Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease

Gaig C 1 , Compta Y 2 , Heidbreder A 3 , Marti MJ 2 , Titulaer MJ 4 , Crijnen Y 4 Show all authors , Högl B 5 , Lewerenz J 6 , Erro ME 7 , Garcia-Monco JC 8 , Nigro P 9 , Tambasco N 9 , Patalong-Ogiewa M 10 , Erdler M 11 , Macher S 12 , Berger-Sieczkowski E 12 , Höftberger R 13 , Geis C 14 , Hutterer M 15 , Milán-Tomás A 16 , Martin-Bastida A 16 , Manzanares LL 17 , Quintas S 17 , Höglinger GU 18 , Möhn N 18 , Schoeberl F 19 , Thaler FS 20 , Asioli GM 21 , Provini F 21 , Plazzi G 22 , Berganzo K 23 , Blaabjerg M 24 , Brüggemann N 25 , Farias T 26 , Ng CF 27 , Giordana C 28 , Herrero-San Martín A 29 , Huebra L 30 , Kotschet K 31 , Liendl H 32 , Montojo T 33 , Morata C 34 , Perez JP 35 , Puertas I 36 , Seifert-Held T 37 , Seitz C 38 , Simabukuro MM 39 , Tellez N 40 , Villacieros-Álvarez J 41 , Willekens B 42 , Sabater L 1 , Iranzo A 1 , Cano JS 1 , Dalmau J 1 , Graus F 43

Affiliations 

  • 1 Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain
  • 2 Department of Neurology, Hospital Clinic, Barcelona, Spain
  • 3 Department Neurology, Division of Sleep Medicine and Neuromuscular Disorders, University Hospital Muenster, Muenster, Germany
  • 4 Department of Neurology, Erasmus MC University Medical Center, Rotterdam, the Netherlands
  • 5 Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria
  • 6 Department of Neurology, Ulm University, Ulm, Germany
  • 7 Neurology Department, Complejo Hospitalario de Navarra, Navarra Institute for Health Research (IdiSNA), Pamplona, Spain
  • 8 Neurology Department. Hospital Universitario de Basurto. Bilbao. Spain (formerly Department of Neurology, Hospital de Galdakao, Spain)
  • 9 Movement Disorders Center, Perugia General Hospital and University of Perugia, Perugia, Italy
  • 10 Department of Neurology and Neurorehabilitation. Faculty of Medical Sciences in Katowice. Medical University of Silesia, Katowice, Poland
  • 11 Department of Neurology, Klinik Donaustadt, Karl-Landsteiner-Institut, Vienna, Austria
  • 12 Department of Neurology, Medical University of Vienna, Austria
  • 13 Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Austria
  • 14 Section Translational Neuroimmunology, Department of Neurology, Jena University Hospital, Jena, Germany
  • 15 Department of Neurology 1, Neuromed Campus, Kepler Universitätsklinikum Linz, Linz, Austria. Department of Neurology with Stroke Unit and Acute Geriatrics, Saint John of God Hospital Linz, Linz, Austria
  • 16 Department of Neurology and Neurosciences, Clínica Universidad de Navarra, Pamplona-Madrid. Spain
  • 17 Department of Neurology, Hospital La Princesa, Madrid, Spain
  • 18 Department of Neurology, Hannover Medical School, Hannover, Germany
  • 19 Department of Neurology, Ludwig-Maximilians-University; Munich, Germany
  • 20 Institute of Clinical Neuroimmunology, University Hospital and Biomedical Center, Ludwig-Maximilians University Munich, Munich, Germany
  • 21 Department of Biomedical and NeuroMotor Sciences (DiBiNeM), Alma Mater Studiorum University of Bologna, Bologna, Italy
  • 22 Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio Emilia, Modena, Italy. IRCCS, Institute of Neurological Sciences of Bologna, Bologna, Italy
  • 23 Neurology Department, Cruces University Hospital, Barakaldo, Spain
  • 24 Department of Neurology, Odense University Hospital, Odense, Denmark
  • 25 Department of Neurology and Institute of Neurogenetics, University of Lübeck, Lübeck, Germany
  • 26 Department of Neurology, Medical Clinic of Hospital Geral Cleriston Andrade, Feira de Santana, Brazil
  • 27 Neurology Unit, Department of Medicine, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia
  • 28 Department of Movement Disorders and Neurology, CHU Nice, Nice, France
  • 29 Department of Neurology, Hospital Universitario 12 de Octubre, Madrid, Spain
  • 30 Department of Psychobiology, Universidade Federal de São Paulo, São Paulo, Brazil
  • 31 Clinical Neurosciences, St Vincent's Hospital, Melbourne, Australia
  • 32 Department of Neurology, LKH Murtal, Standort Knittelfeld, Austria
  • 33 Department of Neurology, Fundación Jiménez Diaz, Madrid, Spain
  • 34 Department of Neurology, Hospital Universitari i Politècnic La Fe, Valencia, Spain
  • 35 Department of Neurology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain
  • 36 Department of Neurology, Hospital La Paz, Madrid, Spain
  • 37 Department of Neurology, Medical Universtity of Graz, Graz, Austria
  • 38 Department of Neurology, Mainz University Hospital, Mainz, Germany
  • 39 Department of Neurology, University of São Paulo Medical School, São Paulo, Brazil
  • 40 Department of Neurology, Hospital Clínico Universitario, Valladolid, Spain
  • 41 Department of Neurology, Hospital Universitario Rey Juan Carlos, Madrid, Spain
  • 42 Department of Neurology, Antwerp University Hospital and University of Antwerp, Antwerp, Belgium
  • 43 Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain francesc.graus@idibaps.org
Neurology, 2021 Aug 11.
PMID: 34380749 DOI: 10.1212/WNL.0000000000012639

