Quasi-moyamoya disease (MMD) or moyamoya syndrome is based on various underlying diseases and radiologically simulates MMD, but its disease entity is still unclear. Recent studies have proven specific shrinkage of the involved arteries in MMD. Using 3-dimensional constructive interference in steady state (3D-CISS), therefore, this study aimed to analyze the outer diameter of the involved arteries in quasi-MMD.
This study included 9 patients with quasi-MMD (unilateral type, n = 2; bilateral type, n = 7). Using 3D-CISS, the outer diameter was quantified in the internal carotid artery distal to the posterior communicating artery (C1), the horizontal portion of the middle and anterior cerebral arteries (M1 and A1, respectively), and the basilar artery. Control values were obtained from 17 healthy subjects.
In 7 of 9 patients, the outer diameters of C1, M1, and A1 were significantly smaller than those of the controls. On the other hand, the values were normal in other 2 patients. There was no significant difference in the underlying disorders between the 2 groups. All 3 pediatric patients are categorized into the arterial shrinkage group, but 2 of 6 adult patients were not.
These findings strongly suggest that quasi-MMD is not a uniform disease entity and includes at least 2 pathophysiologically different groups: the arterial shrinkage group and the nonarterial shrinkage group. A certain subgroup of MMD patients may be misdiagnosed as quasi-MMD because of the patients' comorbid disorders and mixed up with the patients who present angiographic findings similar to MMD in spite of the lack of arterial shrinkage.
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- Current state of study on moyamoya disease in Japan.Surg Neurol. 1997; 47: 138-143
- Moyamoya disease: current concepts and future perspectives.Lancet Neurol. 2008; 7: 1056-1066
- Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain.Arch Neurol. 1969; 20: 288-299
- Guidelines for diagnosis and treatment of moyamoya disease (spontaneous occlusion of the circle of Willis).Neurol Med Chir (Tokyo). 2012; 52: 245-266
- Nationwide survey on quasi-moyamoya disease in Japan.Acta Neurochir (Wien). 2014; 156: 935-940
- Moyamoya-like vasculopathy (moyamoya syndrome) in children.Childs Nerv Syst. 2004; 20: 382-391
- Moyamoya syndrome associated with Down syndrome: outcome after surgical revascularization.Pediatrics. 2005; 116: e694-e701
- Moyamoya phenomenon after radiation for optic glioma.J Neurosurg. 1993; 79: 32-35
- A nationwide survey on unilateral moyamoya disease in Japan.Clin Neurol Neurosurg. 2014; 124: 1-5
- Coexisting diseases of moyamoya vasculopathy.J Stroke Cerebrovasc Dis. 2014; 23: 1344-1350
- Diagnosis of moyamoya disease: international standard and regional differences.Neurol Med Chir (Tokyo). 2015; 55: 189-193
- Genetic variant RNF213 c.14576G>A in various phenotypes of intracranial major artery stenosis/occlusion.Stroke. 2013; 44: 2894-2897
- “Real” three-dimensional constructive interference in steady-state imaging to discern microneurosurgical anatomy. Technical note.J Neurosurg. 2003; 98: 625-630
- Outer-diameter narrowing of the internal carotid and middle cerebral arteries in moyamoya disease detected on 3D constructive interference in steady-state MR image: is arterial constrictive remodeling a major pathogenesis?.Acta Neurochir (Wien). 2012; 154: 2151-2157
- High resolution MRI difference between moyamoya disease and intracranial atherosclerosis.Eur J Neurol. 2013; 20: 1311-1318
- Specific shrinkage of carotid forks in moyamoya disease—a novel key finding for diagnosis.Neurol Med Chir (Tokyo). 2015; 55: 796-804
- High-resolution magnetic resonance wall imaging findings of moyamoya disease.Stroke. 2014; 45: 2457-2460
- Sex and side differences of cerebral arterial caliber.Neuroradiology. 1991; 33: 212-216
- Microsurgical anatomy of the middle cerebral artery.J Neurosurg. 1981; 54: 151-169
- Microsurgical anatomy of the middle cerebral artery.Neurol India. 2005; 53: 186-190
- Microsurgical anatomy of the anterior cerebral-anterior communicating-recurrent artery complex.J Neurosurg. 1976; 45: 259-272
- Clinical features and outcome in North American adults with moyamoya phenomenon.Stroke. 2006; 37: 1490-1496
- Genetic analysis of RNF213 c.14576G>A variant in nonatherosclerotic quasi-moyamoya disease.J Stroke Cerebrovasc Dis. 2015; 24: 1075-1079
Published online: March 30, 2016
Accepted: February 20, 2016
Received in revised form: February 16, 2016
Received: February 4, 2016
Source of funding: This study was supported by a grant from the Research Committee on Moyamoya Disease, sponsored by the Ministry of Health, Labor, and Welfare of Japan. The grant number is H26-078. Satoshi Kuroda received the funding.
© 2016 National Stroke Association. Published by Elsevier Inc. All rights reserved.