钴胺缺乏的神经系统损害 (附22例报告)

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目的探讨钴胺缺乏所致神经系统损害的临床特征。方法回顾性分析22例钴胺缺乏患者治疗前后的临床资料。结果以中老年男性患者为主,吸收障碍为主要病因。慢性进行性病程和亚急性病程为主,少数呈慢性复发缓解病程。91%的患者为脊髓病+周围神经病的混合型,86%的患者以双下肢远端感觉障碍起病,91%的患者有周围神经电生理异常,并可在早期出现。神经电生理检查和腓肠神经活检提示周围神经呈脱髓鞘和轴索变性混合改变,重型患者轴索变性较明显。甲钴胺治疗后,脊髓病的缓解早于周围神经病,症状缓解早于电生理改变的恢复,周围神经损害需要比较长的治疗时间,痊愈的患者中仍有2/3遗留神经传导速度轻度降低。结论钴胺缺乏对神经系统的损害最常见的为混合性周围神经病,周围神经病可以早期出现,病程越长、病情越重,轴索变性越明显,且治疗相对更困难。 Objective To investigate the clinical features of nervous system damage caused by cobalt amine deficiency. Methods Retrospective analysis of 22 cases of Cobalt amine deficiency before and after treatment of clinical data. Results mainly in the elderly male patients, malabsorption as the main cause. Chronic progressive and sub-acute course of disease-based duration, a small number of chronic relapse to ease the course. Ninety-one percent of patients were mixed with myelopathy + peripheral neuropathy. Eighty-six percent of patients were diagnosed as having distal sensory disturbances in both lower extremities. Ninety-one percent of patients had peripheral electrophysiological abnormalities that occurred early. Neuro-electrophysiological examination and sural nerve biopsy showed demyelination and axonal degeneration of peripheral nerve mixed changes, severe deformity of axonal more obvious. After mecobalamin treatment, myelopathy was earlier than peripheral neuropathy, symptom relief earlier than the recovery of electrophysiological changes, peripheral nerve damage required longer treatment time, and 2/3 of the remaining patients still had mild nerve conduction velocity reduce. CONCLUSION: Cobalamine deficiency is the most common type of peripheral neuropathy in the nervous system. Peripheral neuropathy can occur early. The longer the course of disease, the more severe the disease is, the more obvious the degeneration of the axon is and the more difficult it is to treat.
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