Moyamoya Disease Associated With Pulmonary Sarcoidosis —Case Report—

Abstract

A 61-year

Moyamoya-Like Vascular Abnormality in Pulmonary Sarcoidosis

“…There was no angiographic evidence of vasculitis, and evidence of meningitis was not present on CSF examination. Moreover, autopsy fi ndings of the case mentioned above [3] showed marked fi brous intimal thickening in the bilateral ICAs but without active infl ammation [3] . Therefore, moyamoya-like vascular abnormality associated with sarcoidosis may not be related to active infl ammation.…”

“…Another study reported segmental narrowing and dilatation of large cerebral arteries [7] , but clinical correlation was not made. To our knowledge, there has been only one reported patient who had moyamoya-like vasculopathy associated with sarcoidosis [3] . Similar to our case, the patient had bilat- eral ICA occlusion at the terminal portion.…”

“…Despite the frequent fi nding of vasculitis and cerebral infarcts at autopsy, clinical ischemic events have been rarely reported in patients with sarcoidosis [2,3,5] . Characteristic postmortem fi ndings of these patients were the presence of sarcoid granuloma in the leptomeninges and brain parenchyma, with the invasion of the arterial wall by epithelioid cell granulomas which disrupted the media and the internal elastica causing stenosis or occlusion.…”

“…Although cerebral vascular involvement is not uncommon, large cerebral artery involvement in sarcoidosis is extremely rare [2,3] . We describe a patient with sarcoidosis who showed angiographic fi ndings similar to moyamoya disease.…”

“…10 3 /mm 3 ) and an elevated serum angiotensin-converting enzyme level (67.8 U/l, normal range 8-52 U/l). Electrocardiogram was normal.…”