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维生素B12缺乏所致的神经系统疾病

Neurological disorders in vitamin B12 deficiency.

作者信息

Pavlov Ch S, Damulin I V, Shulpekova Yu O, Andreev E A

机构信息

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Moscow, Russia.

出版信息

Ter Arkh. 2019 May 16;91(4):122-129. doi: 10.26442/00403660.2019.04.000116.

Abstract

The review discusses thesteps of vitamin B12 metabolism and its role in maintaining of neurological functions. The term "vitamin B12 (cobalamin)" refers to several substances (cobalamins) of a very similar structure. Cobalamin enters the body with animal products. On the peripherу cobalamin circulates only in binding with proteins transcobalamin I and II (complex cobalamin-transcobalamin II is designated as "holotranscobalamin"). Holotranscobalamin is absorbed by different cells, whereas transcobalamin I-binded vitamin B12 - only by liver and kidneys. Two forms of cobalamin were identified as coenzymes of cellular reactions which are methylcobalamin (in cytoplasm) and hydroxyadenosylcobalamin (in mitochondria). The main causes of cobalamin deficiency are related to inadequate intake of animal products, autoimmune gastritis, pancreatic insufficiency, terminal ileum disease, syndrome of intestinal bacterial overgrowth. Relative deficiency may be seen in excessive binding of vitamin B12 to transcobalamin I. Cobalamin deficiency most significantly affects functions of blood, nervous system and inflammatory response. Anemia occurs in 13-15% of cases; macrocytosis is an early sign. The average size of neutrophils and monocytes is the most sensitive marker of megaloblastic hematopoiesis. The demands in vitamin B12 are particularly high in nervous tissue. Hypovitaminosis is accompanied by pathological lesions both in white and gray brain matter. Several types of neurological manifestations are described: subacute combined degeneration of spinal cord (funicular myelinosis), sensomotor polyneuropathy, optic nerve neuropathy, cognitive disorders. The whole range of neuropsychiatric disorders with vitamin B12 deficiency has not been studied well enough. Due to certain diagnostic difficulties they are often regarded as "cryptogenic", "reactive", "vascular» origin. Normal or decreased total plasma cobalamin level could not a reliable marker of vitamin deficiency. In difficult cases the content of holotranscobalamin, methylmalonic acid / homocysteine, and folate in the blood serum should be investigated besides carefully analysis of clinical manifestations.

摘要

这篇综述讨论了维生素B12的代谢步骤及其在维持神经功能中的作用。术语“维生素B12(钴胺素)”指的是几种结构非常相似的物质(钴胺素)。钴胺素通过动物产品进入人体。在外周,钴胺素仅与转钴胺素I和II结合循环(钴胺素 - 转钴胺素II复合物被称为“全转钴胺素”)。全转钴胺素被不同细胞吸收,而与转钴胺素I结合的维生素B12仅被肝脏和肾脏吸收。已确定两种形式的钴胺素作为细胞反应的辅酶,即甲基钴胺素(在细胞质中)和羟基腺苷钴胺素(在线粒体中)。钴胺素缺乏的主要原因与动物产品摄入不足、自身免疫性胃炎、胰腺功能不全、回肠末端疾病、肠道细菌过度生长综合征有关。维生素B12与转钴胺素I过度结合时可能出现相对缺乏。钴胺素缺乏最显著地影响血液、神经系统和炎症反应的功能。13% - 15%的病例会发生贫血;大细胞性贫血是早期迹象。中性粒细胞和单核细胞的平均大小是巨幼细胞性造血最敏感的标志物。神经组织对维生素B12的需求特别高。维生素缺乏症伴有脑白质和灰质的病理病变。描述了几种类型的神经学表现:脊髓亚急性联合变性(索状髓鞘脱失)、感觉运动性多神经病、视神经神经病、认知障碍。维生素B12缺乏引起的整个神经精神障碍范围尚未得到充分研究。由于某些诊断困难,它们常被视为“原因不明的”、“反应性的”、“血管性的”起源。血浆钴胺素总水平正常或降低并非维生素缺乏的可靠标志物。在疑难病例中,除了仔细分析临床表现外,还应检测血清中全转钴胺素、甲基丙二酸/同型半胱氨酸和叶酸的含量。

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