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July 2020; 7 (4) Clinical/Scientific NotesOpen Access

Antibodies to nodal/paranodal proteins in paediatric immune-mediated neuropathy

Desiree De Simoni, Gerda Ricken, Michael Winklehner, Inga Koneczny, Michael Karenfort, Ulf Hustedt, Ulrich Seidel, View ORCID ProfileOmar Abdel-Mannan, Pinki Munot, Simon Rinaldi, Claudia Steen, Michael Freilinger, View ORCID ProfileMarkus Breu, Rainer Seidl, View ORCID ProfileMarkus Reindl, Julia Wanschitz, Cinta Lleixà, Günther Bernert, Klaus-Peter Wandinger, Ralf Junker, View ORCID ProfileLuis Querol, Frank Leypoldt, Kevin Rostásy, Romana Höftberger
First published June 2, 2020, DOI: https://doi.org/10.1212/NXI.0000000000000763
Desiree De Simoni
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Gerda Ricken
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Michael Winklehner
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Inga Koneczny
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Michael Karenfort
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Ulf Hustedt
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Ulrich Seidel
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Omar Abdel-Mannan
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Pinki Munot
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Simon Rinaldi
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Claudia Steen
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Michael Freilinger
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Markus Breu
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Rainer Seidl
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Markus Reindl
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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  • ORCID record for Markus Reindl
Julia Wanschitz
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Cinta Lleixà
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Günther Bernert
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Klaus-Peter Wandinger
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Ralf Junker
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Luis Querol
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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  • ORCID record for Luis Querol
Frank Leypoldt
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Kevin Rostásy
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Romana Höftberger
From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
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Full PDF
Citation
Antibodies to nodal/paranodal proteins in paediatric immune-mediated neuropathy
Desiree De Simoni, Gerda Ricken, Michael Winklehner, Inga Koneczny, Michael Karenfort, Ulf Hustedt, Ulrich Seidel, Omar Abdel-Mannan, Pinki Munot, Simon Rinaldi, Claudia Steen, Michael Freilinger, Markus Breu, Rainer Seidl, Markus Reindl, Julia Wanschitz, Cinta Lleixà, Günther Bernert, Klaus-Peter Wandinger, Ralf Junker, Luis Querol, Frank Leypoldt, Kevin Rostásy, Romana Höftberger
Neurol Neuroimmunol Neuroinflamm Jul 2020, 7 (4) e763; DOI: 10.1212/NXI.0000000000000763

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  1. Desiree De Simoni, MD,
  2. Gerda Ricken, MSc,
  3. Michael Winklehner, MD,
  4. Inga Koneczny, PhD,
  5. Michael Karenfort, MD,
  6. Ulf Hustedt, MD,
  7. Ulrich Seidel, MD,
  8. Omar Abdel-Mannan, MD,
  9. Pinki Munot, MD,
  10. Simon Rinaldi, MD,
  11. Claudia Steen, MD,
  12. Michael Freilinger, MD,
  13. Markus Breu, MD,
  14. Rainer Seidl, MD,
  15. Markus Reindl, PhD,
  16. Julia Wanschitz, MD,
  17. Cinta Lleixà, PhD,
  18. Günther Bernert, MD,
  19. Klaus-Peter Wandinger, MD, PhD,
  20. Ralf Junker, MD,
  21. Luis Querol, MD, PhD,
  22. Frank Leypoldt, MD, PhD,
  23. Kevin Rostásy, MD* and
  24. Romana Höftberger, MD*
  1. From the Division of Neuropathology and Neurochemistry (D.D.S., G.R., M.W., I.K., R.H.), Department of Neurology, Medical University of Vienna, Austria; Department of Neurology (D.D.S.), University Hospital St. Poelten, Austria; Department of General Pediatrics, Neonatology and Pediatric Cardiology (M.K.), University Children's Hospital, Heinrich Heine University Duesseldorf, Germany; Department of Neuropediatric Rehabilitation (U.H.), Vamed Clinic Hattingen, Germany; Department of Neuropediatrics (U.S.), Charité University, Berlin, Germany; Paediatric Neurology (O.A.-M.), Great Ormond Street Hospital for Children, London, United Kingdom; Dubowitz Neuromuscular Centre (P.M.), Great Ormond Street Hospital for Children, London, United Kingdom; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust; Department of Paediatric and Adolescent Medicine (C.S.), St Joseph Hospital, Berlin, Germany; Department of Pediatrics and Adolescent Medicine (M.F., M.B., R.S.), Medical University of Vienna, Austria; Department of Neurology (M.R., J.W.), Medical University of Innsbruck, Austria; Neuromuscular Diseases Unit (C.L., L.Q.), Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Spain; SMZ Süd (G.B.), Kaiser-Franz Josef Hospital with Gottfried von Preyer Children Hospital, Vienna, Austria; Institute of Clinical Chemistry (K.-P.W., R.J., F.L.), University Hospital Schleswig-Holstein, Kiel/Lübeck, Germany; Department of Neurology (F.L.), University Hospital Schleswig-Holstein, Kiel, Germany; and Department of Pediatric Neurology (K.R.), Witten/Herdecke University, Children's Hospital Datteln, Germany.
  1. Correspondence
    Dr. Rostásy k.rostasy{at}kinderklinik-datteln.de or Dr. Höftberger romana.hoeftberger{at}meduniwien.ac.at
Scientific Advisory Boards:
  1. NONE

