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Reversible abnormal functional neuroimaging presentations in polycythemia vera with chorea.

Hui-Chun Huang¹, Yu-Chin Wu, Lee-Yung Shih

¹Department of Neurology, China Medical University Hospital, 404 Taichung, Taiwan.

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|May 12, 2011

Summary

Polycythemia vera with chorea showed altered brain metabolism and dopamine function. These changes normalized after treatment, suggesting a reversible link between polycythemia vera, chorea, and dopaminergic pathways.

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Area of Science:

Neuroscience
Hematology
Nuclear Medicine

Background:

Polycythemia vera is a myeloproliferative neoplasm characterized by increased red blood cell mass.
Chorea is a movement disorder characterized by involuntary, irregular, semi-rhythmic movements.
The interplay between polycythemia vera and neurological manifestations like chorea is not fully understood.

Observation:

A patient with polycythemia vera presented with chorea.
Brain metabolism was assessed using 2-[(18)F]fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET).
Dopamine transporter availability was evaluated using (99m)Tc-labeled tropane dopamine transporter ((99m)Tc-TRODAT-1) single photon emission computed tomography (SPECT).

Findings:

FDG PET revealed altered brain metabolism, particularly in the corticobasal ganglia.
(99m)Tc-TRODAT-1 SPECT indicated disturbed dopaminergic function.
Following phlebotomy and normalization of hematocrit and clinical symptoms, both FDG PET and (99m)Tc-TRODAT-1 SPECT imaging returned towards normal.

Implications:

The findings suggest that polycythemia vera-associated chorea may stem from reversible alterations in corticobasal ganglia metabolism.
Dopaminergic system dysfunction is implicated in the pathophysiology of this condition.
This case highlights the potential utility of advanced neuroimaging in understanding and managing neurological complications of hematological disorders.