脊髓亚急性联合变性75例临床分析
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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B_(12)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B_(12)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B_(12)治疗可以改善患者症状与体征。结论维生素B_(12)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B_(12)治疗有效。
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 B_(12). The onset was chronic or subacute, and main initial symptoms were paresthesia of hands or feet. Laboratory examination showed elevated serum homocysteine(Hey) levels,reduced vitamin B_(12) 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 B_(12) treatment. Conclusions The pathogenesis of SCD is related to vitamin B_(12) deficiency. The clinical manifestations are diverse. Spinal "inverted V sign" is a strong evidence for clear diagnosis, and vitamin B_(12) is effective in the treatment of the disease.
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 B_(12). The onset was chronic or subacute, and main initial symptoms were paresthesia of hands or feet. Laboratory examination showed elevated serum homocysteine(Hey) levels,reduced vitamin B_(12) 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 B_(12) treatment. Conclusions The pathogenesis of SCD is related to vitamin B_(12) deficiency. The clinical manifestations are diverse. Spinal "inverted V sign" is a strong evidence for clear diagnosis, and vitamin B_(12) is effective in the treatment of the disease.
引文
[1] Chen H, Li H, Li Y, Jing J, Raza HK, Zhang Z, Dong L, Ye X,Hua F, Cui G. Clinical and imaging characteristics of subacute combined degeneration complicated with white matter lesions in the brain:a report of five cases[J]. Somatosens Mot Res, 2018,35:119-123.
[2] Xiao CP, Ren CP, Cheng JL, Zhang Y, Li Y, Li BB, Fan YZ.Conventional MRI for diagnosis of subacute combined degeneration(SCD)of the spinal cord due to vitamin B-12deficiency[J]. Asia Pac J Clin Nutr, 2016, 25:34-38.
[3] Nakamura T, Nishi M, Rikitake M, Koga D, Eto J, Tajima D,Toda S, Matsuo M. A case of subacute combined degeneration of the spinal cord due to folic acid and copper deficiency[J].Brain Dev, 2019, 41:111-115.
[4] Cao J, Su ZY, Xu SB, Liu CC. Subacute combined degeneration:a retrospective study of 68 cases with short-term follow-up[J]. Eur Neurol, 2018, 79(5/6):247-255.
[5] Li PP, Zhao B, Zhao XF, Hao XR, Han GR. Clinical study progress of subacute combined degeneration of spinal cord[J].Zhonghua Lin Chuang Yi Shi Za Zhi(Dian Zi Ban), 2016, 10:121-123.[李鹏鹏,赵斌,赵晓峰,郝喜荣,韩国锐.脊髓亚急性联合变性临床研究进展[J].中华临床医师杂志(电子版),2016, 10:121-123.]
[6] Divate PG, Patanwala R. Neurological manifestations of B(12)deficiency with emphasis on its aetiology[J]. J Assoc Physicians India, 2014, 62:400-405.
[7] Lan SY, Kuo CY, Chou CC, Kong SS, Hung PC, Tsai HY, Chen YC, Lin JJ, Chou IJ, Lin KL; PCHAN Study Group.Recreational nitrous oxide abuse related subacute combined degeneration of the spinal cord in adolescents:a case series and literature review[J]. Brain Dev, 2019.[Epub ahead of print]
[8] Garakani A, Jaffe RJ,Savla D, Welch AK, Protin CA, BrysonEO, Mcdowell DM. Neurologic, psychiatric, and other medical manifestations of nitrous oxide abuse:a systematic review of the case literature[J]. Am J Addict, 2016, 25:358-369.
[9] Patel KK, Mejia Munne JC, Gunness VR, Hersey D, Alshafai N, Sciubba D, Nasser R, Gimbel D, Cheng J, Nouri A.Subacute combined degeneration of the spinal cord following nitrous oxide anesthesia:a systematic review of cases[J]. Clin Neurol Neurosurg, 2018, 173:163-168.
[10] Li HT, Chu CC, Chang KH, Liao MF, Chang HS, Kuo HC, Lyu RK. Clinical and electrodiagnostic characteristics of nitrous oxide-induced neuropathy in Taiwan[J]. Clin Neurophysiol,2016, 127:3288-3293.
[11] Kalita J, Chandra S, Bhoi SK, Agarwal R, Misra UK, Shankar SK, Mahadevan A. Clinical, nerve conduction and nerve biopsy study in vitamin B_(12)deficiency neurological syndrome with a short-term follow-up[J]. Nutr Neurosci, 2014, 17:156-163.
