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[脊髓亚急性联合变性:诊断容易,治疗有效]

[Subacute combined degeneration of the spinal cord: easy diagnosis, effective treatment].

作者信息

Jongen J C, Koehler P J, Franke C L

机构信息

Atrium Medisch Centrum, afd. Neurologie, Postbus 4446, 6401 CX Heerlen.

出版信息

Ned Tijdschr Geneeskd. 2001 Jun 30;145(26):1229-33.

Abstract

Four patients, three women aged 49, 47 and 74 years, and a man aged 64 years, presented with progressive sensory deficit, pyramidal tract symptoms and postural instability. Tests revealed megaloblastic anaemia and low vitamin B12 levels. Two of the female patients had undergone gynaecological surgery with nitrous oxide anaesthesia, and the male patient had undergone a gastric resection. Subacute combined degeneration of the spinal cord is a neurological disease based on vitamin B12 deficiency. It involves the posterior and lateral columns of the spinal cord, and sometimes the peripheral nerves, the optic nerve or the brain. An MRI scan of the cervical cord revealed abnormalities for three of the four patients. Following parenteral supplementation of vitamin B12, the symptoms and the MRI abnormalities either disappeared or significantly improved. Vitamin B12 deficiency can cause subacute combined degeneration of the cord by interfering with myelin synthesis. As vitamin B12 deficiency is caused by malabsorption in the gastrointestinal tract, oral supplementation is insufficient. It is essential to recognise this treatable disease at an early stage, and not to reject the possible diagnosis if the MRI findings are abnormal. Simple blood tests can lead to the diagnosis and to effective treatment.

摘要

4例患者,3名女性,年龄分别为49岁、47岁和74岁,1名男性,64岁,表现为进行性感觉障碍、锥体束征和姿势性不稳。检查发现巨幼细胞贫血和维生素B12水平低下。其中2名女性患者接受过一氧化二氮麻醉的妇科手术,男性患者接受过胃切除术。脊髓亚急性联合变性是一种基于维生素B12缺乏的神经系统疾病。它累及脊髓的后索和侧索,有时也累及周围神经、视神经或脑。4例患者中有3例颈椎MRI扫描显示异常。经胃肠外补充维生素B12后,症状及MRI异常表现要么消失,要么显著改善。维生素B12缺乏可通过干扰髓鞘合成导致脊髓亚急性联合变性。由于维生素B12缺乏是由胃肠道吸收不良引起的,口服补充不足。必须在早期识别这种可治疗的疾病,如果MRI结果异常,不要排除可能的诊断。简单的血液检查即可确诊并进行有效治疗。

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