Difficulties in diagnosing moyamoya disease

Y. Melnikova, D. Sadykova, T. P. Makarova, D. M. Yasav, A. F. Khamidullin, M. F. Bikmullin
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Abstract

Moyamoya disease is a progressive cerebrovascular disease characterized by bilateral stenosis of the supraclinoid (proximal) part of the internal carotid arteries and the initial segments of the anterior and middle cerebral arteries, with subsequent involvement of the vertebrobasilar basin and accompanied by the development of a basal network of anastomoses. The disease most often manifests itself at the age of 5 to 30–40 years, which makes this pathology an actual and significant cause of functional disorders in children and young people. Diagnosis of moyamoya disease can be difficult because of the polymorphism of its clinical manifestations. This disease must be taken into account in the differential diagnosis of symptoms of cerebral ischemia, since early detection and therapeutic intervention can improve the prognosis by preventing irreversible brain damage. The article presents its own clinical observation of a 6-year-old child with moyamoya disease with a predominant lesion of the right internal carotid artery basin.
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诊断莫亚莫亚病的困难
莫亚莫亚病(Moyamoya disease)是一种进行性脑血管疾病,其特点是颈内动脉上段(近端)、大脑前动脉和中动脉起始段双侧狭窄,随后累及椎基底动脉盆,并伴有基底吻合网的形成。这种疾病最常在 5 至 30-40 岁时表现出来,因此这种病变是儿童和青少年功能障碍的一个实际而重要的原因。由于临床表现的多态性,moyamoya 病的诊断可能比较困难。在对脑缺血症状进行鉴别诊断时必须考虑到这种疾病,因为早期发现和治疗干预可以防止不可逆的脑损伤,从而改善预后。文章介绍了自己对一名患有莫亚莫亚病的 6 岁儿童的临床观察,该患儿主要病变位于右侧颈内动脉盆。
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