Objective: To analyze the etiology and clinical characteristics of patients with non-structural hemichorea treated in two neurology departments.

Background: Hemichorea was classically associated with contralateral structural lesions of the basal ganglia. However, in the last few years, the metabolic, immunological and even pharmacological causes have gained relevance.

Method: A retrospective analysis of clinical records of patients with non-structural hemichorea evaluated between 2016 and 2021 in two neurology departments.

Results: 9 patients with hemichorea were evaluated (mean age (SD), 67.8 (12.6) years), 5 of them were female. Among the causes we found that 4 were secondary to hyperosmolar non-ketotic hyperglycemia (HNKH), 4 immune-mediated etiology and 1 Polycythemia Vera (PV). Regarding HNKH, 2 were women, with a mean age of 69.75 (SD 13.5), 3 of them had a history of Diabetes and in 1 case hemichorea presented as a diabetic debut. Of the immune-mediated, 2 were associated with Antiphospholipid Syndrome (APS) and 2 with paraneoplastic antibodies (Anti-Tr (DNER) and Anti-CASPR-2). There was no association with cancer detection in these last two cases. The presentation age in the PV patient was 78 years.
In 7 patients the onset was in the right hemibody and 2 in the left, the presentation was subacute in all the patients. The patient with CASPR-2 evolved with progression of symptoms, probably due to the delay in the initiation of immunosuppressive treatment. Brain MRI was abnormal in 22.3% of patients, all of them with HNKH.
Patients with immune-mediated etiology resolved the condition after the administration of corticosteroids and the 2 cases of APS relapsed. Those with HNKH remitted with glycemic control, except for one patient with poor adherence to treatment. Phlebotomy was the treatment of PV with good outcome.

Conclusion: In our series, one of the most frequent etiologies was HNKH, which must be considered even in the absence of a previous diagnosis of Diabetes. Chorea of ​​immune-mediated origin had a very good response to treatment with corticosteroids; however both patients with APS presented minor relapses. In patient with suspect of autoimmune etiology, immunosuppressive treatment should not be delayed.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/non-structural-hemichorea-report-of-nine-cases/