Abstract

OBJECTIVE: Anti-IgLON5 disease is a recently described neurological disease that shares features of autoimmunity and neurodegeneration. Abnormal movements appear to be frequent and important but have not been characterized and are under-reported. Here we describe the frequency and types of movement disorders in a series of consecutive patients with this disease.

METHODS: In this retrospective, observational study, the presence and phenomenology of movement disorders were assessed with a standardized clinical questionnaire. Available videos were centrally reviewed by three experts in movement disorders.

RESULTS: Seventy two patients were included. In 41 (57%) the main reason for initial consultation was difficulty walking along with one or several concurrent movement disorders. At the time of anti-IgLON5 diagnosis, 63 (87%) patients had at least one movement disorder with a median of three per patient. The most frequent abnormal movements were gait and balance disturbances (52 patients, 72%), chorea (24, 33%), bradykinesia (20, 28%), dystonia (19, 26%), abnormal body postures or rigidity (18, 25%), and tremor (15, 21%). Other hyperkinetic movements (myoclonus, akathisia, myorhythmia, myokymia, or abdominal dyskinesias) occurred in 26 (36%) patients. The craniofacial region was one of the most frequently affected by multiple concurrent movement disorders (23 patients, 32%) including dystonia (13), myorhythmia (6), chorea (4) or myokymia (4). Considering any body region, the most frequent combination of multiple movement disorders consisted of gait instability or ataxia associated with craniofacial dyskinesias or generalized chorea observed in 31(43%) of patients. In addition to abnormal movements, 87% of patients had sleep alterations, 74% bulbar dysfunction, and 53% cognitive impairment. Fifty-five (76%) patients were treated with immunotherapy, resulting in important and sustained improvement of the movement disorders in only seven (13%) cases.

CONCLUSIONS: Movement disorders are a frequent and leading cause of initial neurological consultation in patients with anti-IgLON5 disease. Although multiple types of abnormal movements can occur, the most prevalent are disorders of gait, generalized chorea, and dystonia and other dyskinesias that frequently affect craniofacial muscles. Overall, anti-IgLON5 disease should be considered in patients with multiple movement disorders, particularly if they occur in association with sleep alterations, bulbar dysfunction, or cognitive impairment.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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