A clinical case of cerebellar ataxia with neuropathy and vestibular areflexia syndrome in the presence of polycythemia vera
https://doi.org/10.14412/2074-2711-2019-3-74
Abstract
Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) is a slowly progressive ataxic disorder characterized by the triad of cerebellar impairment, bilateral vestibular hypofunction, and sensory polyneuropathy. The paper describes a clinical case of this syndrome in a 40-year-old patient who has been followed up by hematologists for polycythemia vera for 10 years.
About the Authors
Sh. M. Safin
Bashkir State Medical University, Ministry of Health of Russia
Russian Federation
3, Lenin St., Ufa 450008
Russian Federation
3, Lenin St., Ufa 450008
B. A. Bakirov
Bashkir State Medical University, Ministry of Health of Russia
Russian Federation
3, Lenin St., Ufa 450008
Russian Federation
3, Lenin St., Ufa 450008
Kh. P. Derevyanko
Bashkir State Medical University, Ministry of Health of Russia
Russian Federation
3, Lenin St., Ufa 450008
Russian Federation
3, Lenin St., Ufa 450008
D. E. Baikov
Bashkir State Medical University, Ministry of Health of Russia
Russian Federation
3, Lenin St., Ufa 450008
Russian Federation
3, Lenin St., Ufa 450008
M. M. Khafizov
Bashkir State Medical University, Ministry of Health of Russia
Russian Federation
3, Lenin St., Ufa 450008
Russian Federation
3, Lenin St., Ufa 450008
References
1. Szmulewicz D, McLean C, MacDougall H, et al. CANVAS an update: Clinical presentation, investigation and management. J Vestib Res. 2014;24(5-6):465-74. doi: 10.3233/VES140536.
2. Taki M, Nakamura T, Matsuura H, et al. Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS). Auris Nasus Larynx. 2018 Aug;45(4):866-870. doi: 10.1016/j.anl.2017.10.008. Epub 2017 Oct 28.
3. Pal'chun VT, Guseva AL, Chistov SD, et al. Otoneurological examination of a patient with dizziness. Vestnik otorinolaringologii. 2015;(5):60-6. (In Russ.).
4. Szmulewicz DJ, McLean CA, Rodriguez ML, et al. Dorsal root ganglionopathy is responsible for the sensory impairment in CANVAS. Neurology. 2014 Apr 22;82(16):1410-5. doi: 10.1212/WNL.0000000000000352. Epub 2014 Mar 28.
5. Umeh C, Polydefkis M, Chaudhry V, et al. Sweat Gland Denervation in Cerebellar Ataxia with Neuropathy and Vestibular Areflexia Syndrome (CANVAS). Mov Disord Clin Pract. 2016 Apr 5;4(1):46-48. doi: 10.1002/mdc3.12355. eCollection 2017 Jan-Feb.
6. Cazzato D, Bella ED, Dacci P, et al. Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome: a slowly progressive disorder with a stereotypical presentation. J Neurol. 2016 Feb;263(2):245-249. doi: 10.1007/s00415-015-7951-9. Epub 2015 Nov 14.
7. Stradnikov VA, Fisenko EP, editors. Normal'naya ul'trazvukovaya anatomiya vnutrennikh organov i poverkhnostno raspolozhennykh struktur [Normal ultrasound anatomy of internal organs and surface structures]. Moscow: Strom; 2012.
8. Teregulova DR, Bakirov BA, Baikov DE, et al. Neurological complications of chronic lymphocytic leukemia in adults. Nevrologicheskii vestnik imeni V.M. Bekhtereva. 2015;47(3):59-66. (In Russ.).
9. Savchengo VG, redaktor. Algoritmy diagnostiki i protokoly lecheniya zabolevanii sistemy krovi [Algorithms of diagnostics and protocols of treatment of diseases of the blood system]. Volume 1. Moscow: Praktika; 2018. 196 p.
