Moyamoya vasculopathy in a patient with scleroderma: A case report and review of the literature

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Objective: There is a paucity of data on moyamoya syndrome (MMS) in the setting of scleroderma. We aim to present such a case and systematically review the literature in order to define the clinical, radiologic and outcome features of this condition and propose an association between the pathological mechanisms underlying the vascular changes of MMS and scleroderma. Background: Moyamoya disease and syndrome constitute two distinct pathological entities. MMS represents a secondary vasculopathy associated with a systemic inflammatory process. The main angiographic finding is progressive distal internal carotid artery occlusion which results in either ischemic or hemorrhagic sequelae. Design/Methods: Case report and comprehensive English language Medline search using the keywords “moyamoya syndrome” and “systemic sclerosis”. Results: Case report: 54 year-old female with a history of previous stroke presented with right hemiparesis, dysarthria and right hand and digit swelling. Brain MRI demonstrated patchy acute ischemic infarcts in the cortical and subcortical right middle cerebral artery (MCA) territory and chronic infarcts in bilateral cortical and subcortical regions. Vessel imaging revealed moyamoya-type vasculopathy. Laboratory testing revealed positive ANA and Scl- 70 antibodies. Aggressive medical management was instituted with antiplatelet, statin and antihypertensive medications, and tight risk factor control. External carotid to internal carotid bypass surgery is contemplated. Literature review: We identified 2 additional cases. Including our case, the mean age to stroke onset was 52.7 years. Clinical presentation was with hemiparesis (n=1), TIA (n=1) and nonspecific neurological complaints (n=1). Patients received IV tPAand risk factor control (n=1) with improvement, and high dose IV steroids and cyclosporin (n=1) with subsequent reduction in frequency of TIA events. Conclusions: MMS in the context of scleroderma is a very rare entity. The angiographic findings are result of a systemic process at the cellular level, independent of atheromatous changes. They may represent progressive vasculopathy different from the classically described Suzuki stages for moyamoya disease.




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