Moyamoya: Difference between revisions
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==Background== | ==Background== | ||
[[File:Circle of Willis en.png|thumb|Circle of Willis]] | |||
*A cerebrovascular disease characterized by progressive stenosis/occlusion of the arteries around the Circle of Willis. | *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. | *The narrowing or blockage stimulates angiogenesis to provide collateral circulation to the brain. | ||
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==Clinical Features== | ==Clinical Features== | ||
*The developed collateral circulation is prone to bleeding, aneurysm, thrombosis exhibiting: focal neurological deficits, TIA, epilepsy, and | *The developed collateral circulation is prone to bleeding, aneurysm, thrombosis exhibiting: [[focal neurological deficits]], [[TIA]], [[epilepsy]], and bowel/bladder incontinence. | ||
*Children predominately exhibit ischemic | *Children predominately exhibit [[ischemic stroke]]. | ||
*Approximately 2/3rd of children exhibit Electroencephalography (EEG) abnormalities: high voltage slow waves that occur during hyperventilation. | *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 | *Can be associated with atherosclerosis, [[meningitis]], [[vasculitis]], autoimmune diseases, hematological conditions, [[brain tumor]]s, and chromosomal abnormalities | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
*Hemorrhagic stroke/Ischemic stroke | *[[Hemorrhagic stroke]]/[[Ischemic stroke]] | ||
*Giant cell arteritis | *[[Giant cell arteritis]], [[vasculitis]] | ||
* | *[[Head trauma]] | ||
*Basilar | *Basilar artery thrombosis | ||
*Blood | *Blood dyscrasias | ||
*Cavernous | *[[Cavernous sinus thrombosis]] | ||
*Cerebral | *Cerebral aneurysms | ||
*Dissection | *Dissection syndromes (e.g. intracranial [[vertebral and carotid artery dissection]]) | ||
*Carotid atherosclerosis | *Carotid atherosclerosis | ||
* | *Fibromuscular dysplasia | ||
*Craniopharyngioma | *Craniopharyngioma | ||
==Evaluation== | ==Evaluation== | ||
*Head CT and/or brain MRI are important studies to visualize infarctions and brain hemorrhages. | [[File:Moyamoya disease-MRI T1.png|thumb|T1-weighted MR image of moyamoya disease. Flow void in the basal ganglia is indicated by the arrow.]] | ||
[[File:MRA Moya-moya-disease.jpg|thumb|Left: MIP reconstructed MR angiography of a 11-year-old girl with moyamoya disease. | |||
Right: healthy patient, for comparison.]] | |||
*[[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. | *CT can show dilation of the sulci accompanies by focal ventricular enlargement. | ||
*MRI has higher sensitivity for detecting ischemic regions. | *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. | *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== | ==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 | ||
Latest revision as of 22:03, 25 January 2023
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 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 (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