Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease

Carles Gaig, Yaroslau Compta, Anna Heidbreder, Maria J Marti, Maarten J Titulaer, Yvette Crijnen, Birgit Högl, Jan Lewerenz, María Elena Erro, Juan Carlos Garcia-Monco, Pasquale Nigro, Nicola Tambasco, Maja Patalong-Ogiewa, Marcus Erdler, Stefan Macher, Evelyn Berger-Sieczkowski, Romana Höftberger, Christian Geis, Markus Hutterer, Angela Milán-TomásAntonio Martin-Bastida, Lydia Lopez Manzanares, Sonia Quintas, Günter U Höglinger, Nora Möhn, Florian Schoeberl, Franziska S Thaler, Gian Maria Asioli, Federica Provini, Giuseppe Plazzi, Koldo Berganzo, Morten Blaabjerg, Norbert Brüggemann, Tarsis Farias, Chen Fei Ng, Caroline Giordana, Alejandro Herrero-San Martín, Lucio Huebra, Katya Kotschet, Herburg Liendl, Teresa Montojo, Carlos Morata, Jesus Perez Perez, Inmaculada Puertas, Thomas Seifert-Held, Caspar Seitz, Mateus Mistieri Simabukuro, Nieves Tellez, Javier Villacieros-Álvarez, Barbara Willekens, Lidia Sabater, Alex Iranzo, Joan Santamaria Cano, Josep Dalmau, Francesc Graus


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.

Original languageEnglish
Publication statusPublished - 2021

Research Areas and Centers

  • Academic Focus: Center for Brain, Behavior and Metabolism (CBBM)


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