不典型脊髓亚急性联合变性的临床特征分析

Study on the clinical analysis of patients with atypical spinal subacute combined degeneration

目的探讨不典型脊髓亚急性联合变性（SCD）临床特点和诊断思维。方法对46例SCD患者的临床资料行回顾性分析，并对13例SCD患者首诊误诊的临床资料行重点分析。结果在46例SCD患者中，脊髓后侧索和周围神经、侧索和周围神经、后索和周围神经及后侧索同时受损者分别占52．17％，10．87％，10．87％，10．87％，仅有后索或侧索受损者分别占8．70％，6．52％。血红蛋白（Hb）降低者占43．48％，平均红细胞体积（MCV）升高者占71．74％；血清VitB12降低者占50％（21／42）；脊髓MRI检查发现异常者占28．57％（6／21）。在13例首诊误诊患者中，4例仅有后索和3例仅有侧索受损，侧索和周围神经、后索和周围神经同时受损者各2例，后侧索、后侧索和周围神经同时受损者各1例；5例血清VitB12降低，脊髓MRI检查显示异常1例；首诊误诊率占28．26％（13／46）。结论不典型SCD主要临床特点可为仅累及后索或侧索的非联合性脊髓受损表现，或后索和侧索受损表现不均衡，VitB12等检查可正常。准确识别脊髓后侧索受损的阳性表现、查找体内VitB12缺乏的佐证、排他疾病及VitB12试验性治疗等是避免不典型SCD误诊的有效措施。

Objective To explore the clinical characteristics and diagnosis thinking of atypical spi- nal subacute combined degeneration （SCD）. Methods The clinical data of 46 cases with SCD were ana- lyzed retrospectively, and the data of 13 cases with atypical SCD misdiagnosed were further analyzed. Results In 46 patients with SCD, posterolateral funiculus and peripheral nerve, lateral funiculus and pe- ripheral nerve, posterior funiculus and peripheral nerve and posterolateral funiculus impaired accounted for 52.17%, 10.87~, 10.87% and 10.87%, respectively, only the posterior funiculus or lateral funic- ulus damaged accounted for 8.70% , 6.52%. Hemoglobin（Hb） decreased accounted for 4a. 48%, The mean corpuscular volume increased accounted for 71.74%; Serum Vit B12 lower accounted for 50% （21/ 42）; Spinal MRI examination revealed abnormal accounted for 28.57 % （6/21）. In 13 cases of misdiag- nosed patients, four cases damaged in posterior funiculus, three in lateral funieulus, two in lateral funiculus and peripheral nerve, and two in posterior funieulus and peripheral nerve. The other two cases damaged in posterolateral funiculus, and in posterolateral funiculus and peripheral nerve. The serum Vit B12 level was low in 5 of 13 cases. Spinal MRI showed abnormal in 1 case, Misdiagnosed rate was 28.26% （13/46）for the first diagnosis. Conclusions The main clinical features of atypical SCD can be summarized that damages of spinal cord only involve in posterior funiculus or lateral funiculus, or posterior funiculus and lateral funiculus damaged performance was not balanced. The other examinations such as Vit B12 can be normal. To identify the positive signs of damaged posternlateral funieulus, find the evidence of body Vit B12 deficiency, eliminate the other neurological diseases, and give the Vit B12 experimental treatment are probably the effective measures to avoid misdiagnosing atypical SCD.