Moyamoya: Difference between revisions
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==Differential Diagnosis== | ==Differential Diagnosis== | ||
*Hemorrhagic stroke/Ischemic stroke | *[[Hemorrhagic stroke]]/[[Ischemic stroke]] | ||
*Giant cell arteritis | *[[Giant cell arteritis]], [[vasculitis]] | ||
* | *[[Head trauma]] | ||
*Basilar Artery Thrombosis | *Basilar Artery Thrombosis | ||
*Blood Dyscrasias | *Blood Dyscrasias | ||
*Cavernous | *[[Cavernous sinus thrombosis]] | ||
*Cerebral Aneurysms | *Cerebral Aneurysms | ||
*Dissection Syndromes | *Dissection Syndromes | ||
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==Management== | ==Management== | ||
*Symptomatic treatment: decrease elevated | *Symptomatic treatment: decrease [[elevated ICP]], improve cerebral blood flow, control active seizures, pain control , supplemental O2 | ||
*Avoid hypotension, hypervolemia, hypernatremia, hypocarbia | *Avoid hypotension, hypervolemia, hypernatremia, hypocarbia | ||
*Ventricular drainage if there is hemorrhage | *Ventricular drainage if there is hemorrhage | ||
Revision as of 18:30, 27 September 2017
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 bowl/bladder incontinence.
- Children predominately exhibit ischemic strokes.
- 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 tumors, 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
- Carotid atherosclerosis
- Fibromusclar 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