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September 11, 2018; 91 (11) Resident & Fellow Section

Teaching Video NeuroImages: A treatable rare cause of chorea

Malco Rossi, Angel Cammarota, Marcelo Merello, Martin Nogues
First published September 10, 2018, DOI: https://doi.org/10.1212/WNL.0000000000006160
Malco Rossi
From the Movement Disorders Section (M.R., A.C., M.M.), Neurology Department (M.N.), Raúl Carrea Institute for Neurological Research (FLENI); and Argentine National Scientific and Technological Research Council (CONICET) (M.M.), Buenos Aires, Argentina.
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Angel Cammarota
From the Movement Disorders Section (M.R., A.C., M.M.), Neurology Department (M.N.), Raúl Carrea Institute for Neurological Research (FLENI); and Argentine National Scientific and Technological Research Council (CONICET) (M.M.), Buenos Aires, Argentina.
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Marcelo Merello
From the Movement Disorders Section (M.R., A.C., M.M.), Neurology Department (M.N.), Raúl Carrea Institute for Neurological Research (FLENI); and Argentine National Scientific and Technological Research Council (CONICET) (M.M.), Buenos Aires, Argentina.
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Martin Nogues
From the Movement Disorders Section (M.R., A.C., M.M.), Neurology Department (M.N.), Raúl Carrea Institute for Neurological Research (FLENI); and Argentine National Scientific and Technological Research Council (CONICET) (M.M.), Buenos Aires, Argentina.
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Teaching Video NeuroImages: A treatable rare cause of chorea
Malco Rossi, Angel Cammarota, Marcelo Merello, Martin Nogues
Neurology Sep 2018, 91 (11) e1089; DOI: 10.1212/WNL.0000000000006160

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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

Reference

  1. 1.↵
    1. Bruyn GW,
    2. Padberg G
    . Chorea and polycythaemia. Eur Neurol 1984;23:26–33.
    OpenUrlPubMed

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