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Polycythemia can cause chorea, a movement disorder, predominantly in women over 50. This condition, polycythemic chorea, is linked to blood viscosity and brain metabolism, not caudate nucleus infarcts.
Area of Science:
- Neurology
- Hematology
- Movement Disorders
Background:
- Chorea is a neurological disorder characterized by involuntary, irregular, dance-like movements.
- Polycythemia is a condition where the body makes too many red blood cells, increasing blood viscosity.
Purpose of the Study:
- To analyze the characteristics of chorea as a symptom of polycythemia.
- To investigate the demographic, clinical, and potential pathophysiological aspects of polycythemic chorea.
Main Methods:
- Retrospective analysis of 35 cases of chorea associated with polycythemia.
- Clinical observation and assessment of patient demographics, symptom presentation, and treatment responses.
Main Results:
- Polycythemic chorea predominantly affects females over 50 (4:1 female:male ratio).
- Symptoms include generalized chorea, faciolingual and brachial muscle involvement, and hypotonia.
- Treatment response varies, with haloperidol, venesection, or 32P-therapy showing efficacy, though spontaneous remission or recurrence can occur.
Conclusions:
- Polycythemia should be considered in elderly patients presenting with chorea, especially 'senile' chorea.
- The underlying mechanism is hypothesized to be a neostriatal hyperviscosity syndrome affecting brain metabolism.
- Further research is needed to confirm the role of dopamine and platelets in polycythemic chorea.
Abstract:
An analysis is presented of 35 cases of chorea as a symptom of polycythaemia. This analysis reveals: (a) whereas polycythaemia occurs predominantly in males (3:2), polycythaemic chorea (PC) occurs predominantly in females (5:2), at a real ratio of female:male = 4:1, the prevalence being 1-2.5% of polycythaemic patients; (b) PC manifests predominantly after the age of 50 (8 cases before, 27 after 50 years), making polycythaemia the first disorder to be considered in cases of so-called 'senile' chorea; (c) PC is generalised, with predominant involvement of faciolingual and brachial muscles, and associated with muscular hypotonia; (d) PC may last from periods of weeks to years, usually responds to haloperidol, venesection or 32P-treatment, but may persist, or recur with treatment, or remit spontaneously, and (e) no relationship exists between the choreatic syndrome and (the rare finding of) a small infarct in the caudate nucleus. The cause of the choreatic syndrome in polycythaemia is presumably to be explained as a neostriatal hyperviscosity syndrome producing venous stasis, reduced brain blood flow and impaired tissular O2/glucose metabolism. The state of dopaminergic hyperactivity is presumably enhanced by relatively increased neostriatal catecholestrogens. The hypothesis of polycythaemic excess of dopamine-laden platelets releasing excess of dopamine in the neostriatum needs to be confirmed by laboratory evidence of platelet counts.