Shevell M I, Rosenblatt D S
Department of Neurology/Neurosurgery, McGill University, Montreal, Quebec, Canada.
Can J Neurol Sci. 1992 Nov;19(4):472-86.
The following review indicates that the impact of cobalamin on neurologic disease extends far beyond the traditional myelopathy of classical pernicious anemia. The delineation of a broad spectrum of inherited disorders of cobalamin processing has served to illustrate and precisely define each step in the normal absorption, transport and intracellular metabolism of this essential vitamin. Recent clinical work has extended the boundaries of acquired cobalamin deficiency to encompass a variety of neuropsychiatric disturbances without identifiable concomitant hematologic derangements and emphasized the utility and sensitivity of new laboratory tests. These findings will demand increased vigilance from clinicians so that atypical and subtle cobalamin deficiency states will be readily diagnosed. The wide range of neurologic dysfunction observed in both inherited and acquired disorders of cobalamin metabolism challenges basic scientists to delineate cobalamin's presumed important role in the normal development and homeostasis of the nervous system.
以下综述表明，钴胺素对神经系统疾病的影响远远超出了经典恶性贫血的传统脊髓病。对钴胺素加工的一系列广泛遗传性疾病的描述有助于阐明并精确界定这种必需维生素在正常吸收、运输和细胞内代谢中的每一个步骤。最近的临床研究将获得性钴胺素缺乏的范围扩大到包括各种无明显伴随血液学紊乱的神经精神障碍，并强调了新实验室检测的实用性和敏感性。这些发现将要求临床医生提高警惕，以便能容易地诊断出非典型和轻微的钴胺素缺乏状态。在钴胺素代谢的遗传性和获得性疾病中观察到的广泛神经功能障碍，促使基础科学家去阐明钴胺素在神经系统正常发育和体内平衡中可能发挥的重要作用。
