J Clin Neurol.  2020 Oct;16(4):624-632. 10.3988/jcn.2020.16.4.624.

Clinical Features and Outcomes of Intracranial Aneurysm Associated with Moyamoya Disease

  • 1Department of Neurosurgery, Bucheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 2Department of Neurosurgery, Severance Stroke Center, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
  • 3Department of Neurosurgery, Yongin Severance Hospital, Yonsei University College of Medicine, Yongin, Korea
  • 4Department of Pediatric Neurosurgery, Severance Children’s Hospital, Yonsei University College of Medicine, Seoul, Korea


Background and Purpose
Moyamoya disease (MMD) is a rare form of intracranial stenoocclusive disease that can be associated with intracranial aneurysms. We evaluated the clinical features and outcomes of MMD-associated aneurysms while focusing on their locations.
Between January 1998 and December 2018 there were 1,302 adult and pediatric patients diagnosed as MMD at a single institution. These patients included 38 with 44 MMD-associated aneurysms. The MMD-associated aneurysms were classified into two groups based on their locations: major-artery aneurysms and non-major-artery aneurysms. The clinical and radiological data for patients with MMD-associated aneurysms were reviewed retrospectively.
The 44 MMD-associated aneurysms comprised 28 in major arteries and 16 in nonmajor arteries. All of the major-artery aneurysms were initially unruptured lesions, and follow-up angiography showed that 23 (82.1%) had an improved or stable status and 5 (17.9%) had a worse status. The non-major-artery aneurysms comprised 10 ruptured and 6 unruptured lesions, and follow-up angiography showed that 11 (68.8%) had improved or were stable and 5 (31.2%) had worsened. At the latest follow-up, there were four cases of unfavorable outcome: two initial hemorrhagic insults, one treatment-related morbidity, and one repeatedhemorrhage case.
MMD-associated aneurysms occurred in 3.3% of the MMD cohort in this study, of which 63.6% were major-artery aneurysms and 36.4% were non-major-artery aneurysms. The major-artery group included 17.9% that became angiographically worse, while 31.2% were growing or hemorrhaging in the non-major-artery group.


moyamoya disease; aneurysm; pseudoaneurysm; outcome
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