Multiple cerebral aneurysms in a patient with CREST syndrome.
Systemic sclerosis (SSc) associated with cerebral aneurysm is rare. We describe a patient with multiple cerebral aneurysms with the calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia (CREST) variant of SSc. A 61-year-old woman with a 20-year history of CREST syndrome was incidentally found to have four cerebral aneurysms located at the C2, C3 and C5 segments of the right internal carotid artery (ICA) and the C2 segment of the left ICA. The bilateral C2 segment aneurysms were successfully clipped using 2-stage surgery. To date, intracranial aneurysms have been reported in only two other patients with CREST syndrome. We hypothesize that the pathogenesis of the aneurysm is related to CREST syndrome. Elucidating the true incidence of cerebral aneurysms associated with CREST syndrome would help to clarify the relationship between SSc-related autoantibodies and aneurysm formation.
Masuoka
Jun
J
Department of Neurosurgery, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan. masuoka@cc.saga-u.ac.jp
Murao
Kenichi
K
Nagata
Izumi
I
Iihara
Koji
K
D017675:CREST Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D033162:Incidental Findings; D002532:Intracranial Aneurysm / Q000150:complications* / Q000000981:diagnostic imaging* / Q000601:surgery; D018810:Magnetic Resonance Angiography; D008875:Middle Aged; D011859:Radiography