Since the first description of immune-mediated cerebellar ataxias (IMCAs) by Charcot in 1868, several milestones have been reached in our understanding of this group of neurological disorders. IMCAs have diverse etiologies, such as gluten ataxia, postinfectious cerebellitis, paraneoplastic cerebellar degeneration, opsoclonus myoclonus syndrome, anti-GAD ataxia, and primary autoimmune cerebellar ataxia. The cerebellum, a vulnerable autoimmune target of the nervous system, has remarkable capacities (collectively known as the cerebellar reserve, closely linked to plasticity) to compensate and restore function following various pathological insults. Therefore, good prognosis is expected when immune-mediated therapeutic interventions are delivered during early stages when the cerebellar reserve can be preserved. However, some types of IMCAs show poor responses to immunotherapies, even if such therapies are introduced at an early stage. Thus, further research is needed to enhance our understanding of the autoimmune mechanisms underlying IMCAs, as such research could potentially lead to the development of more effective immunotherapies. We underscore the need to pursue the identification of robust biomarkers.
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DPPX antibody-mediated disease mimicking Wernicke’s encephalopathy Yasmin Soliman, Neha Irani, Kevin O'Connor BMJ Case Reports.2025; 18(1): e262573. CrossRef
The cerebellum in epilepsy Christopher Elder, Rebecca Kerestes, Puneet Opal, Maria Marchese, Orrin Devinsky Epilepsia.2025;[Epub] CrossRef
Different Purkinje cell pathologies cause specific patterns of progressive gait ataxia in mice Dick Jaarsma, Maria B. Birkisdóttir, Randy van Vossen, Demi W.G.D. Oomen, Oussama Akhiyat, Wilbert P. Vermeij, Sebastiaan K.E. Koekkoek, Chris I. De Zeeuw, Laurens W.J. Bosman Neurobiology of Disease.2024; 192: 106422. CrossRef
Paraneoplastic Cerebellar Degeneration Associated with Breast Cancer: A Case Report and a Narrative Review Rosario Luca Norrito, Maria Grazia Puleo, Chiara Pintus, Maria Grazia Basso, Giuliana Rizzo, Tiziana Di Chiara, Domenico Di Raimondo, Gaspare Parrinello, Antonino Tuttolomondo Brain Sciences.2024; 14(2): 176. CrossRef
Immune‐mediated spastic ataxia masquerading as clinically probable multisystem atrophy in an elderly woman Rithvik Ramesh, Anuhya Chadalawada, Pedapati Radhakrishna, Lakshmi Narasimhan Ranganathan, Philo Hazeena, Sundar Shanmugam, Deepa Avadhani Clinical and Experimental Neuroimmunology.2024; 15(3): 146. CrossRef
Paraneoplastic neurological syndromes: upgraded approaches to diagnosis V. N. Grigoryeva, E. A. Ruina Russian neurological journal.2024; 29(1): 4. CrossRef
Neuronal antibodies in nonparaneoplastic autoimmune cerebellar ataxias Albert Saiz, Francesc Graus Current Opinion in Neurology.2024; 37(3): 322. CrossRef
Paraneoplastic Cerebellar Ataxia: A Case Report and Literature Review 静 刘 Advances in Clinical Medicine.2024; 14(03): 350. CrossRef
Autoantibodies related to ataxia and other central nervous system manifestations of gluten enteropathy Tsvetelina Velikova, Georgi Vasilev, Russka Shumnalieva, Lyubomir Chervenkov, Dimitrina Georgieva Miteva, Milena Gulinac, Stamatios Priftis, Snezhina Lazova World Journal of Clinical Cases.2024; 12(12): 2031. CrossRef
Clinical Presentation, Management, and Diagnostic Performance of 2021 Criteria for Paraneoplastic Neurologic Syndromes in Childhood Ji Zhou, Mei Jin, Yan Su, Xiuwei Zhuo, Libing Fu, Xiaotun Ren, Changhong Ren, Anna Zhou, Jiuwei Li, Weihua Zhang Neurology Neuroimmunology & Neuroinflammation.2024;[Epub] CrossRef
Recent advances in diagnosis of immune-mediated cerebellar ataxias: novel concepts and fundamental questions on autoimmune mechanisms Hiroshi Mitoma, Mario Manto Journal of Neurology.2024; 271(10): 7046. CrossRef
Onconeural antibody-associated cerebellar ataxia: An analysis Rohan Mahale, Sandeep M, Anitha Mahadevan, Nitish Kamble, Vikram Holla, Pramod K. Pal, Ravi Yadav Annals of Movement Disorders.2024; 7(2): 102. CrossRef
Roles of CDR2 and CDR2L in Anti-Yo Paraneoplastic Cerebellar Degeneration: A Literature Review Pablo S. Martínez Lozada, Rafael Mancero Montalvo, Andrea Iturralde Carrillo, Maria Montesdeoca-Lozada, Jose A. Rodas, Jose E. Leon-Rojas International Journal of Molecular Sciences.2024; 26(1): 70. CrossRef
Autoimmunologische Kleinhirnerkrankungens Niklas Vogel, Christian Hartmann, Sven Meuth, Nico Melzer Nervenheilkunde.2023; 42(01/02): 73. CrossRef
Evaluation and management of acute high-grade immunotherapy-related neurotoxicity Marcelo Sandoval, Adriana H. Wechsler, Zahra Alhajji, Jayne Viets-Upchurch, Patricia Brock, Demis N. Lipe, Aisha Al-breiki, Sai-Ching J. Yeung Heliyon.2023; 9(3): e13725. CrossRef
Consensus Paper: Latent Autoimmune Cerebellar Ataxia (LACA) Mario Manto, Marios Hadjivassiliou, José Fidel Baizabal-Carvallo, Christiane S Hampe, Jerome Honnorat, Bastien Joubert, Hiroshi Mitoma, Sergio Muñiz-Castrillo, Aasef G. Shaikh, Alberto Vogrig The Cerebellum.2023; 23(2): 838. CrossRef
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Immune-mediated ataxias: Guide to clinicians Alex T. Meira, Marianna P.M. de Moraes, Matheus G. Ferreira, Gustavo L. Franklin, Flávio M. Rezende Filho, Hélio A.G. Teive, Orlando G.P. Barsottini, José Luiz Pedroso Parkinsonism & Related Disorders.2023; 117: 105861. CrossRef
Gluten Ataxia: an Underdiagnosed Condition Marios Hadjivassiliou, R. A. Grϋnewald The Cerebellum.2022; 21(4): 620. CrossRef
Clinical Problem Solving: Decreased Level of Consciousness and Unexplained Hydrocephalus Naomi Niznick, Ronda Lun, Daniel A. Lelli, Tadeu A. Fantaneanu The Neurohospitalist.2022; 12(2): 312. CrossRef
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Acute Cerebellar Inflammation and Related Ataxia: Mechanisms and Pathophysiology Md. Sorwer Alam Parvez, Gen Ohtsuki Brain Sciences.2022; 12(3): 367. CrossRef
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Update on Paraneoplastic Cerebellar Degeneration Philipp Alexander Loehrer, Lara Zieger, Ole J. Simon Brain Sciences.2021; 11(11): 1414. CrossRef