Autoimmune and paraneoplastic chorea: A narrative review
Debayan Dutta, Rohit Keshav, Jacky Ganguly, Gulnara Saidakhmetova, Soumava Mukherjee, Purba Basu, Hrishikesh KumarAbstract
INTRODUCTION:
Chorea is a hyperkinetic, dance-like movement disorder with numerous genetic and acquired etiologies. Autoimmune chorea is a subset of acquired chorea in which antibody-mediated disruption of basal ganglia circuits is the underlying mechanism. It may occur in the context of autoimmune encephalitis (e.g., N-methyl-D-aspartate [NMDA] encephalitis), paraneoplastic movement disorders (e.g., anti-CV2/collapsin response mediator protein 5 [CRMP5] encephalitis), systemic autoimmune disorders (e.g., systemic lupus erythematosus [SLE]), antiphospholipid syndrome (APS), or post-streptococcal infection in children (Sydenham’s chorea).
OBJECTIVE:
This review aims to summarize the available literature and propose a clinical approach to chorea associated with various autoimmune disorders.
METHODS:
Following a structured selection process, 104 articles were included, most of which consisted of case series and case reports.
RESULTS:
Asymmetric or unilateral chorea may be the initial manifestation of an underlying autoimmune disorder. However, chorea often occurs in conjunction with other hyperkinetic movement disorders. The most common distribution patterns include limb chorea, orofacial chorea/dyskinesia, and generalized chorea. Early initiation of treatment is associated with favorable outcomes, except in paraneoplastic etiologies.
CONCLUSION:
Autoimmune chorea is a treatable, reversible antibody-associated movement disorder. Increased awareness, early recognition, comprehensive antibody testing, and age-appropriate malignancy screening are essential for optimal diagnosis and management.