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Chorea and polycythaemia vera.

Catriona Gribbin1, Arshi Yasmin2, Paul Gallagher3

  • 1Department of Neurology, Institute of Neurological Sciences, Glasgow, UK catriona.gribbin2@nhs.scot.

Practical Neurology
|October 27, 2023
PubMed
Summary
This summary is machine-generated.

Polycythaemia vera can cause late-onset chorea even with normal blood counts. Early diagnosis of this treatable condition is crucial for patients experiencing new choreiform movements.

Keywords:
HAEMATOLOGYMOVEMENT DISORDERS

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Area of Science:

  • Neurology
  • Hematology
  • Internal Medicine

Background:

  • Chorea is a hyperkinetic movement disorder characterized by involuntary, irregular, and purposeless movements.
  • Polycythaemia vera (PV) is a myeloproliferative neoplasm characterized by increased red blood cell mass, often associated with thrombotic and hemorrhagic complications.
  • Neurological manifestations of PV can occur, but chorea is an uncommon presentation, especially when hematocrit and hemoglobin levels are within normal limits.

Purpose of the Study:

  • To report two cases of late-onset chorea associated with polycythaemia vera.
  • To highlight the importance of considering polycythaemia vera in the differential diagnosis of new-onset chorea, even in the absence of overt polycythemia.
  • To emphasize the treatable nature of polycythaemia vera and the potential for neurological improvement with appropriate management.

Main Methods:

  • Case report of two patients presenting with new-onset chorea.
  • Review of clinical history, neurological examination findings, and laboratory results, including complete blood count and hematocrit.
  • Diagnostic workup for secondary causes of chorea.

Main Results:

  • Two patients developed chorea with hematocrit and hemoglobin levels within the normal reference range.
  • Diagnosis of polycythaemia vera was confirmed in both patients despite the absence of elevated red blood cell counts.
  • Treatment of polycythaemia vera led to improvement or resolution of choreiform movements in the reported cases.

Conclusions:

  • Polycythaemia vera should be considered in the differential diagnosis of late-onset chorea, irrespective of hematocrit and hemoglobin levels.
  • Early recognition and treatment of polycythaemia vera can prevent serious complications and potentially reverse neurological symptoms like chorea.
  • This presentation underscores the diverse and sometimes subtle neurological manifestations of polycythaemia vera.