Polycythemia vera complicated by chorea: A case report and the review of Chinese and international literature

Raza, Hafiz Khuram; Chansysouphanthong, Thitsavanh; Singh, Sonali; Amir, Achiya; Raza, M.W.; Zhang, Z.; Cui, Guiyun; Chen, Hao

doi:10.1016/j.neurol.2020.11.013

Cited by 3 publications

(2 citation statements)

References 15 publications

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“…In these cases, as a direct result of the increase in cells, there is an increased likelihood of potential blockage events within the vascular spaces of the individual. In PV, neurological sequelae have been commonly reported secondary to this pathophysiology [9][10][11][12]. Therefore, in the case of the patient, while he may have had all the key findings for a classical Bell's palsy, this pertinent piece of pathophysiological disease state required that the team pursue further work up to ensure that this was not a microvascular insult of one of the pontine perforators.…”

Section: Jak2 Mutations and Neurological Sequelaementioning

confidence: 99%

Cranial nerve VII on gadolinium contrast-enhanced magnetic resonance imaging in the case of Bell’s palsy

Alvarez,

Becher,

Varkey

et al. 2023

Exploration of Medicine

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Bell’s palsy is a rapid unilateral peripheral paralysis of cranial nerve VII whose etiology is varied, most commonly associated with an acute infectious or inflammatory demyelinating process. Nerve injury can result in edema because of increased permeability of vascular structures, which can sometimes be seen as a locus of enhancement of magnetic resonance imaging (MRI). Bell’s palsy is typically considered a clinical diagnosis and the specificity and sensitivity of imaging have been poorly studied. Herein is describe a case of a 73-year-old male who presented to the emergency department with left-sided facial droop and no other focal neurological abnormalities. With a history of a Janus kinase 2 (JAK2) mutation and the new initial facial drooping, acute cerebrovascular insult was high on the differential. Initial labs and computerized tomography (CT) head were inconclusive, but MRI showed pronounced enhancement of the left distal internal carotid artery (ICA) with contiguous enhancement of the labyrinthine, geniculate, and tympanic segments of the left facial nerve. Diagnosing Bell’s palsy can be a challenge as there are numerous postulated etiologies stemming from trauma, infection, and neoplasm; with infection (particularly viral) postulated to be the most likely source. Though MRI is currently not validated as a tool in expediting Bell’s palsy diagnosis, findings such as the enhancement seen here provide some insight into the benefit of MRI as a diagnostic modality in some cases. This case is unique both for the diagnostic dilemma between stroke and Bell’s palsy and the potential for MRI imaging to help guide clinical decision-making into treatment.

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Section: Jak2 Mutations and Neurological Sequelaementioning

confidence: 99%

Cranial nerve VII on gadolinium contrast-enhanced magnetic resonance imaging in the case of Bell’s palsy

Alvarez,

Becher,

Varkey

et al. 2023

Exploration of Medicine

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“…Неврологические симптомы могут быть начальными проявлениями ИП либо служить признаком прогрессирования гематологического заболевания [8]. В литературе описаны немногочисленные случаи развития хореи на фоне ИП (преимущественно у лиц пожилого возраста) [9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24]. Двигательные проявления в данных случаях были описаны как генерализованные, беспорядочные, непроизвольные движения с преимущественным вовлечением верхних конечностей и орофациальной мускулатуры, в некоторых случаях описано развитие левосторонней гемихореи.…”

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A case of chorea with cognitive impairment associated with polycythemia vera

Shpilyukova,

Smetanina,

Kolpina

et al. 2023

RJTAO

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Polycythemia vera (PV) is a clonal myeloproliferative disorder that is often associated with neurological symptoms. Rare manifestations of PV may include movement disorders (mainly chorea) and cognitive impairment (CI), which are fully or partially reversible with appropriate treatment. We present a case of chorea with CI in a 69-year-old man with a confirmed с1849G>T (V617F) mutation in the JAK2 gene with no vascular pathology on neuroimaging. In this patient, there is a clear correlation between the regression of the movement disorders with therapeutic phlebotomy and the start of treatment with hydroxyurea, which allows the conclusion that chorea has the secondary form. The cognitive symptoms remained at the same level after the start of treatment and during the one-year follow-up and did not develop further. In view of the patient's age, a concomitant neurodegenerative disease cannot be ruled out. In elderly patients with new-onset chorea and CI, it is therefore important to consider PV as a possible cause of these disorders, as early diagnosis of this condition allows timely initiation of effective treatment and prevention of the development of complications.

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Ischemic penumbra-induced hemichorea complicated by polycythemia vera: Promotion of thalamic disinhibition

Lee,

Jung,

Kwag

et al. 2024

Neurol Sci

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Polycythemia vera complicated by chorea: A case report and the review of Chinese and international literature

Cited by 3 publications

References 15 publications

Cranial nerve VII on gadolinium contrast-enhanced magnetic resonance imaging in the case of Bell’s palsy

Cranial nerve VII on gadolinium contrast-enhanced magnetic resonance imaging in the case of Bell’s palsy

A case of chorea with cognitive impairment associated with polycythemia vera

Ischemic penumbra-induced hemichorea complicated by polycythemia vera: Promotion of thalamic disinhibition

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