Abstract Details

Title Moyamoya Syndrome in the Setting of Sturge Weber: A Case Report

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P13 - Poster Session 13 (8:00 AM-9:00 AM)

Poster/Presentation
Number 13-010

Background SWS, a neurocutaneous syndrome characterized by facial capillary malformation, ocular abnormalities (glaucoma and choroidal hemangioma) and leptomeningeal capillary-venous malformations. Its occurrence is sporadic, caused by GNAQ mutation, with a frequency between 1/20,000 - 50,000 live births. Moyamoya Disease (MMD) is a progressive steno-occlusive disease in the terminal portions of the internal carotid artery (ICA). It is called syndrome when related to another clinical condition. The incidence rate of MMD is 0.086 to 2.3 in 100,000. Both the conditions are extremely rare and co-existence has only been documented once before in a patient in late adulthood.

Objective To report a case of a patient developing Moyamoya Syndrome (MMS) in the setting of Sturge-Weber Syndrome.

Design/Methods N/A

Results A 19 year-old previously healthy female presented to our hospital with occipital migraines associated with neck stiffness and vomiting for 4 days. Physical exam revealed right-sided face and neck hemangioma which were present since birth associated with impaired sensation. She also had left arm numbness for years. Labs including CSF examination was normal. CTA head and neck showed right ICA that originates from the aorta with aneurysmal dilation at the level of terminus ICA and Moyamoya appearance of the proximal right MCA. MRI showed mild right brainstem atrophy, remote right thalamic lacunar infarct and right leptomeningeal enhancement concerning for Sturge-Weber Syndrome. Diagnostic angiogram showed right ICA occlusion at the origin with collateral flow from the right vertebral artery and right M1 occlusion with Moyamoya collateral formation. Headache improved with analgesics and she was discharged with aspirin 81mg daily without any surgical intervention.

Conclusions While SWS is associated with leptomeningeal vascular malformation, it is crucial to recognize other associated intracranial vasculopathies such as Moyamoya, a rare association – as SWS may complicate the symptomatology by ‘vascular steal phenomenon’ and may also cause relative decrease in brain perfusion by the shunt.

Authors/Disclosures
Aaisha Mozumder, MD PRESENTER	Dr. Mozumder has nothing to disclose.
	No disclosure on file
Richard C. Simon	Mr. Simon has nothing to disclose.
	No disclosure on file
Firas Kaddouh, MD	Dr. Kaddouh has nothing to disclose.