Moyamoya disease: Treatment and prognosis
- Nijasri Charnnarong Suwanwela, MD
Nijasri Charnnarong Suwanwela, MD
- Professor of Neurology
- Chulalongkorn University Medical School
- Section Editors
- Jose Biller, MD, FACP, FAAN, FAHA
Jose Biller, MD, FACP, FAAN, FAHA
- Section Editor — Stroke
- Professor of Neurology and Neurological Surgery
- Chairman Department of Neurology
- Loyola University Chicago
- Stritch School of Medicine
- Douglas R Nordli, Jr, MD
Douglas R Nordli, Jr, MD
- Section Editor — Pediatric Neurology
- Chief of Neurology
- Children’s Hospital Los Angeles
- Vice Chair of Neurology
- USC Keck School of Medicine
Moyamoya disease is a unique chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation.
This topic will review the prognosis and treatment of moyamoya. Other aspects of the disease are discussed separately. (See "Moyamoya disease: Etiology, clinical features, and diagnosis".)
For children and adults with moyamoya and acute stroke, acute treatment is mainly symptomatic and directed towards reducing elevated intracranial pressure, improving cerebral blood flow, and controlling seizures . Ventricular drainage and/or hematoma removal is often required for patients with intracerebral hemorrhage.
During hospitalization for acute stroke or surgery, the following management issues deserve special attention, especially in children with moyamoya :
●Precautions to minimize crying and hyperventilation: both can lower PaCO2 and thereby induce or worsen cerebral ischemia by causing vasoconstriction [2,3].
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