78 upregulation. Inflammation was moderate and characterized by CD8 DISCUSSION: A possible potentiating effect of pathogenic GlyR antibodies together with T cells directed against neurons may have led to the severe and progressive clinical course.
Indexed as
AutoantibodiesEncephalomyelitisGlutamate DecarboxylaseMuscle RigidityMyoclonusReceptors, GlycineAgedFatal OutcomeHumansMaleStiff-Person SyndromeAutoantibodiesGlutamate Decarboxylaseglutamate decarboxylase 2Receptors, Glycine
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