[12] Gao JL, Liu JJ, Gao L, Li WD, Zhang Y, Du TY, Huang G.Analysis of 3 atypical cases of subacute combined degeneration[J].Nao Yu Shen Jing Ji Bing Za Zhi,2016, 24:513-518.[高金立,刘尖尖,高蕾,李卫东,张颖,杜铁英,黄光.亚急性联合变性不典型性三例分析[J].脑与神经疾病杂志,2016, 24:513-518.]
[13] Bi HY, Zhang Q, Zhao YM, Li JM, Zhang YB. The clinical analysis of 24 patients with subacute combined degeneration of the spinal cord[J]. Zhongguo Shen Jing Mian Yi Xue He Shen Jing Bing Xue Za Zhi,2014, 21:254-256.[毕鸿雁,张芹,赵亚明,李继梅,张拥波.脊髓亚急性联合变性24例临床分析[J].中国神经免疫学和神经病学杂志,2014, 21:254-256.]
[14] Li J, Zhang L, Zhang Y, Deng B, Bi X. Misdiagnosis of spinal subacute combined degeneration in a patient with elevated serum B_(12)concentration and sensory deficit level[J]. Neurol Sci, 2016, 37:1577-1578.
[15] Schrempf W, Eulitz M, Neumeister V, Siegert G, Koch R,Reichmann H, Storch A. Utility of measuring vitamin B_(12)and its active fraction, holotranscobalamin, in neurological vitamin B_(12)deficiency syndromes[J]. J Neurol, 2011, 258:393-401.
[16] Matsuura H, Nakamura T. Inverted V sign:subacute combined degeneration of the spinal cord[J]. QJM, 2018, 111:65-66.
[17] Sun HY, Lee JW, Park KS, Wi JY, Kang HS. Spine MR imaging features of subacute combined degeneration patients[J]. Eur Spine J, 2014, 23:1052-1058.
[18] Jain KK, Malhotra HS, Garg RK, Gupta PK, Roy B, Gupta RK.Prevalence of MR imaging abnormalities in vitamin B_(12)deficiency patients presenting with clinical features of subacute combined degeneration of the spinal cord[J]. J Neurol Sci,2014, 342(1/2):162-166.
[19] Jiang M, Hu HT, Yan X, Pang Y, Yan LR, Ma ZG, Guo XL,Chen J, Han YT, Qin ZH, Tang S, Gao Q, You XF. Differences of abnomal MRI and clinical manifestations in different periods of subacute combined degeneration in different periods on the rate of abnormal MRI and clinical manifestations[J]. Zhong Feng Yu Shen Jing Ji Bing Za Zhi,2013, 30:222-225.[姜明,胡洪涛,闫欣,庞英,阎立荣,马志刚,郭笑磊,陈捷,韩仰同,秦朝晖,唐澍,高倩,尤小凡.亚急性联合变性病程不同时期MRI上病灶出现率及临床表现的差别[J].中风与神经疾病杂志,2013, 30:222-225.]
[20] Vasconcelos OM, Poehm EH, Mccarter RJ, Campbell WW,Quezado ZM. Potential outcome factors in subacute combined degeneration:review of observational studies[J]. J Gen Intern Med, 2006, 21:1063-1068.
[2] Xiao CP, Ren CP, Cheng JL, Zhang Y, Li Y, Li BB, Fan YZ.Conventional MRI for diagnosis of subacute combined degeneration(SCD)of the spinal cord due to vitamin B-12deficiency[J]. Asia Pac J Clin Nutr, 2016, 25:34-38.
[3] Nakamura T, Nishi M, Rikitake M, Koga D, Eto J, Tajima D,Toda S, Matsuo M. A case of subacute combined degeneration of the spinal cord due to folic acid and copper deficiency[J].Brain Dev, 2019, 41:111-115.
[4] Cao J, Su ZY, Xu SB, Liu CC. Subacute combined degeneration:a retrospective study of 68 cases with short-term follow-up[J]. Eur Neurol, 2018, 79(5/6):247-255.
[5] Li PP, Zhao B, Zhao XF, Hao XR, Han GR. Clinical study progress of subacute combined degeneration of spinal cord[J].Zhonghua Lin Chuang Yi Shi Za Zhi(Dian Zi Ban), 2016, 10:121-123.[李鹏鹏,赵斌,赵晓峰,郝喜荣,韩国锐.脊髓亚急性联合变性临床研究进展[J].中华临床医师杂志(电子版),2016, 10:121-123.]
[6] Divate PG, Patanwala R. Neurological manifestations of B(12)deficiency with emphasis on its aetiology[J]. J Assoc Physicians India, 2014, 62:400-405.
