Moyamoya: Difference between revisions
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==Background== | ==Background== | ||
MoyaMoya is a cerebrovascular disease characterized by progressive stenosis/occlusion of the arteries around the Circle of Willis. | *MoyaMoya is 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. | *On X-ray 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. | |||
On X-ray Angiography these small blood vessels give off a puff of smoke appearance also known as MoyaMoya in Japanese. | *There is mounting evidence there is a genetic cause of the disease. | ||
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== | ==Clinical Features== | ||
The developed collateral circulation is prone to bleeding, aneurysm, thrombosis exhibiting: focal neurological deficits, TIA, epilepsy, and bowl/bladder incontinence. | *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. | |||
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 | |||
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== | ==Differential Diagnosis== | ||
Hemorrhagic stroke/Ischemic stroke | *Hemorrhagic stroke/Ischemic stroke | ||
*Giant cell arteritis/Vasculitis | |||
Giant cell arteritis/Vasculitis | *Cranial trauma | ||
*Basilar Artery Thrombosis | |||
Cranial trauma | *Blood Dyscrasias | ||
*Cavernous Sinus Syndrome | |||
Basilar Artery Thrombosis | *Cerebral Aneurysms | ||
*Dissection Syndromes | |||
Blood Dyscrasias | *Carotid atherosclerosis | ||
*Fibromusclar Dysplasia | |||
Cavernous Sinus Syndrome | *Craniopharyngioma | ||
Cerebral Aneurysms | |||
Dissection Syndromes | |||
Carotid atherosclerosis | |||
Fibromusclar Dysplasia | |||
Craniopharyngioma | |||
==Evaluation== | ==Evaluation== | ||
Head CT and/or brain MRI are important studies to visualize infarctions and brain hemorrhages. | *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. | ||
*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. | |||
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== | ==Management== | ||
Symptomatic treatment: decrease elevated intracranial pressure, improve cerebral blood flow, control active seizures, pain control , supplemental O2 | *Symptomatic treatment: decrease elevated intracranial pressure, 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 | ||
*Minimize crying/hyperventilation a decreased PaCO2 can worsen ischemia by vasoconstriction | |||
Ventricular drainage if there is hemorrhage | *Antithrombotic and thrombolytic surgery has not been systematically analyzed for Moyamoya disease | ||
Minimize crying/hyperventilation a decreased PaCO2 can worsen ischemia by vasoconstriction | |||
Antithrombotic and thrombolytic surgery has not been systematically analyzed for Moyamoya disease | |||
==Disposition== | ==Disposition== | ||
*Admit | |||
==See Also== | |||
==External Links== | ==External Links== | ||
==References== | ==References== | ||
< | <references/> | ||
[[Category:Neurology]] | |||
[[Category:Vascular]] | |||
Revision as of 05:50, 7 September 2017
Background
- MoyaMoya is 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 X-ray 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
- Cranial trauma
- Basilar Artery Thrombosis
- Blood Dyscrasias
- Cavernous Sinus Syndrome
- 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 intracranial pressure, 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