Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease

Gaig, C; Compta, Y; Heidbreder, A; Marti, MJ; Titulaer, MJ; Crijnen, Y; Hogl, B; Lewerenz, J; Erro, ME; Garcia-Monco, JC; Nigro, P; Tambasco, N; Patalong-Ogiewa, M; Erdler, M; Macher, S; Berger-Sieczkowski, E; Hoftberger, R; Geis, C; Hutterer, M; Milan-Tomas, A; Martin-Bastida, A; Manzanares, LL; Quintas, S; Hoglinger, GU; Mohn, N; Schoberl, F; Thaler, FS; Asioli, GM; Provini, F; Plazzi, G; Berganzo, K; Blaabjerg, M; Bruggemann, N; Farias, T; Ng, CF; Giordana, C; Herrero-San Martin, A; Huebra, L; Kotschet, K; Liendl, H; Montojo, T; Morata, C; Perez, JP; Puertas, I; Seifert-Held, T; Seitz, C; Simabukuro, MM; Tellez, N; Villacieros-Alvarez, J; Willekens, B; Sabater, L; Iranzo, A; Cano, JS; Dalmau, J; Graus, F

Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease

Background and Objectives Anti-IgLON5 disease is a recently described neurologic disease that shares features of autoimmunity and neurodegeneration. Abnormal movements appear to be frequent and important but have not been characterized and are underreported. We describe the frequency and types of mo...

