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亚急性联合变性:临床、电生理及磁共振成像表现

Subacute combined degeneration: clinical, electrophysiological, and magnetic resonance imaging findings.

作者信息

Hemmer B, Glocker F X, Schumacher M, Deuschl G, Lücking C H

机构信息

Department of Neurology, University of Freiburg, Germany.

出版信息

J Neurol Neurosurg Psychiatry. 1998 Dec;65(6):822-7. doi: 10.1136/jnnp.65.6.822.

Abstract

OBJECTIVE

Vitamin B12 deficiency is a systemic disease that often affects the nervous system. One of the most prevalent manifestations is subacute combined degeneration (SCD) of the spinal cord. To access the clinical, electrophysiological, and structural abnormalities associated with SCD, a study was conducted in nine patients.

METHODS

Clinical, electrophysiological (electroneurography, somatosensory and motor evoked potentials), and MRI evaluations were performed in patients before and after treatment.

RESULTS

The most prominent clinical and electrophysiological findings in all patients were dysfunctions of the posterior column. Corresponding hyperintense lesions in the posterior column of the spinal cord were found in two patients by T2 weighted MRI. Damage to the central motor pathway was identified in four patients. Demyelinating neuropathy was present in one patient and axonal neuropathy in four. All patients showed improvement of their symptoms after treatment with cobalamin. Abnormalities of the spinal cord on MRI disappeared early in recovery. Motor evoked potentials and median somatosensory evoked potentials typically normalised after treatment, whereas tibial somatosensory evoked potentials remained abnormal in most patients.

CONCLUSIONS

Clinical, electrophysiological, and MRI findings associated with SCD in vitamin B12 deficiency are diverse. Thus vitamin B12 deficiency should be considered in the differential diagnosis of all spinal cord, peripheral nerve, and neuropsychiatric disorders.

摘要

目的

维生素B12缺乏是一种常影响神经系统的全身性疾病。最常见的表现之一是脊髓亚急性联合变性(SCD)。为了解与SCD相关的临床、电生理和结构异常,对9例患者进行了一项研究。

方法

在治疗前后对患者进行临床、电生理(神经电图、体感和运动诱发电位)及MRI评估。

结果

所有患者最突出的临床和电生理表现为后柱功能障碍。通过T2加权MRI在2例患者中发现脊髓后柱相应的高信号病变。在4例患者中发现中枢运动通路受损。1例患者存在脱髓鞘性神经病变,4例存在轴索性神经病变。所有患者经钴胺素治疗后症状均有改善。MRI上脊髓的异常在恢复早期消失。运动诱发电位和正中神经体感诱发电位通常在治疗后恢复正常,而大多数患者胫神经体感诱发电位仍异常。

结论

维生素B12缺乏症中与SCD相关的临床、电生理和MRI表现多种多样。因此,在所有脊髓、周围神经和神经精神疾病的鉴别诊断中均应考虑维生素B12缺乏。

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Subacute combined degeneration.亚急性联合变性
J Neurol Neurosurg Psychiatry. 1998 Dec;65(6):807. doi: 10.1136/jnnp.65.6.807.

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2
Evoked responses in vitamin B12 deficiency.
Ann Neurol. 1981 Apr;9(4):407-9. doi: 10.1002/ana.410090417.
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The peripheral neuropathy of vitamin B12 deficiency.
J Neurol Sci. 1984 Oct;66(1):117-26. doi: 10.1016/0022-510x(84)90147-3.
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Diagnosis of megaloblastic anemia.
Annu Rev Med. 1991;42:311-22. doi: 10.1146/annurev.me.42.020191.001523.
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Neurologic aspects of cobalamin deficiency.钴胺素缺乏的神经学方面
Medicine (Baltimore). 1991 Jul;70(4):229-45. doi: 10.1097/00005792-199107000-00001.

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