Frequency and Characterization of Movement Disorders in Anti-IgLON5 Disease
Por:
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
Publicada:
5 oct 2021
Resumen:
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.
Filiaciones:
Gaig, C:
Inst Invest Biomed August Pi & Sunyer IDIBAPS, Neuroimmunol Program, Barcelona, Spain
Hosp Clin Barcelona, Dept Neurol, Barcelona, Spain
Hosp Clin Barcelona, Multidisciplinary Sleep Disorders Unit, Barcelona, Spain
Compta, Y:
Hosp Clin Barcelona, Dept Neurol, Barcelona, Spain
Hosp Clin Barcelona, Parkinsons Dis & Movement Disorders Unit, Barcelona, Spain
Heidbreder, A:
Univ Hosp Muenster, Dept Neurol, Div Sleep Med & Neuromuscular Disorders, Munster, Germany
Med Univ Innsbruck, Dept Neurol, Innsbruck, Austria
Marti, MJ:
Hosp Clin Barcelona, Dept Neurol, Barcelona, Spain
Hosp Clin Barcelona, Parkinsons Dis & Movement Disorders Unit, Barcelona, Spain
Titulaer, MJ:
Erasmus MC Univ Med Ctr, Dept Neurol, Rotterdam, Netherlands
Crijnen, Y:
Erasmus MC Univ Med Ctr, Dept Neurol, Rotterdam, Netherlands
Hogl, B:
Med Univ Innsbruck, Dept Neurol, Innsbruck, Austria
Lewerenz, J:
Ulm Univ, Dept Neurol, Ulm, Germany
Erro, ME:
Complejo Hosp Navarra, Navarra Inst Hlth Res IdiSNA, Neurol Dept, Pamplona, Spain
Garcia-Monco, JC:
Hosp Univ Basun, Neurol Dept, Bilbao, Spain
Hosp Galdakao, Dept Neurol, Bizkaia, Spain
Nigro, P:
Perugia Gen Hosp, Movement Disorders Ctr, Perugia, Italy
Univ Perugia, Perugia, Italy
Tambasco, N:
Perugia Gen Hosp, Movement Disorders Ctr, Perugia, Italy
Univ Perugia, Perugia, Italy
Patalong-Ogiewa, M:
Med Univ Silesia, Fac Med Sci Katowice, Dept Neurol & Neurorehabil, Katowice, Poland
Erdler, M:
Karl Landsteiner Inst, Dept Neurol, Klin Donaustadt, Vienna, Austria
Hoftberger, R:
Med Univ Vienna, Dept Neurol, Div Neuropathol & Neurochem, Vienna, Austria
Geis, C:
Jena Univ Hosp, Dept Neurol, Sect Translat Neuroimmunol, Jena, Germany
Hutterer, M:
Kepler Univ Klinikum Linz, Dept Neurol 1, Neuromed Campus, Linz, Austria
St John God Hosp Linz, Stroke Unit, Dept Neurol, Linz, Austria
St John God Hosp Linz, Acute Geriatr, Linz, Austria
Milan-Tomas, A:
Clin Univ Navarra, Dept Neurol & Neurosci, Pamplona, Spain
Martin-Bastida, A:
Clin Univ Navarra, Dept Neurol & Neurosci, Pamplona, Spain
Manzanares, LL:
Hosp La Princesa, Dept Neurol, Madrid, Spain
Quintas, S:
Hosp La Princesa, Dept Neurol, Madrid, Spain
Hoglinger, GU:
Hannover Med Sch, Dept Neurol, Hannover, Germany
Mohn, N:
Hannover Med Sch, Dept Neurol, Hannover, Germany
Schoberl, F:
Ludwig Maximilians Univ Munchen, Dept Neurol, Univ Hosp, Munich, Germany
Ludwig Maximilians Univ Munchen, Univ Hosp, Inst Clin Neuroimmunol, Munich, Germany
Ludwig Maximilians Univ Munchen, Biomed Ctr, Munich, Germany
