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Moyamoya syndrome in hemoglobin E-beta thalassemia: A rare presentation and association.
J Postgrad Med 2018 Oct-Dec; 64(4):240-242JP

Abstract

Moyamoya disease is an idiopathic, nonatherosclerotic, noninflammatory, chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis, typically the supraclinoid internal carotid arteries, followed by extensive collateralization, which are prone to thrombosis, aneurysm, and hemorrhage. Secondary moyamoya phenomenon or moyamoya syndrome (MMS) occurs in a wide range of clinical scenarios including prothrombotic states such as sickle cell anemia, but the association with other hemoglobinopathies is less frequently observed. We describe a case of a 25-year-old female with hemoglobin E-beta thalassemia who had a rare presentation of MMS in the form of choreoathetoid movements in the left upper and lower extremities. We describe this association, primarily to emphasize thalassemia as an extremely rare but a potential etiology of MMS. Since MMS is a progressive disease, it is important to diagnose and initiate treatment to prevent worsening of the disease and recurrence of stroke.

Authors+Show Affiliations

Department of Pediatrics, Nanavati Super Speciality Hospital, Mumbai, Maharashtra, India.Department of Radiology, Nanavati Super Speciality Hospital, Mumbai, Maharashtra, India.Department of Radiology, Nanavati Super Speciality Hospital, Mumbai, Maharashtra, India.Department of Pediatrics, Nanavati Super Speciality Hospital, Mumbai, Maharashtra, India.

Pub Type(s)

Case Reports

Language

eng

PubMed ID

29943740

Citation

Doctor, P N., et al. "Moyamoya Syndrome in Hemoglobin E-beta Thalassemia: a Rare Presentation and Association." Journal of Postgraduate Medicine, vol. 64, no. 4, 2018, pp. 240-242.
Doctor PN, Choudhari A, Verma M, et al. Moyamoya syndrome in hemoglobin E-beta thalassemia: A rare presentation and association. J Postgrad Med. 2018;64(4):240-242.
Doctor, P. N., Choudhari, A., Verma, M., & Merchant, R. H. (2018). Moyamoya syndrome in hemoglobin E-beta thalassemia: A rare presentation and association. Journal of Postgraduate Medicine, 64(4), pp. 240-242. doi:10.4103/jpgm.JPGM_468_17.
Doctor PN, et al. Moyamoya Syndrome in Hemoglobin E-beta Thalassemia: a Rare Presentation and Association. J Postgrad Med. 2018;64(4):240-242. PubMed PMID: 29943740.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Moyamoya syndrome in hemoglobin E-beta thalassemia: A rare presentation and association. AU - Doctor,P N, AU - Choudhari,A, AU - Verma,M, AU - Merchant,R H, PY - 2018/6/27/pubmed PY - 2018/6/27/medline PY - 2018/6/27/entrez KW - Choreoathetoid movement KW - Moyamoya syndrome KW - hemoglobin E-beta thalassemia SP - 240 EP - 242 JF - Journal of postgraduate medicine JO - J Postgrad Med VL - 64 IS - 4 N2 - Moyamoya disease is an idiopathic, nonatherosclerotic, noninflammatory, chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis, typically the supraclinoid internal carotid arteries, followed by extensive collateralization, which are prone to thrombosis, aneurysm, and hemorrhage. Secondary moyamoya phenomenon or moyamoya syndrome (MMS) occurs in a wide range of clinical scenarios including prothrombotic states such as sickle cell anemia, but the association with other hemoglobinopathies is less frequently observed. We describe a case of a 25-year-old female with hemoglobin E-beta thalassemia who had a rare presentation of MMS in the form of choreoathetoid movements in the left upper and lower extremities. We describe this association, primarily to emphasize thalassemia as an extremely rare but a potential etiology of MMS. Since MMS is a progressive disease, it is important to diagnose and initiate treatment to prevent worsening of the disease and recurrence of stroke. SN - 0972-2823 UR - https://www.unboundmedicine.com/medline/citation/29943740/Moyamoya_syndrome_in_hemoglobin_E-beta_thalassemia:_A_rare_presentation_and_association L2 - http://www.jpgmonline.com/article.asp?issn=0022-3859;year=2018;volume=64;issue=4;spage=240;epage=242;aulast=Doctor DB - PRIME DP - Unbound Medicine ER -