A 72-year-old woman developed progressive choreic movements involving the right hemibody and the orofacial muscles (video 1). Her examination was otherwise unremarkable, without ataxia. Brain MRI, blood glucose, thyroid function, and rheumatologic panel were unremarkable. Genetic testing for Huntington disease and C9orf72 expansions were negative. Initial hematocrit and hemoglobin levels were normal, but increased to abnormal values (56% and 18.5 g/dL) 1 year after chorea onset. The JAK2V617F mutation was positive, suggesting a diagnosis of chorea due to polycythemia vera. Chorea disappeared with phlebotomy and hydroxyurea. Neurologists should suspect this rare treatable entity in elderly patients with chorea even with initially normal hematocrit levels.1
Video 1
Patient shows predominately right hemichorea and subtle involvement of orofacial muscles due to polycythemia vera that resolve completely after treatment with phlebotomy and hydroxyurea.Download Supplementary Video 1 via http://dx.doi.org/10.1212/006160_Video_1
Author contributions
Malco Rossi: study concept and design, acquisition, analysis, and interpretation of data, draft of manuscript. Angel Cammarota: study concept and design, acquisition, analysis, and interpretation of data, draft of manuscript. Marcelo Merello: study concept and design, interpretation of data, critical revision of manuscript for intellectual content. Martin Nogues: study concept and design, interpretation of data, critical revision of manuscript for intellectual content.
Study funding
No targeted funding reported.
Disclosure
The authors report no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.
Footnotes
Teaching slides links.lww.com/WNL/A663
- © 2018 American Academy of Neurology
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