Background: Patients with major cerebral artery steno-occlusion and the formation of the moyamoya-like vessels associated with some other disorders have been distinguished from moyamoya disease and classified as moyamoya syndrome. The hemodynamic and metabolic backgrounds of the moyamoya syndrome associated with atherosclerosis have not yet been investigated. We aimed to elucidate the hemodynamic and metabolic characteristics associated with the development of basal moyamoya-like vessels in moyamoya syndrome with atherosclerosis. Methods: Twenty-one patients with chronic unilateral atherosclerotic steno-occlusive lesions of the internal carotid artery or middle cerebral artery (MCA) were enrolled in the study. Based on the angiographic findings, the patients were classified into 2 groups: the moyamoya syndrome group (n = 7) and the non-moyamoya-syndrome group (n = 14). We conducted angiographic evaluations of the extent of the development of basal moyamoya-like vessels in the moyamoya syndrome group. The cerebral blood flow, cerebral metabolic rate of oxygen, oxygen extraction fraction (OEF) and cerebral blood volume were measured using PET in the ipsilateral MCA area in the patients and in normal controls (n = 6). Results: The OEF in the ipsilateral MCA area, except in the basal ganglia, was significantly higher in the moyamoya syndrome group than in the non-moyamoya-syndrome group (p < 0.001). The extent of the development of basal moyamoya-like vessels was closely correlated with the elevation of the OEF (r > 0.999, p < 0.001). Conclusion: The basal moyamoya-like vessels are evidence of misery perfusion in patients with unilateral chronic atherosclerotic steno-occlusive lesions of major cerebral artery trunks.

Keywords:
Moyamoya syndrome, Atherosclerosis, Cerebrovascular disease, Chronic cerebral ischemia, Cerebral blood flow, Intracranial arterial stenosis
1.
Suzuki J, Takaku A: Cerebrovascular ‘moyamoya’ disease: disease showing abnormal net-like vessels in base of brain. Arch Neurol 1969;20:288–299.
2.
Piao R, Oku N, Kitagawa K, Imaizumi M, Matsushita K, Yoshikawa T, Takasawa M, Osaki Y, Kimura Y, Kajimoto K, Hori M, Hatazawa J: Cerebral hemodynamics and metabolism in adult moyamoya disease: comparison of angiographic collateral circulation. Ann Nucl Med 2004;18:115–121.
3.
Togao O, Mihara F, Yoshiura T, Tanaka A, Noguchi T, Kuwabara Y, Kaneko K, Matsushima T, Honda H: Cerebral hemodynamics in Moyamoya disease: correlation between perfusion-weighted MR imaging and cerebral angiography. AJNR Am J Neuroradiol 2006;27:391–397.
4.
Adams HP Jr: Moya-Moya; in Bogousslavsky J, Caplan LR (eds): Uncommon Causes of Stroke. Cambridge, Cambridge University Press, 2001, pp 241–257.
5.
Hinshaw DB Jr, Thompson JR, Hasso AN: Adult arteriosclerotic moyamoya. Radiology 1976;118:633–636.
6.
Cerrato P, Grasso M, Lentini A, Destefanis E, Bosco G, Caprioli M, Bradac GB, Bergui M: Atherosclerotic adult Moya-Moya disease in a patient with hyperhomocysteinaemia. Neurol Sci 2007;28:45–47.
7.
Beneficial effect of carotid endarterectomy in symptomatic patients with high-grade carotid stenosis. North American Symptomatic Carotid Endarterectomy Trial Collaborators. N Engl J Med 1991;325:445–453.
8.
Frackowiak RS, Lenzi GL, Jones T, Heather JD: Quantitative measurement of regional cerebral blood flow and oxygen metabolism in man using 15O and positron emission tomography: theory, procedure, and normal values. J Comput Assist Tomogr 1980;4:727–736.
9.
Lammertsma AA, Jones T: Correction for the presence of intravascular oxygen-15 in the steady-state technique for measuring regional oxygen extraction ratio in the brain. 1. Description of the method. J Cereb Blood Flow Metab 1983;3:416–424.
10.
Takeuchi R, Sengoku T, Matsumura K: Usefulness of fully automated constant ROI analysis software for the brain: 3DSRT and FineSRT. Radiat Med 2006;24:538–544.
11.
Tanaka Y, Nariai T, Nagaoka T, Akimoto H, Ishiwata K, Ishii K, Matsushima Y, Ohno K: Quantitative evaluation of cerebral hemodynamics in patients with moyamoya disease by dynamic susceptibility contrast magnetic resonance imaging – comparison with positron emission tomography. J Cereb Blood Flow Metab 2006;26:291–300.
12.
Yamada I, Himeno Y, Suzuki S, Matsushima Y: Posterior circulation in moyamoya disease: angiographic study. Radiology 1995;197:239–246.
13.
Fukui M, Kono S, Sueishi K, Ikezaki K: Moyamoya disease. Neuropathology 2000;20(suppl):S61–S64.
14.
Park J, Hwang YH, Baik SK, Kim YS, Park SH, Hamm IS: Angiographic examination of spontaneous putaminal hemorrhage. Cerebrovasc Dis 2007;24:434–438.
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