Primary Angiitis of the Central Nervous System: Report of Two Cases and Review of the Literature

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Abstract
Two cases of primary angiitis of the central nervous system with subarachnoid hemorrhage are reported. One patient was admitted with very thin and localized sulcal subarachnoid hemorrhage, followed by the development of multiple parenchymal edema. Although the first angiography showed only a mild narrowing of an artery, the second angiography demonstrated multiple segmental narrowings of arteries. An open biopsy failed to show histological changes. The second patient suffered from sylvian subarachnoid hemorrhage along with multiple intracerebral hemorrhages, with angiography that was diagnostic for angiitis. In both cases, predonine was initiated and further angiography clearly demonstrated a resolution of the angiitis. The usefulness of serial angiography for diagnosis in comparison with surgical biopsy and for monitoring the treatment of this disease is discussed. The occurrence of subarachnoid hemorrhage in this disease is also reviewed.