Abstract
Churg-Strauss syndrome (CSS) is a systemic small-vessel vasculitis characterized by the presence of asthma, hypereosinophilia, and necrotizing vasculitis with extravascular eosinophil granulomas. Neurologic involvement in CSS is frequent, usually manifesting as peripheral neuropathy. Central nervous system involvement is much less frequently reported than peripheral neuropathy.
We report a rare case of CSS with unruptured cerebral aneurysm. The patient, 34-year-old man, had suffered from asthma, paranasal sinusitis, peripheral neuropathy, and eosinophilia. He was treated with prednisone and cyclophosphamide under the diagnosis of CSS. Four years later, he presented with syncope by vasovagal reflex during hospitalization. On brain MRI and MRA, a cerebral aneurysm was found incidentally. Angiography demonstrated fusiform aneurysm on the peripheral portion of the middle cerebral artery (MCA). Clipping was performed to obliterate the aneurysm and to clip-reconstruct the parent vessel, which was composed of a single inflow and outflow vessel. Extracranial-intracranial bypass was not performed, as flow to the distal MCA was restored. Microscopic examination of the excised aneurysmal wall revealed increased fibrous connective tissue without infiltration of eosinophils or inflammatory cells.
This case was considered very rare; the literature contains very few cases of cerebral vascular disease associated with CSS. We can’t exclude the possibility that the aneurysm was idiopathic and unrelated to his vasculitis. However, we think this is unlikely because of the location, shape and formation of the lesion. Standard treatment for cerebral aneurysm associated with vasculitis has not been identified because the natural history of each patient is unknown, so appropriate management is recommended.