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Autores:	Gaig, C, Compta, Y, Heidbreder, A, Marti, MJ, Titulaer, MJ, Crijnen, Y, Hogl, B, Lewerenz, J, Erro, ME, Garcia-Monco, JC, Nigro, P, Tambasco, N, Patalong-Ogiewa, M, Erdler, M, Macher, S, Berger-Sieczkowski, E, Hoftberger, R, Geis, C, Hutterer, M, Milan-Tomas, A, Martin-Bastida, A, Manzanares, LL, Quintas, S, Hoglinger, GU, Mohn, N, Schoberl, F, Thaler, FS, Asioli, GM, Provini, F, Plazzi, G, Berganzo, K, Blaabjerg, M, Bruggemann, N, Farias, T, Ng, CF, Giordana, C, Herrero-San Martin, A, Huebra, L, Kotschet, K, Liendl, H, Montojo, T, Morata, C, Perez, JP, Puertas, I, Seifert-Held, T, Seitz, C, Simabukuro, MM, Tellez, N, Villacieros-Alvarez, J, Willekens, B, Sabater, L, Iranzo, A, Cano, JS, Dalmau, J, Graus, F
Tipo de recurso:	artículo
Estado:	Versión publicada
Fecha de publicación:	2021
País:	España
Institución:	Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
Repositorio:	r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
OAI Identifier:	oai:iibsantpau.fundanetsuite.com:p5322
Acceso en línea:	https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=5322 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8520389
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spelling	Frequency and Characterization of Movement Disorders in Anti-IgLON5 DiseaseGaig, CCompta, YHeidbreder, AMarti, MJTitulaer, MJCrijnen, YHogl, BLewerenz, JErro, MEGarcia-Monco, JCNigro, PTambasco, NPatalong-Ogiewa, MErdler, MMacher, SBerger-Sieczkowski, EHoftberger, RGeis, CHutterer, MMilan-Tomas, AMartin-Bastida, AManzanares, LLQuintas, SHoglinger, GUMohn, NSchoberl, FThaler, FSAsioli, GMProvini, FPlazzi, GBerganzo, KBlaabjerg, MBruggemann, NFarias, TNg, CFGiordana, CHerrero-San Martin, AHuebra, LKotschet, KLiendl, HMontojo, TMorata, CPerez, JPPuertas, ISeifert-Held, TSeitz, CSimabukuro, MMTellez, NVillacieros-Alvarez, JWillekens, BSabater, LIranzo, ACano, JSDalmau, JGraus, FBackground and Objectives Anti-IgLON5 disease is a recently described neurologic disease that shares features of autoimmunity and neurodegeneration. Abnormal movements appear to be frequent and important but have not been characterized and are underreported. 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 3 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 1 movement disorder with a median of 3 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%) 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 7 (13%) cases. Discussion Movement disorders are a frequent and leading cause of initial neurologic 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.LIPPINCOTT WILLIAMS & WILKINS2021info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttps://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=5322https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8520389NEUROLOGYISSN: 00283878ISSNe: 1526632Xreponame:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pauinstname:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)Inglésinfo:eu-repo/semantics/openAccessoai:iibsantpau.fundanetsuite.com:p53222026-06-14T12:41:47Z
dc.title.none.fl_str_mv	Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease
title	Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease
spellingShingle	Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease Gaig, C
title_short	Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease
title_full	Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease
title_fullStr	Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease
title_full_unstemmed	Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease
title_sort	Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease
dc.creator.none.fl_str_mv	Gaig, C Compta, Y Heidbreder, A Marti, MJ Titulaer, MJ Crijnen, Y Hogl, B Lewerenz, J Erro, ME Garcia-Monco, JC Nigro, P Tambasco, N Patalong-Ogiewa, M Erdler, M Macher, S Berger-Sieczkowski, E Hoftberger, R Geis, C Hutterer, M Milan-Tomas, A Martin-Bastida, A Manzanares, LL Quintas, S Hoglinger, GU Mohn, N Schoberl, F Thaler, FS Asioli, GM Provini, F Plazzi, G Berganzo, K Blaabjerg, M Bruggemann, N Farias, T Ng, CF Giordana, C Herrero-San Martin, A Huebra, L Kotschet, K Liendl, H Montojo, T Morata, C Perez, JP Puertas, I Seifert-Held, T Seitz, C Simabukuro, MM Tellez, N Villacieros-Alvarez, J Willekens, B Sabater, L Iranzo, A Cano, JS Dalmau, J Graus, F
author	Gaig, C
author_facet	Gaig, C Compta, Y Heidbreder, A Marti, MJ Titulaer, MJ Crijnen, Y Hogl, B Lewerenz, J Erro, ME Garcia-Monco, JC Nigro, P Tambasco, N Patalong-Ogiewa, M Erdler, M Macher, S Berger-Sieczkowski, E Hoftberger, R Geis, C Hutterer, M Milan-Tomas, A Martin-Bastida, A Manzanares, LL Quintas, S Hoglinger, GU Mohn, N Schoberl, F Thaler, FS Asioli, GM Provini, F Plazzi, G Berganzo, K Blaabjerg, M Bruggemann, N Farias, T Ng, CF Giordana, C Herrero-San Martin, A Huebra, L Kotschet, K Liendl, H Montojo, T Morata, C Perez, JP Puertas, I Seifert-Held, T Seitz, C Simabukuro, MM Tellez, N Villacieros-Alvarez, J Willekens, B Sabater, L Iranzo, A Cano, JS Dalmau, J Graus, F
author_role	author
author2	Compta, Y Heidbreder, A Marti, MJ Titulaer, MJ Crijnen, Y Hogl, B Lewerenz, J Erro, ME Garcia-Monco, JC Nigro, P Tambasco, N Patalong-Ogiewa, M Erdler, M Macher, S Berger-Sieczkowski, E Hoftberger, R Geis, C Hutterer, M Milan-Tomas, A Martin-Bastida, A Manzanares, LL Quintas, S Hoglinger, GU Mohn, N Schoberl, F Thaler, FS Asioli, GM Provini, F Plazzi, G Berganzo, K Blaabjerg, M Bruggemann, N Farias, T Ng, CF Giordana, C Herrero-San Martin, A Huebra, L Kotschet, K Liendl, H Montojo, T Morata, C Perez, JP Puertas, I Seifert-Held, T Seitz, C Simabukuro, MM Tellez, N Villacieros-Alvarez, J Willekens, B Sabater, L Iranzo, A Cano, JS Dalmau, J Graus, F
author2_role	author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author
description	Background and Objectives Anti-IgLON5 disease is a recently described neurologic disease that shares features of autoimmunity and neurodegeneration. Abnormal movements appear to be frequent and important but have not been characterized and are underreported. 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 3 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 1 movement disorder with a median of 3 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%) 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 7 (13%) cases. Discussion Movement disorders are a frequent and leading cause of initial neurologic 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.
publishDate	2021
dc.date.none.fl_str_mv	2021
dc.type.none.fl_str_mv	info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion
format	article
status_str	publishedVersion
dc.identifier.none.fl_str_mv	https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=5322 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8520389
url	https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=5322 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8520389
dc.language.none.fl_str_mv	Inglés
language_invalid_str_mv	Inglés
dc.rights.none.fl_str_mv	info:eu-repo/semantics/openAccess
eu_rights_str_mv	openAccess
dc.publisher.none.fl_str_mv	LIPPINCOTT WILLIAMS & WILKINS
publisher.none.fl_str_mv	LIPPINCOTT WILLIAMS & WILKINS
dc.source.none.fl_str_mv	NEUROLOGY ISSN: 00283878 ISSNe: 1526632X reponame:r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau instname:Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
instname_str	Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau)
reponame_str	r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
collection	r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
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Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease

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