1987 Volume 27 Issue 1 Pages 42-50
A rare case of multiple intracranial berry aneurysms associated with fibromuscular dysplasia (FMD) and mixed connective tissue disease (MCTD) is reported together with a review of the literature, particularly that of Japan.
The patient was a 59-year-old woman who had a subarachnoid hemorrhage during the course of MCTD. Angiographically, multiple intracranial aneurysms and “string of beads” were demonstrated in both cervical internal carotid arteries. The three aneurysms of the left internal carotid and middle cerebral arteries were treated surgically, and the remaining aneurysms in the right middle cerebral artery and at the top of the basilar artery were to be treated at a later date. Although the immediate postoperative course was uneventful, 1 month after surgery the patient died suddenly of sepsis with disseminated intravascular coagulation. At autopsy, FMD involving medial fibroplasia with aneurysms was found in the internal carotid and renal arteries. The intracranial aneurysms showed features typical of saccular aneurysms related to the arterial fork. Other than the aneurysms, no changes due to FMD were noted in the cerebral arteries. Although characteristic MCTD lesions were present in the visceral organs, no specific changes due to MCTD were seen in the brain.
Among the 29 patients with cervico-cephalic FMD reported in Japan, 15 had intracranial aneurysms. These included four cases of multiple aneurysms and 11 cases of rupture. No association between FMD and MCTD has been reported in the world literature.