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  1. NONE

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  1. NONE

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  1. NONE

Employment, Commercial Entity:
  1. NONE

Consultancies:
  1. NONE

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  1. NONE

Other Activities:
  1. NONE

Clinical Procedures or Imaging Studies:
  1. NONE

Research Support, Commercial Entities:
  1. NONE

Research Support, Government Entities:
  1. NONE

Research Support, Academic Entities:
  1. NONE

Research Support, Foundations and Societies:
  1. NONE

Stock/Stock Options/Board of Directors Compensation:
  1. NONE

License Fee Payments, Technology or Inventions:
  1. NONE

Royalty Payments, Technology or Inventions:
  1. NONE

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  1. NONE

Stock/Stock Options, Medical Equipment & Materials:
  1. NONE

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  1. NONE

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  1. NONE

Gifts:
  1. NONE

Funding for Travel or Speaker Honoraria:
  1. NONE

Editorial Boards:
  1. NONE

Patents:
  1. NONE

Publishing Royalties:
  1. NONE

Employment, Commercial Entity:
  1. NONE

Consultancies:
  1. NONE

Speakers' Bureaus:
  1. NONE

Other Activities:
  1. NONE

Clinical Procedures or Imaging Studies:
  1. NONE

Research Support, Commercial Entities:
  1. NONE

Research Support, Government Entities:
  1. NONE

Research Support, Academic Entities:
  1. NONE

Research Support, Foundations and Societies:
  1. NONE

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  1. NONE

License Fee Payments, Technology or Inventions:
  1. NONE

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  1. NONE

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  1. NONE

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  1. NONE

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  1. NONE

Gifts:
  1. NONE

Funding for Travel or Speaker Honoraria:
  1. NONE

Editorial Boards:
  1. NONE

Patents:
  1. NONE

Publishing Royalties:
  1. NONE

Employment, Commercial Entity:
  1. NONE

Consultancies:
  1. NONE

Speakers' Bureaus:
  1. NONE

Other Activities:
  1. NONE

Clinical Procedures or Imaging Studies:
  1. NONE

Research Support, Commercial Entities:
  1. NONE

Research Support, Government Entities:
  1. NONE

Research Support, Academic Entities:
  1. NONE

Research Support, Foundations and Societies:
  1. NONE

Stock/Stock Options/Board of Directors Compensation:
  1. NONE

License Fee Payments, Technology or Inventions:
  1. NONE

Royalty Payments, Technology or Inventions:
  1. NONE

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  1. NONE

Stock/Stock Options, Medical Equipment & Materials:
  1. NONE

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  1. NONE

Funding for Travel or Speaker Honoraria:
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  1. NONE

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  1. NONE

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  1. NONE

Employment, Commercial Entity:
  1. NONE

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  1. NONE

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  1. NONE

Other Activities:
  1. NONE

Clinical Procedures or Imaging Studies:
  1. NONE

Research Support, Commercial Entities:
  1. NONE

Research Support, Government Entities:
  1. I am funded by a Hertha Firnberg project grant by the Austrian Science Find (FWF). Project Grant Nr. T996-B30

Research Support, Academic Entities:
  1. NONE

Research Support, Foundations and Societies:
  1. I am funded by a Hertha Firnberg project grant by the Austrian Science Find (FWF). Project Grant Nr. T996-B30

Stock/Stock Options/Board of Directors Compensation:
  1. NONE

License Fee Payments, Technology or Inventions:
  1. NONE

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  1. NONE

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  1. NONE

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  1. NONE

Funding for travel or speaker honoraria:
  1. Speaker Honorar from Novartis Pharma

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  1. NONE

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  1. NONE

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  1. NONE

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  1. NONE

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  1. NONE

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  1. NONE

Other Activities:
  1. NONE

Clinical Procedures or Imaging Studies:
  1. NONE

Research Support, Commercial Entities:
  1. NONE

Research Support, Government Entities:
  1. NONE

Research Support, Academic Entities:
  1. NONE

Research Support, Foundations and Societies:
  1. NONE

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  1. NONE

License Fee Payments, Technology or Inventions:
  1. NONE

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  1. NONE

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  1. NONE

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  1. (1) Allergan an IPSEN

Gifts:
  1. NONE

Funding for travel or speaker honoraria:
  1. Travel and speaker honoraria from Allergan, Ipsen, Braun and UCB pharma

