Ver el código de «Moyamoya»

==Background==
*A cerebrovascular disease characterized by progressive stenosis/occlusion of the arteries around the Circle of Willis. 
*The narrowing or blockage stimulates angiogenesis to provide collateral circulation to the brain. 
*On angiography, these small blood vessels give off a "puff of smoke" appearance (also known as "MoyaMoya" in Japanese). 
*The disease is found more commonly in Asian countries and was first descried in Japanese literature in 1957. 
*There is mounting evidence there is a genetic cause of the disease.

==Clinical Features==
*The developed collateral circulation is prone to bleeding, aneurysm, thrombosis exhibiting: [[focal neurological deficits]], [[TIA]], [[epilepsy]], and bowel/bladder incontinence. 
*Children predominately exhibit [[ischemic stroke]].
*Approximately 2/3rd of children exhibit Electroencephalography (EEG) abnormalities: high voltage slow waves that occur during hyperventilation. 
*Can be associated with atherosclerosis, [[meningitis]], [[vasculitis]], autoimmune diseases, hematological conditions, [[brain tumor]]s, and chromosomal abnormalities

==Differential Diagnosis==
*[[Hemorrhagic stroke]]/[[Ischemic stroke]]
*[[Giant cell arteritis]], [[vasculitis]]
*[[Head trauma]]
*Basilar artery thrombosis
*Blood dyscrasias 
*[[Cavernous sinus thrombosis]]
*Cerebral aneurysms
*Dissection syndromes (e.g. intracranial [[vertebral and carotid artery dissection]])
*Carotid atherosclerosis 
*Fibromuscular dysplasia
*Craniopharyngioma 

==Evaluation==
*[[Head CT]] and/or [[brain MRI]] are important studies to visualize infarctions and brain hemorrhages. 
*CT can show dilation of the sulci accompanies by focal ventricular enlargement.
*MRI has higher sensitivity for detecting ischemic regions. 
*Magnetic Resonance Angiographic studies can demonstrate occlusions within the circle of Willis and better visualize the collateral vasculature. It is preferred over CTA in most institutions.

==Management==
*Symptomatic treatment: decrease [[elevated ICP]], improve cerebral blood flow, control active seizures, pain control , supplemental [[O2]]
*Avoid hypotension, hypervolemia, hypernatremia, hypocarbia
*Ventricular drainage if there is hemorrhage 
*Minimize crying/hyperventilation a decreased PaCO2 can worsen ischemia by vasoconstriction
*Antithrombotic and thrombolytic surgery has not been systematically analyzed for Moyamoya disease 

==Disposition==
*Admit 

==See Also==


==External Links==


==References==
<references/>

[[Category:Neurology]]
[[Category:Vascular]]