Affiliations: Department of Neurology, Division of Neurophysiology and Neurocritical Care Children’s National Medical Center, Washington, DC, USA | Department of Pediatrics, Division of Hematology and Oncology, Washington University School of Medicine, St. Louis, MO, USA | Department of Hematology, Children’s National Medical Center, Washington, DC, USA
Note: [] Corresponding author: Jessica L Carpenter, Department of Neuroscience, Children’s National Medical Center, 111 Michigan Ave NW, Washington, DC 20010 USA. Tel.: +1 202 476 2120; Fax: +1 202 2864; E-mail: jcarpent@childrensnational.org.
Abstract: Moyamoya, a pattern of progressive stenosis of the ICA associated with secondary formation of collateral vessels at the base of the brain, is a rare but well described cerebral vasculopathy. Moyamoya can be idiopathic but is also associated with several genetic disorders, inflammatory conditions and radiation exposure. Despite extensive research on the topic, the underlying mechanisms for how and why Moyamoya develops are not well understood. Moyamoya syndrome in sickle cell disease is one of the more studied subsets of the disease and recent experience with this subtype may have implications for understanding of all types of Moyamoya. Treatment options for Moyamoya are limited, with the mainstays of therapy restricted to surgical revascularization procedures.