Editorial Boards:
  1. Pädiatrische Praxis, 2020, Issue 6 in press

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Employment, Commercial Entity:
  1. CEO, med+ED, Agency for Medical Education 2005 until now

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Other Activities:
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Clinical Procedures or Imaging Studies:
  1. NONE

Research Support, Commercial Entities:
  1. Technical grant from EMS for shock wave therapy in childhood

Research Support, Government Entities:
  1. NONE

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  1. NONE

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  1. NONE

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  1. NONE

License Fee Payments, Technology or Inventions:
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  1. NONE

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  1. NONE

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  1. NONE

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  1. NONE

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  1. NONE

Other Activities:
  1. NONE

Clinical Procedures or Imaging Studies:
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  1. NONE

Research Support, Academic Entities:
  1. NONE

Research Support, Foundations and Societies:
  1. NONE

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  1. NONE

License Fee Payments, Technology or Inventions:
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Funding for Travel or Speaker Honoraria:
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  1. NONE

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  1. NONE

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  1. NONE

Consultancies:
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  1. 1. GAIN patient charity medical advisory board (unpaid) 2. ABN research council member (unpaid) 3. Inflammatory Neuropathy Consortium board member (unpaid)

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  1. (1) Austrian Science Funds (FWF), project P32699, PI, 2019-2022 (2) Austrian Federal Ministry of Science, Research and Education, grant BIG WIG MS, PI, 2013-2017 (3) Austrian Research promotion Agency (FFG), Bridge 1 project Nr. 853209, PI, 2016-2018 (4) Austrian Science Funds (FWF), project W1206, PI, 2007-2018 (5), Austrian Science Funds (FWF), project ZFP326990, PI, 2019-2022

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  1. NONE

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  1. NONE

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  1. Commercial: CSL Behring, Novartis and Biogen, travel grant

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  1. GBS-CIDP Foundation International (

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  1. Institute of Laboratory Medicine, University Hospital Schleswig- Holstein Campus Kiel, Germany, commercial antibody testing service, 20%

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Patients with nodal/paranodal antibodies represent a specific subgroup of inflammatory peripheral neuropathies, whose clinical presentation with a prolonged subacute phase, additional symptoms such as ataxia and tremor, and poor treatment response to IV immunoglobulin (IVIG) often differs from classic Guillain-Barré syndrome (GBS) or chronic inflammatory demyelinating polyneuropathy (CIDP).1

Previous studies on nodo/paranodopathies mainly focused on adult patients, whereas the clinical spectrum of pediatric patients is less well established. We reviewed the clinical presentation of 54 children with GBS (n = 42) and CIDP (n = 12) and retrospectively screened for antibodies against neurofascin155 (NF155), NF186, NF140, contactin-1 (CNTN1), contactin-associated protein1 (CASPR1), and glycine-receptor (GlyR) using cell-based assays2,3; 1 patient was additionally tested with CNTN1-ELISA.4 All cases with sufficient serum were tested for ganglioside-IgG-, IgA-, and IgM-antibodies against GM1 (n = 42), GD1a (n = 18), GD1b (n = 23), and GQ1b (n = 21).5 Clinical and paraclinical information of all patients is summarized in the table. The study was approved by the ethics committee (EK1773/2016).

View this table:
Table

Clinical and paraclinical data of patients with GBS and CIDP

Children with classic GBS

Of 42 children with GBS, 26 were classified as acute inflammatory demyelinating polyneuropathy (AIDP), 7 as acute motor/motor-sensory axonal neuropathy (AMAN/AMSAN) by nerve conduction velocity according to Hadden criteria,6 4 as Miller-Fisher syndrome (MFS), and 2 as MFS/GBS overlap. Three patients with GBS could not be classified because of lack of nerve-conduction studies. In 25 of 35 patients (71.4%), an infection was reported within 4 weeks before symptom onset (13 gastrointestinal, 4 respiratory, and 8 unspecified). Eight patients had IgG-ganglioside antibodies (19.0%), 6 IgM (14.2%), and 1 IgA (2.4%). Nodal/paranodal antibodies were not detected. Patients with AMAN/AMSAN (5/7 with reported infection: 1 campylobacter jejuni, 1 varicella-zoster virus, and 3 unspecified) were more often ganglioside antibody positive (6/7) than patients with AIDP (4/26; likelihood ratio 12.419) or MFS (2/4).