10. Migliaccio AA, Halmagyi GM, McGarvie LA, et al. Cerebellar ataxia with bilateral vestibulopathy: description of a syndrome and its characteristic clinical sign. Brain. 2004 Feb;127(Pt 2):280-93. Epub 2003 Nov 7.
11. Szmulewicz DJ, Waterston JA, MacDougall HG, et al. Cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS): a review of the clinical features and video-oculographic diagnosis. Ann N Y Acad Sci. 2011 Sep;1233:139-47. doi: 10.1111/j.1749-6632.2011.06158.x.
12. Davies R. Bedside neuro-otological examination and interpretation of commonly used investigations. J Neurol Neurosurg Psychiatry. 2004 Dec;75 Suppl 4:iv32-44.
13. Guseva SA, Bessmel'tsev SS, Abdulkadyrov KM, et al. Istinnaya politsitemiya [True polycythemia]. Saint-Petersburg: Logos; 2009. 405 p.
14. Demidova AV, Kotsyubinskii NN, Mazurov VI. Eritremiya i vtorichnye eritrotsitozy [Erythremia and secondary erythrocytosis]. Saint-Petersburg: SPbMAPO; 2001. 228 p.
15. Maffioli M, Mora B, Passamonti F, et al. Polycythemia vera: from new, modified diagnostic criteria to new therapeutic approaches. Clin Adv Hematol Oncol. 2017 Sep;15(9):700-707.
16. Ania BJ, Suman VJ, Sobell JL, et al. Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935–1989. Am J Hematol. 1994 Oct;47(2):89-93.
17. McMullin MF. The classification and diagnosis of erythrocytosis. Int J Lab Hematol. 2008 Dec;30(6):447-59. doi: 10.1111/j.1751-553X.2008.01102.x. Epub 2008 Sep 23.
18. Marchioli R, Finazzi G, Landolfi R, et al. Vascular and Neoplastic Risk in a Large Cohort of Patients With Polycythemia Vera. J Clin Oncol. 2005 Apr 1;23(10):2224-32. Epub 2005 Feb 14.
19. Passamonti F, Malabarba L, Orlandi E, et al. Polycythemia vera in young patients: a study on the long-term risk of thrombosis, myelofibrosis, and leukemia. Haematologica. 2003 Jan;88(1):13-8.
20. Cervantes F, Passamonti F, Barosi G. Life expectancy and prognostic factors in the classic BCR. ABL-negative myeloproliferative disorders. Leukemia. 2008 May;22(5):905-14. doi: 10.1038/leu.2008.72. Epub 2008 Apr 3.
21. Passamonti F, Griesshammer M, Palandri F. Ruxolitinib for the treatment of inadequately controlled polycythaemia vera without splenomegaly (RESPONSE-2): a randomised, open-label, phase 3b study. Lancet Oncol. 2017 Jan;18(1):88-99. doi: 10.1016/S1470-2045(16) 30558-7. Epub 2016 Dec 2.
22. Gerds AT, Dao KH. Polycythemia Vera Management and Challenges in the Community Health Setting. Oncology. 2017; 92(4):179-189. doi: 10.1159/000454953. Epub 2017 Jan 18.
23. Tefferi A, Barbui T. Polycythemia vera and essential thrombocythemia: 2017 update on diagnosis, risk-stratification, and management. Am J Hematol. 2017 Jan;92(1):94-108. doi: 10.1002/ajh.24607.
Review
For citations:
Safin SM, Bakirov BA, Derevyanko KP, Baikov DE, Khafizov MM. A clinical case of cerebellar ataxia with neuropathy and vestibular areflexia syndrome in the presence of polycythemia vera. Nevrologiya, neiropsikhiatriya, psikhosomatika = Neurology, Neuropsychiatry, Psychosomatics. 2019;11(3):74-77. https://doi.org/10.14412/2074-2711-2019-3-74
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