Moyamoya Disease: A Rare Sickle Cell Trait Neurological Complication

Introduction: Sickle cell disease is an inherited blood disorder with multisystem complications, including cerebrovascular accidents primarily due to micro infarcts. However, people with sickle cell trait, the heterozygous form of sickle cell disease, usually lead a normal, healthy life. Very rarely, severe tissue hypoxia, acidosis, dehydration, and hypothermia in sickle cell trait increase red blood cell polymerization and sickling, which could lead to micro infarcts in blood vessels. Moyamoya disease is a rare neurovascular complication caused by repeated strokes after arterial cerebral infarction. It commonly manifests as ischemic stroke in young patients, but mainly as intracranial hemorrhage in adults. Case presentation: A 14-year-old girl with sickle cell trait, who had been living a normal and healthy life, started to complain of severe persisting headache, migraine, drowsiness, and a tendency to sleep. Brain magnetic resonance imaging and angiography revealed moyamoya disease. Hematological treatment with hydroxyurea, aspirin, and an NSAID was commenced for this patient. Conclusion: Under rare circumstances, sickle cell trait could lead to sickle cell disease complications. Although moyamoya has been seen in sickle cell disease, it is a very rare neurological complication of sickle cell trait. To the best of our knowledge, only three cases of moyamoya have been reported in individuals with sickle cell trait. Hassan Al-Jafar1*, Khaled Hashem2, Ali Abo Alhasan3, Shashishekhar Lamdhade4, Salma AlDallal1 and Fayza Abdulla Alhajri2 1Department of Haematology, Amiri Hospital, Gulf street, Kuwait City, Kuwait 2Department of Radiology, Amiri Hospital, Gulf street, Kuwait City, Kuwait 3Department of Neurosurgery, Ibn Sina Hospital, Sabah Medical Region, Kuwait 4Department of Neurology, Amiri Hospital, Gulf street, Kuwait City, Kuwait *Address for Correspondence Hassan Al-Jafar, Department of Haematology, Amiri Hospital, Gulf street, Kuwait city, Kuwait, E-mail: [email protected] Submission: 17 November 2015 Accepted: 21 January 2016 Published: 26 January 2016 Copyright: © 2016 Al-Jafar H, et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Reviewed & Approved by: Dr. Jennifer Larimore, Department of Biology and Neuroscience Program, Agnes Scott College, USA Case Report Open Access