Asioli, GM:
Alma Mater Studiorum Univ Bologna, Dept Biomed & NeuroMotor Sci DiBiNeM GMA, FP, Bologna, Italy
Provini, F:
Alma Mater Studiorum Univ Bologna, Dept Biomed & NeuroMotor Sci DiBiNeM GMA, FP, Bologna, Italy
IRCCS Ist Sci Neurol Bologna, Bologna, Italy
Plazzi, G:
Univ Modena & Reggio Emilia, Dept Biomed Metab & Neural Sci, Bologna, Italy
Inst Neurol Sci Bologna, IRCCS, Bologna, Italy
Berganzo, K:
Cruces Univ Hosp, Neurol Dept, Baracaldo, Spain
Blaabjerg, M:
Odense Univ Hosp, Dept Neurol, Odense, Denmark
Bruggemann, N:
Univ Lubeck, Dept Neurol, Lubeck, Germany
Univ Lubeck, Inst Neurogenet, Lubeck, Germany
Farias, T:
Hosp Geral Cleriston Andrade, Dept Neurol, Med Clin, Feira De Santana, BA, Brazil
Ng, CF:
Univ Kebangsaan Malaysia, Dept Med, Neurol Unit, Med Ctr, Kuala Lumpur, Malaysia
Giordana, C:
CHU Nice, Dept Movement Disorders & Neurol, Nice, France
Herrero-San Martin, A:
Hosp Univ 12 Octubre, Dept Neurol, Madrid, Spain
Huebra, L:
Univ Fed Sao Paulo, Dept Psychobiol, Sao Paulo, SP, Brazil
Kotschet, K:
St Vincents Hosp, Clin Neurosci, Melbourne, Vic, Australia
Liendl, H:
LKH Murtal, Dept Neurol, Standort Knittelfeld, Austria
Montojo, T:
Fdn Jimenez Diaz, Dept Neurol, Madrid, Spain
Morata, C:
Hosp Univ & Politecn La Fe, Dept Neurol, Valencia, Spain
Perez, JP:
Hosp Santa Creu & Sant Pau, Dept Neurol, Barcelona, Spain
Puertas, I:
Hosp La Paz, Dept Neurol, Madrid, Spain
Seifert-Held, T:
Med Univ Graz, Dept Neurol, Graz, Austria
Seitz, C:
Mainz Univ Hosp, Dept Neurol, Mainz, Germany
Simabukuro, MM:
Univ Sao Paulo, Dept Neurol, Med Sch, Sao Paulo, SP, Brazil
Tellez, N:
Hosp Clin Univ, Dept Neurol, Valladolid, Spain
Villacieros-Alvarez, J:
Hosp Univ Rey Juan Carlos, Dept Neurol, Madrid, Spain
Willekens, B:
Antwerp Univ Hosp, Dept Neurol, Antwerp, Belgium
Univ Antwerp, Antwerp, Belgium
Sabater, L:
Inst Invest Biomed August Pi & Sunyer IDIBAPS, Neuroimmunol Program, Barcelona, Spain
Iranzo, A:
Inst Invest Biomed August Pi & Sunyer IDIBAPS, Neuroimmunol Program, Barcelona, Spain
Hosp Clin Barcelona, Dept Neurol, Barcelona, Spain
Hosp Clin Barcelona, Multidisciplinary Sleep Disorders Unit, Barcelona, Spain
Cano, JS:
Inst Invest Biomed August Pi & Sunyer IDIBAPS, Neuroimmunol Program, Barcelona, Spain
Hosp Clin Barcelona, Dept Neurol, Barcelona, Spain
Hosp Clin Barcelona, Multidisciplinary Sleep Disorders Unit, Barcelona, Spain
Dalmau, J:
Inst Invest Biomed August Pi & Sunyer IDIBAPS, Neuroimmunol Program, Barcelona, Spain
Hosp Clin Barcelona, Dept Neurol, Barcelona, Spain
Inst Catalana Recerca & Estudis Avancats ICREA, Barcelona, Spain
Graus, F:
Inst Invest Biomed August Pi & Sunyer IDIBAPS, Neuroimmunol Program, Barcelona, Spain
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