Chorea and a Frontal Lobe Syndrome: A Rare Neurological Presentation of Polycythemia Vera; A Case Report

Severs, Mirjam; Boelens, Hendrik M.; Diraoui, Shiraz B.; Schuur, Jacqueline

doi:10.1111/j.1532-5415.2011.03850.x

Cited by 10 publications

(9 citation statements)

References 6 publications

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“…It is promising that each of these reports has shown at least partial, subjective reversibility of the cognitive deficits. In addition, in our case as well as the patient with frontal lobe syndrome described by Severs et al [19] in 2012, neuropsychological testing showed significant improvement after polycythemia vera treatment.…”

Section: Discussionsupporting

confidence: 77%

“…All these patients presented with cognitive impairment including confusion, psychomotor slowing, general behavioral changes, and frontal lobe syndrome. One of the patients, like the first case we presented, even met the criteria for clinical dementia [16][17][18][19][20]. It is promising that each of these reports has shown at least partial, subjective reversibility of the cognitive deficits.…”

Section: Discussionmentioning

confidence: 72%

“…Whereas, chorea is a rare but recognized symptom of polycythemia vera, neuropsychiatric symptoms have only been reported in 8 former published reports (shown in Table 2) [13][14][15][16][17][18][19][20]. The first reports describing psychiatric symptoms date from the early nineties, in which 3 patients were admitted with acute mania or depression [13][14][15].…”

Section: Discussionmentioning

confidence: 99%

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Neuropsychiatric Derangement by Polycythemia Vera: A Case Report of an Unexpected Disease Presentation and Review of the Literature

et al. 2021

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Cerebral infarction as well as other thromboses, headaches, and visual complaints are well-known symptoms of polycythemia vera. However, chorea and neuropsychiatric disturbances are less recognized consequences of this chronic disease. Whereas chorea is a rare but acknowledged symptom of polycythemia vera, neuropsychiatric symptoms have only sporadically been reported. We depict 2 patients with an unusual presentation of polycythemia vera. Our first patient presented with right-sided hemiballism and psychosis, and the second patient had a long diagnostic trajectory of unexplained chorea. In both cases diagnosis of JAK2 positive polycythemia vera was established, and in both cases remarkable recovery occurred after the initiation of phlebotomies. The underlying pathophysiology of these symptoms has not been clearly elucidated. Because of the unfamiliarity of the link between especially neuropsychiatric symptoms and polycythemia, current reported numbers are probably an underestimation. Benefit of treatment appears to be large. We seek to create more awareness among physicians about this phenomenon.

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Section: Discussionsupporting

confidence: 77%

Section: Discussionmentioning

confidence: 72%

Section: Discussionmentioning

confidence: 99%

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Neuropsychiatric Derangement by Polycythemia Vera: A Case Report of an Unexpected Disease Presentation and Review of the Literature

et al. 2021

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“…PV‐associated chorea is a treatable state that improves with the normalization of hematocrit . The duration of chorea before the consecutive phlebotomies was 5 years in the present case, which was much longer compared with a few days to 6 months in the previous reports. It is noteworthy that the chorea resolved completely as the hematocrit normalized despite the long duration.…”

Section: Discussionmentioning

confidence: 44%

Treatable chorea associated with polycythemia vera

Miyagawa

Ueda

Ohtomo

et al. 2014

Neurology & Clinical Neurosc

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We report a case of a 70-year-old woman with polycythemia vera and 5 years of persistent generalized chorea. Despite the long duration of the choreic condition, it completely resolved after the normalization of the hematocrit by serial phlebotomies. Diffuse cerebral hypoperfusion evaluated by 99m Tc-hexamethyl-propylene amine oxime (HMPAO) single-photon emission computed tomography (SPECT) improved in accordance with the intensive hematocrit control. We recommend an increased awareness of this rare, but treatable cause of chorea in elderly patients.

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“…For the rest, there was no information regarding the type of evaluation that was employed [1,4,12,14,27]. Two of the patients were found to have mild cognitive impairment, one had moderate impairment, and all three had PV [15,28,29].…”

Section: Reviewing the Available Literaturementioning

confidence: 99%

Chorea and Cognitive Impairment in JAK2V617F-Positive Myeloproliferative Disorders: A Case Report and Literature Review

Butnariu,

Antonescu-Ghelmez,

Moraru

et al. 2023

Medicina

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Chorea is a hyperkinetic movement disorder, accompanied by dystonia, myoclonus, tics, stereotypies, and tremors. It is characterized by excessive, purposeless movements that are distressing, irregularly timed, and randomly distributed. Chorea can be present in many diseases, such as hereditary, metabolic disturbance, drug-induced, and functional disorders, and, rarely, genetic, autoimmune, and infectious diseases. Primary myelofibrosis (PMF) is a myeloproliferative neoplasm that leads to ineffective clonal hematopoiesis, fibrous tissue deposits in the bone marrow, extramedullary hematopoiesis, and splenomegaly. In rare cases, following uncertain pathological mechanisms, it can present with chorea, particularly affecting the limbs, head, and orofaciolingual muscles. We present a case of a male patient with evolving PMF over several years who was admitted for progressive cognitive impairment and generalized involuntary movement disorder. We also present a review of all cases of myeloproliferative disorders presenting with chorea published in the last 40 years.

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Chorea and a Frontal Lobe Syndrome: A Rare Neurological Presentation of Polycythemia Vera; A Case Report

Cited by 10 publications

References 6 publications

Neuropsychiatric Derangement by Polycythemia Vera: A Case Report of an Unexpected Disease Presentation and Review of the Literature

Neuropsychiatric Derangement by Polycythemia Vera: A Case Report of an Unexpected Disease Presentation and Review of the Literature

Treatable chorea associated with polycythemia vera

Chorea and Cognitive Impairment in JAK2V617F-Positive Myeloproliferative Disorders: A Case Report and Literature Review

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