Histological findings observed in major vascular lesions of MMD include fibrocellular thickening of the intima, waving of the internal elastic lamina, and attenuation of the media.
The pathology-inducing cerebral ischemia or hemorrhage requires further investigation. Clues to understanding the disease could be revealed from a study of racial differences between Asians and ethno-Europeans and from a comparison with moyamoya syndrome.
Multidimensional research with further histological, neuroimaging, molecular-biological, and genetic approaches could clarify the etiology of MMD.
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23
Introduction
The Research Committee on Spontaneous Occlusion of the Circle of Willis of the Ministry of Health and Welfare, Japan, defines typical moyamoya disease as specific angiographic findings of diffuse stenotic or occlusive lesions of the bilateral carotid fork and unique collateral vessels at the base of the brain. However, there are some atypical cases of moyamoya disease that show unilateral lesions on angiography and a normal terminal portion of contralateral internal carotid artery or proximal middle cerebral artery. These patients are categorized as having ‘unilateral’ moyamoya disease. Kelly et al. reported that patients with angiographically unilateral lesions comprised up to 18% of patients with moyamoya disease who were treated surgically [1] . Recently, however, asymptomatic moyamoya diseases detected by MRI have been increasing, so that the real incidence is thought to be higher than expected. Also, until now, the natural history of unilateral moyamoya disease has been unclear, and whether it is an early form of moyamoya disease remains controversial.
Here, although there have been only a small number of reports related to this topic, we will review the clinical features and progression of disease in unilateral moyamoya disease.