Autoimmune disorders, have become one of the fastest growing set of disorders not only due to improvement in diagnostic armamentarium but also due to significant therapeutic implications. With this implode in autoimmune disorders, the spectrum of autoimmune movement disorders have been steadily increasing. Albeit, Rheumatic chorea is earliest and well-known autoimmune movement disorder, the spectrum encompasses a mixture of hypokinetic, hyperkinetic, ataxic and mixed movement disorders. A brief overview of these disorders is given in table -1. Many of these are associated with underlying malignancies or could indicate towards Paraneoplastic syndromes. High degree of clinical suspicion and proper history and associated findings, give way for diagnostic workup in these patients. Autoantibody testing remains the anchor point in diagnosing these cases, however, Imaging, CSF analysis, electrophysiological evaluation add up to a considerable diagnostic value. The treatment protocol includes symptomatic therapy, immunotherapy and where applicable appropriate oncological interventions. Symptomatic therapies are directed towards the primary clinical symptoms and include dopaminergic agents, dopamine blockers, anticholinergics, antiepileptics, etc. The immunotherapy is based upon the type of antibodies detected and includes pulse methylprednisolone and IVIG therapies in acute phases and azathioprine, mycophenolate or oral glucocorticoids for the chronic phase. Therapeutic outcomes are dependent on the type of antibodies detected and the underlying primary pathology. Many of these patients have good clinical response and outcomes, if detected and intervened early.
Table -1: Overview of autoimmune movement disorders
| Antigen | Antibody | Associated movement disorders |
| LGI1, CASPR2 | VGKC–complex antibody | Brainstem encephalitis, ataxia, chorea, myoclonus, REM sleep behavior disorder |
| GluN1 | NMDA receptor antibody | Chorea, catatonia, orofacial dyskinesias, stereotypies, OMS |
| GluA1, 2 | AMPA receptor antibody | Ataxia |
| GABA-B | GABA-B receptor antibody | Ataxia, OMS |
| P/Q and N-type calcium channels | P/Q and N-type calcium channel antibody | Ataxia, myoclonus |
| alpha1 subunit GlyR | Glycine receptor antibody | PERM, SPS, and variants |
| GAD65 intracellular, but may be neuronal surface as well | GAD65 antibody | SPS, stiff-man phenomena, ataxia, chorea, ophthalmoplegia, parkinsonism |
| DPPX | DPPX-IgG | Ataxia, myoclonus, parasomnias, PERM |
| mGluR1 | mGluR1 | Ataxia |
| IgLON5 | IgLON5-IgG | Parkinsonism, parasomnias |
| ELAVL (Hu) | ANNA-1 | Brainstem encephalitis, ataxia |
| NOVA 1, 2 (Ri) | ANNA-2 | Jaw dystonia, OMS |
| PNMA1, PNMA2 (Ma1, Ma2) | Ma1, Ma2 | Parkinsonism, brainstem encephalitis
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| CDR2 | PCA-1 | Cerebellar ataxia, brainstem encephalitis |
| Unknown | PCA-2 | Ataxia, brainstem encephalitis |
| DNER | PCA-Tr | Cerebellar ataxia |
| CRMP-5 | CRMP-5 IgG | Chorea, ataxia |
| Amphiphysin | Amphiphysin IgG | Stiff-person phenomena |
| ZIC4 | ZIC4 antibody | Cerebellar ataxia |