摘要：

目的 总结脊髓亚急性联合变性的临床特点并分析其治疗与预后结果。方法与结果 2014 年 2 月至 2018 年 2 月共诊断与治疗 75 例脊髓亚急性联合变性患者，其病因与维生素 B₁₂ 摄取、吸收、结合或转运障碍有关。患者均呈慢性或亚急性发病，多以双手或双足感觉异常为首发症状；实验室检测血清同型半胱氨酸水平升高、维生素 B₁₂ 水平降低、血清抗内因子抗体和抗胃壁细胞抗体多呈阳性反应；MRI 检查脊髓病灶主要位于颈髓和胸髓后索和侧索， “倒 V 征”为特征性影像学表现。维生素 B₁₂ 治疗可以改善患者症状与体征。结论 维生素 B₁₂ 缺乏是脊髓亚急性联合变性的重要病因，其临床表现复杂多样，脊髓“倒 V 征”是明确诊断的重要依据，维生素 B₁₂ 治疗有效。

关键词: 脊髓亚急性联合变性, 维生素 B12缺乏, 高半胱氨酸, 磁共振成像

Abstract:

Objective To summarize the clinical manifestations, laboratory, electrophysiological and imaging features, treatment and prognosis of subacute combined degeneration of the spinal cord (SCD). Methods and Results The clinical data of 75 SCD patients from February 2014 to February 2018 were collected and analyzed retrospectively. The etiology of SCD is related to disorders in uptake, absorption, combination or transport of vitamin B₁₂. The onset was chronic or subacute, and main initial symptoms were paresthesia of hands or feet. Laboratory examination showed elevated serum homocysteine (Hcy) levels, reduced vitamin B₁₂ levels and positive anti-intrinsic factor and anti-parietal cell antibodies. Spinal MRI showed that the lesions often occurred in the posterior and lateral funiculus of cervical and thoracic spinal cord. The "inverted V sign" was considered to be the characteristic imaging manifestation. All patients showed improved symptoms and signs to different degrees after vitamin B₁₂ treatment. Conclusions The pathogenesis of SCD is related to vitamin B₁₂ deficiency. The clinical manifestations are diverse. Spinal "inverted V sign" is a strong evidence for clear diagnosis, and vitamin B₁₂ is effective in the treatment of the disease.

Key words: Subacute combined degeneration, Vitamin B12 deficiency, Homocysteine, Magnetic resonance imaging