BMJ Case Reports 2018; doi:10.1136/bcr-2017-223867
  • Unusual presentation of more common disease/injury

Sudden hemichorea and frontal lobe syndrome: a rare presentation of unbalanced polycythaemia vera

  1. Valentín Mateos
  1. Department of Neurology, Centro Medico de Asturias, Oviedo, Asturias, Spain
  1. Correspondence to Carmen García-Cabo, c.garciacabo{at}
  • Accepted 2 May 2018
  • Published 14 May 2018


Polycythaemia vera (PV) is an haematological neoplasm that frequently presents neurological symptoms. However, chorea is a rare complication of this disease, occurring in less than 5% of the patients. Cognitive impairment related to PV unbalanced is also a rare complication, and it can improve with proper treatment. We present a 96-year-old-man with acute-onset hemichorea and frontal lobe syndrome with no vascular pathology in the basal ganglia or frontal region. A clear relationship was observed between the onset of involuntary movements and the cognitive impairment and worsening of haematological parameters in the patient. After causal and symptomatic treatment, the patient’s clinical status improved. In the elderly, PV must be considered as a cause of acute chorea and sudden cognitive impairment, as early diagnosis leads to effective treatment and prevention of complications.


  • Contributors CG-C: conception or design of the work, data collection, data analysis and interpretation, drafting the article. JF-D: critical revision of the article. VM: final approval of the version to be published.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

Register for free content

The full text of all Editor's Choice articles and summaries of every article are free without registration

The full text of Images in ... articles are free to registered users

Only fellows can access the full text of case reports (apart from Editor's Choice) - become a fellow today, or encourage your institution to, so that together we can grow and develop this resource

Don't forget to sign up for content alerts so you keep up to date with all the case reports as they are published, and let us know what you think by commenting on the Editor's blog

Navigate This Article