Children with nodal/paranodal antibodies

Five of 12 children, who met the EFNS/PNS criteria for CIDP, had nodal/paranodal antibodies: 2 pan-neurofascin (NF155/NF186/140 triple positive), 1 NF155, and 2 CNTN1-antibodies. The IgG-subclass distribution was determined by flow cytometry analysis.7 IgG4 was the predominant subclass in all patients and ranged from 75% to 100%. In addition, 1 patient with pan-neurofascin-antibodies tested positive for GlyR-antibodies but did not develop stiff-person syndrome or progressive encephalomyelitis with rigidity, and the significance of this finding needs further investigation. The mean age was 7.9 years (range 3–11), and the male:female ratio was 3:2. The median duration of hospitalization was 13 days (range 2–28). One pan-neurofascin-patient was initially diagnosed as GBS and reclassified as CIDP during disease course, the other patients had a chronic onset with slow progression over months or years. One child had a gastrointestinal infection before symptom onset. One CNTN1-patient showed cranial nerve involvement and optic neuritis during disease course. All children had ataxia, 4 neuropathic pain (all except 1 pan-neurofascin), and 3 (2 CNTN1, and 1 pan-neurofascin) tremor. At the peak of disease, 3 children needed a walking aid (Hughes 3) and 2 were bedridden (Hughes 4). None of the children had renal dysfunction. The mean CSF white cell count was 4.6 μL (range 0–21), and the mean CSF protein was 292.4 mg/dL (range 75–619).

The mean time of follow-up was 32 months (range 17–57). The 2 CIDP patients with pan-neurofascin-antibodies initially showed no or only partial response to IVIG and therefore received corticosteroids, 1 along with plasma exchange and the other with mycophenolate. Both recovered only very slowly over up to 4 years with a modified Rankin Scale (mRS) score of 1 at the last follow-up. The NF155-patient did not respond to IVIG and corticosteroids and subsequently received immunoadsorption and rituximab, leading to significant clinical improvement. After 8 months, he relapsed in association with normalization of the CD19/20 ratio and again rapidly improved after another dose of rituximab, with a mRS score of 2 at the last follow-up. One patient with CNTN1-antibodies worsened despite monthly IVIG and corticosteroids given over 4 months. After treatment was switched to rituximab, he improved rapidly in the following weeks and remained stable since then. The second child with CNTN1-antibodies showed only partial response to IVIG with relapses in conjunction with infections. This child improved significantly after rituximab application with a mRS score of 2 at the last follow-up.

In summary, our study demonstrates that nodal/paranodal antibodies occur in a subgroup of paediatric patients with CIDP, but not GBS. Children with AMAN/AMSAN frequently have ganglioside antibodies. Children with CIDP and atypical/prolonged disease course with high Hughes score (>2), sensory ataxia, prominent neuropathic pain, and tremor may have nodal/paranodal antibodies. These patients often do not sufficiently respond to IVIG, whereas in our case series, rituximab led to prompt improvement in 3 children. Optimal treatment strategies for children with nodal/paranodal antibodies have to be further determined in larger studies.

Study funding

This work was partly supported by grants from the “Jubiläumsfonds der Österreichischen Nationalbank,” project 16919 (R. Höftberger), the GBS/CIDP Foundation International (J. Wanschitz), Austrian Science Fund FWF, DOC 33-B27 (R. Höftberger, M. Winklehner) and I3334-B27 (R. Höftberger), Hertha Firnberg project number T996-B30 (I. Koneczny), the PI16/000627 grant of the Fondo de Investigaciones Sanitarias—Instituto de Salud Carlos III (fondos FEDER) (L. Querol), personal grant SLT006/17/00131 of the Pla estratègic de Recerca i Innovació en Salut (PERIS), Departament de Salut, Generalitat de Catalunya (L. Querol), and the German Ministry of Education and Research (BMBF, 01 GM1908A).

Disclosure

D. De Simoni, G. Ricken, M. Winklehner, I. Koneczny, M. Karenfort, U. Hustedt, U. Seidel, O. Abdel-Mannan, P. Munot, S. Rinaldi, C. Steen, M. Freilinger, M. Breu, R. Seidl, M. Reindl, J. Wanschitz, C. Lleixá, G. Bernert, K.P. Wandinger, R. Junker, L. Querol, F. Leypoldt, K. Rostásy, and R. Höftberger report no disclosures relevant to the manuscript. Go to Neurology.org/NN for full disclosures.

Acknowledgment

The authors thank Valerie Pichler and Sopie Dürauer for technical assistance and Andreas Spittler from the Flow Cytometry Core facility of the MUV for technical support.

Appendix Authors

Table

Footnotes

  • Go to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.

  • ↵* These authors contributed equally to this work.

  • The Article Processing Charge was funded by Austrian Science Fund.

  • Received March 8, 2020.
  • Accepted in final form April 29, 2020.
  • Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution License 4.0 (CC BY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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