2021/01/11 by Hiroshi Mitoma, Mario Manto, Marios Hadjivassiliou · 1 citation
Medicine · Neuroscience · #Autoimmune Neurological Disorders and Treatments #Genetic Neurodegenerative Diseases #Peripheral Neuropathies and Disorders
paper · pdf · doi:10.14802/jmd.20040
openalex publication_date 2021/01/11 · openalex created_date 2025/10/10 · openalex updated_date 2026/06/13
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.