[7] Lan SY, Kuo CY, Chou CC, Kong SS, Hung PC, Tsai HY, Chen YC, Lin JJ, Chou IJ, Lin KL; PCHAN Study Group.Recreational nitrous oxide abuse related subacute combined degeneration of the spinal cord in adolescents:a case series and literature review[J]. Brain Dev, 2019.[Epub ahead of print]
[8] Garakani A, Jaffe RJ,Savla D, Welch AK, Protin CA, BrysonEO, Mcdowell DM. Neurologic, psychiatric, and other medical manifestations of nitrous oxide abuse:a systematic review of the case literature[J]. Am J Addict, 2016, 25:358-369.
[9] Patel KK, Mejia Munne JC, Gunness VR, Hersey D, Alshafai N, Sciubba D, Nasser R, Gimbel D, Cheng J, Nouri A.Subacute combined degeneration of the spinal cord following nitrous oxide anesthesia:a systematic review of cases[J]. Clin Neurol Neurosurg, 2018, 173:163-168.
[10] Li HT, Chu CC, Chang KH, Liao MF, Chang HS, Kuo HC, Lyu RK. Clinical and electrodiagnostic characteristics of nitrous oxide-induced neuropathy in Taiwan[J]. Clin Neurophysiol,2016, 127:3288-3293.
[11] Kalita J, Chandra S, Bhoi SK, Agarwal R, Misra UK, Shankar SK, Mahadevan A. Clinical, nerve conduction and nerve biopsy study in vitamin B_(12)deficiency neurological syndrome with a short-term follow-up[J]. Nutr Neurosci, 2014, 17:156-163.
[12] Gao JL, Liu JJ, Gao L, Li WD, Zhang Y, Du TY, Huang G.Analysis of 3 atypical cases of subacute combined degeneration[J].Nao Yu Shen Jing Ji Bing Za Zhi,2016, 24:513-518.[高金立,刘尖尖,高蕾,李卫东,张颖,杜铁英,黄光.亚急性联合变性不典型性三例分析[J].脑与神经疾病杂志,2016, 24:513-518.]
[13] Bi HY, Zhang Q, Zhao YM, Li JM, Zhang YB. The clinical analysis of 24 patients with subacute combined degeneration of the spinal cord[J]. Zhongguo Shen Jing Mian Yi Xue He Shen Jing Bing Xue Za Zhi,2014, 21:254-256.[毕鸿雁,张芹,赵亚明,李继梅,张拥波.脊髓亚急性联合变性24例临床分析[J].中国神经免疫学和神经病学杂志,2014, 21:254-256.]
[14] Li J, Zhang L, Zhang Y, Deng B, Bi X. Misdiagnosis of spinal subacute combined degeneration in a patient with elevated serum B_(12)concentration and sensory deficit level[J]. Neurol Sci, 2016, 37:1577-1578.
[15] Schrempf W, Eulitz M, Neumeister V, Siegert G, Koch R,Reichmann H, Storch A. Utility of measuring vitamin B_(12)and its active fraction, holotranscobalamin, in neurological vitamin B_(12)deficiency syndromes[J]. J Neurol, 2011, 258:393-401.
[16] Matsuura H, Nakamura T. Inverted V sign:subacute combined degeneration of the spinal cord[J]. QJM, 2018, 111:65-66.
[17] Sun HY, Lee JW, Park KS, Wi JY, Kang HS. Spine MR imaging features of subacute combined degeneration patients[J]. Eur Spine J, 2014, 23:1052-1058.
[18] Jain KK, Malhotra HS, Garg RK, Gupta PK, Roy B, Gupta RK.Prevalence of MR imaging abnormalities in vitamin B_(12)deficiency patients presenting with clinical features of subacute combined degeneration of the spinal cord[J]. J Neurol Sci,2014, 342(1/2):162-166.
[19] Jiang M, Hu HT, Yan X, Pang Y, Yan LR, Ma ZG, Guo XL,Chen J, Han YT, Qin ZH, Tang S, Gao Q, You XF. Differences of abnomal MRI and clinical manifestations in different periods of subacute combined degeneration in different periods on the rate of abnormal MRI and clinical manifestations[J]. Zhong Feng Yu Shen Jing Ji Bing Za Zhi,2013, 30:222-225.[姜明,胡洪涛,闫欣,庞英,阎立荣,马志刚,郭笑磊,陈捷,韩仰同,秦朝晖,唐澍,高倩,尤小凡.亚急性联合变性病程不同时期MRI上病灶出现率及临床表现的差别[J].中风与神经疾病杂志,2013, 30:222-225.]
[20] Vasconcelos OM, Poehm EH, Mccarter RJ, Campbell WW,Quezado ZM. Potential outcome factors in subacute combined degeneration:review of observational studies[J]. J Gen Intern Med, 2006, 21:1063-1068.