Shahedah, KK;
Di Lazzaro, G;
Magrinelli, F;
Mulroy, E;
Latorre, A;
Bhatia, KP;
(2021)
Late‐Onset Chorea in JAK2‐Associated Essential Thrombocythemia.
Movement Disorders Clinical Practice
, 8
(1)
pp. 145-148.
10.1002/mdc3.13105.
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Abstract
Somatic mutations in JAK2 are associated with chronic myeloproliferative neoplasms (MPNs), including polycythemia vera, essential thrombocythemia, and primary myelofibrosis (PMF). The most common variant, Val617Phe (JAK2V617F), accounts for >95% of polycythemia vera and 50%–60% of essential thrombocythemia and PMF cases.1, 2 Chorea is a well‐described neurological complication of polycythemia vera,3 encompassing acute‐onset occasionally reversible hemichorea and slowly progressive generalized chorea with prominent oromandibular involvement.3 The former usually has a vascular etiology, secondary to thrombosis, hemorrhage, or hyperviscosity, whereas the latter has a poorly elucidated pathophysiology.3 Here, we describe a case of late‐onset generalized chorea with prominent oromandibular involvement associated with JAK2V617F‐positive essential thrombocythemia (rather than polycythemia vera) and speculate on its pathophysiology.
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