- Department of Neurosurgery, Nagasaki University School of Medicine, Nagasaki, Japan
Department of Neurosurgery, Nagasaki University School of Medicine, Nagasaki, Japan
DOI:10.4103/2152-7806.106579Copyright: © 2013 Hayashi K This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Hayashi K, Horie N, Nagata I. A case of unilateral moyamoya disease suffered from intracerebral hemorrhage due to the rupture of cerebral aneurysm, which appeared seven years later. Surg Neurol Int 29-Jan-2013;4:17
How to cite this URL: Hayashi K, Horie N, Nagata I. A case of unilateral moyamoya disease suffered from intracerebral hemorrhage due to the rupture of cerebral aneurysm, which appeared seven years later. Surg Neurol Int 29-Jan-2013;4:17. Available from: http://sni.wpengine.com/surgicalint_articles/a-case-of-unilateral-moyamoya-disease-suffered-from-intracerebral-hemorrhage-due-to-the-rupture-of-cerebral-aneurysm-which-appeared-seven-years-later/
Background:Whether unilateral moyamoya disease (MMD), confirmed by steno-occlusive lesion at the terminal portion of internal carotid artery with formation of moyamoya vessels unilaterally and normal or equivocal findings contralaterally, is an early form of definite (bilateral) MMD remains controversial. It is well-known that adult patients with MMD tend to suffer from cerebral hemorrhage, occasionally due to the rupture of aneurysm arising from moyamoya vessel.
Case Description:A 61-year-old woman was diagnosed as unilateral MMD incidentally and followed by magnetic resonance imaging annually. Seven years after the diagnosis, cerebral aneurysm appeared on the moyamoya vessel. Before further examination, the aneurysm ruptured and resulted in massive cerebral hemorrhage.
Conclusion:Even in the unilateral MMD, cerebral hemorrhage may occur due to the rupture of cerebral aneurysm. Careful follow-up is recommended and early treatment is required once cerebral aneurysm is detected.
Keywords: Cerebral aneurysm, Intracerebral hemorrhage, moyamoya disease, Unilateral
Moyamoya disease (MMD) is characterized by progressive occlusion of the internal carotid artery (ICA) or its terminal branches, associated with formation of extensive collateral vessels (moyamoya vessels) at the base of the brain. The diagnostic criteria of the Research Committee on Moyamoya Disease (Spontaneous Occlusion of the Circle of Willis) of the Ministry of Health and Welfare of Japan (RCMJ) considers only cases with bilateral lesions as definite MMD.[
A 61-year-old woman without any risk factor of arteriosclerosis visited a nearby hospital because of mild head injury. Magnetic resonance (MR) imaging demonstrated no apparent traumatic lesion. However, flow-void sign of the right middle cerebral artery was sluggish [
Cerebral angiography. (a) Right cerebral angiography reveals severe stenosis at the terminal portion of the internal carotid artery with development of moyamoya vessels, (b) Left carotid angiography shows no apparent abnormality, (c) Left vertebral angiography shows no apparent abnormality. The leptominingeal anastomosis is developed in the right side
Computed tomography (CT). (a) CT shows massive intraventricular hemorrhage, (b) CT with contrast medium revealed enlargement of the cerebral aneurysm, (c) CT angiography showed cerebral aneurysm arising from right anterior choroidal artery, (d) CT angiography coronal image showed cerebral aneurysm (arrow) arising from right anterior choroidal artery (arrow head)
Whether unilateral lesion, confirmed by typical angiographic evidence of MMD unilaterally and normal or equivocal findings contralateral, is an early form of definite (bilateral) MMD remains controversial.[
Cerebral hemorrhage is main clinical manifestation in adult patient with MMD. The fragile moyamoya vessels are considered as a cause of the hemorrhage. Even in unilateral MMD, hemorrhage is a significant clinical manifestation. Ikezaki et al. found that 58% of the adult unilateral MMD patients suffered from hemorrhagic stroke.[
The inherent difficulty in surgical treatment of these aneurysms, due to deep location, small size, and sometimes unfavorable morphology, is compounded in the setting of MMD by vessel hemodynamic fragility.[
In conclusion, cerebral aneurysm may appear during follow-up of unilateral MMD and can be a cause of intracerebral hemorrhage. Once aneurysm is detected, further examination and early treatment is required.
1. Fukui M. Guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis (‘moyamoya’ disease). Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare, Japan. Clin Neurol Neurosurg. 1997. 99: S238-40
2. Harreld JH, Zomorodi AR. Embolization of an unruptured distal lenticulostriate aneurysm associated with moyamoya disease. AJNR Am J Neuroradiol. 2011. 32: E42-3
3. Hayashi K, Suyama K, Nagata I. Clinical features of unilateral moyamoya disease. Neurol Med Chir (Tokyo). 2010. 50: 378-85
4. Ikezaki K, Inamura T, Kawano T, Fukui M. Clinical features of probable moyamoya disease in Japan. Clin Neurol Neurosurg. 1997. 99: S173-7
5. Kasamo S, Asakura T, Yamamoto Y, Kobayashi E. Unilateral moyamoya disease associated with multiple aneurysms. A case report and review of the literature. Neurol Med Chir (Tokyo). 1984. 24: 30-4
6. Kawaguchi S, Sakaki T, Morimoto T, Kakizaki T, Kamada K. Characteristics of intracranial aneurysms associated with moyamoya disease. A review of 111 cases. Acta Neurochir (Wien). 1996. 138: 1287-94
7. Kawano T, Fukui M, Hashimoto N, Yonekawa Y. Follow-up study of patients with “unilateral” moyamoya disease. Neurol Med Chir (Tokyo). 1994. 34: 744-7
8. Kelly ME, Bell-Stephens TE, Marks MP, Do HM, Steinberg GK. Progression of unilateral moyamoya disease: A clinical series. Cerebrovasc Dis. 2006. 22: 109-15
9. Kim SH, Kwon OK, Jung CK, Kang HS, Oh CW, Han MH. Endovascular treatment of ruptured aneurysms or pseudoaneurysms on the collateral vessels in patients with moyamoya disease. Neurosurgery. 2009. 65: 1000-4
10. Kuroda S, Houkin K. Moyamoya disease: Current concepts and future perspectives. Lancet Neurol. 2008. 7: 1056-66
11. Kuroda S, Houkin K, Kamiyama H, Abe H. Effects of surgical revascularization on peripheral artery aneurysms in moyamoya disease: Report of three cases. Neurosurgery. 2001. 49: 463-8