| Abstract [eng] |
Autoimmune and paraneoplastic chorea are rare but clinically significant causes of involuntary hyperkinetic movement disorders arising from immune-mediated dysfunction of the basal ganglia. Unlike hereditary or degenerative causes of chorea, these conditions are potentially reversible, making early recognition essential for improving neurological outcomes and, in paraneoplastic cases, enabling early detection of underlying malignancy. Despite their importance, these disorders remain underdiagnosed due to heterogeneous clinical presentations and limited standardized diagnostic frameworks. The aim of this study was to summarize current evidence regarding the clinical features, underlying mechanisms, diagnosis, and management of autoimmune and paraneoplastic chorea. The objectives were to define the clinical spectrum, evaluate immunological mechanisms and neuronal autoantibodies, assess diagnostic approaches, and identify treatment strategies and factors influencing outcomes. 4 This study was conducted as a structured literature review of autoimmune and paraneoplastic chorea. Relevant publications were identified through literature searches in PubMed, Scopus, Web of Science, and Google Scholar. Due to heterogeneity among the available studies, findings were synthesized using a narrative descriptive approach. The findings demonstrate that autoimmune chorea encompasses parainfectious, systemic autoimmune, paraneoplastic, and idiopathic autoimmune forms. Clinical presentation is typically subacute and often associated with additional neurological or systemic features. Diagnostic evaluation relies on phenotype-driven assessment, supported by neuroimaging, CSF analysis, and targeted antibody testing. Treatment approaches include immunotherapy, tumor-directed therapy in paraneoplastic cases, and symptomatic management. Outcomes vary widely, with better responses observed in non-paraneoplastic and neuronal surface antibody-associated conditions. In conclusion, autoimmune and paraneoplastic chorea represent heterogeneous but treatable disorders. A structured, phenotype-based diagnostic approach combined with early therapeutic intervention is critical to optimize patient outcomes. |
