视神经脊髓炎谱系疾病39例临床分析
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摘要
目的了解视神经脊髓炎谱系疾病的临床特征,为临床医生鉴别诊断提供参考依据。方法回顾性分析本科2015年7月至2018年12月收治的39例视神经脊髓炎谱系疾病患者的临床资料。结果 39例视神经脊髓炎谱系疾病患者中,女33例,男6例;平均年龄(44.97±15.57)岁;病程1 d～8年,平均发作(2.02±1.50)次。脊髓受累36例,脊髓病灶分布于1～20椎体节段,通常分布于(6.07±4.34)椎体节段。脑脊液AQP4-IgG阳性25例,有24例行血清抗核抗体检测,8例阳性。25例行血清抗SSA及SSB抗体检测,抗SSA抗体阳性7例,抗SSB抗体阳性1例。21例行甲状腺功能检测,3例为亚临床甲亢,1例为亚临床甲减。结论视神经脊髓炎谱系疾病以中青年女性多见,呈多相病程,常累及脊髓(病灶分布通常多于3个椎体节段)。除特征性抗AQP4-IgG抗体外,还可检测到自身免疫抗体及甲状腺功能异常。部分患者脑脊液AQP4-IgG阴性,脊髓病灶不超过3个椎体节段。
Objective To analyse the clinical characteristics of neuromyelitis optica spectrum disorder(NMOSD),therefore to improve the.clinicians' s ablility of differential diagnosis of the diseases. Methods The clinical data of 39 NMOSD patients admitted to our department from July 2015 to December 2018 were retrospectively analysed. Results Among the 39 cases with NMOSD, there were 33 female and 6 male, and the average age was(44.97±15.57) year old, and the course of disease was 1 day to 8 years, and average attack was(2.02±1.50) times. There was spinal cord involvement in 36 patients, and the average length of spinal cord lesion was(6.07±4.34) vertebral segment. Twenty-five patients were tested with posivive AQP4-IgG test in cerebrospinal fluid. The serum antinuclear antibody was measured in 24 cases and detected as positive in 8 cases. The serum anti-SSA and SSB antibody were tested in 25 cases and detected as positive in 7 cased and 1 case, respectively. Twenty-one patients underwent thyroid function test, and 3 with subclinical hyperthyroidism and 1 with subclinical hypothyroidism. Conclusion NMOSD patients are common in young and middle-aged women, with a multi-phase course of disease, and often involve the spinal cord(usually cover more than 3 vertebral segments). In addition to AQP4-IgG detected in cerebrospinal fluid, other autoimmune antibodies and abnormal thyroid dysfunction are often detected in serum. However, in some patients with NMOSD, the AQP4-IgG was negative in the cerebrospinal fluid or the spinal cord lesion did not exceed 3 vertebral segments.
Objective To analyse the clinical characteristics of neuromyelitis optica spectrum disorder(NMOSD),therefore to improve the.clinicians' s ablility of differential diagnosis of the diseases. Methods The clinical data of 39 NMOSD patients admitted to our department from July 2015 to December 2018 were retrospectively analysed. Results Among the 39 cases with NMOSD, there were 33 female and 6 male, and the average age was(44.97±15.57) year old, and the course of disease was 1 day to 8 years, and average attack was(2.02±1.50) times. There was spinal cord involvement in 36 patients, and the average length of spinal cord lesion was(6.07±4.34) vertebral segment. Twenty-five patients were tested with posivive AQP4-IgG test in cerebrospinal fluid. The serum antinuclear antibody was measured in 24 cases and detected as positive in 8 cases. The serum anti-SSA and SSB antibody were tested in 25 cases and detected as positive in 7 cased and 1 case, respectively. Twenty-one patients underwent thyroid function test, and 3 with subclinical hyperthyroidism and 1 with subclinical hypothyroidism. Conclusion NMOSD patients are common in young and middle-aged women, with a multi-phase course of disease, and often involve the spinal cord(usually cover more than 3 vertebral segments). In addition to AQP4-IgG detected in cerebrospinal fluid, other autoimmune antibodies and abnormal thyroid dysfunction are often detected in serum. However, in some patients with NMOSD, the AQP4-IgG was negative in the cerebrospinal fluid or the spinal cord lesion did not exceed 3 vertebral segments.
引文
[1]Huda S,Whittam D,Bhojak M,et al. Neuromyelitis optica spectrum disorders[J].Clin Med(Lond),2019,19(2):169-176.
[2]Wingerchuk DM,Lennon VA,Lucchinetti CF,et al. The spectrum of neuromyelitis optica[J].Lancet Neurol,2007,6(9):805-815.
[3]Ciccarelli O,Cohen JA,Reingold SC,et al. Spinal cord involvement in multiple sclerosis and neuromyelitis optica spectrum disorders[J].Lancet Neurol,2019,18(2):185-197.
[4]Wingerchuk DM,Banwell B,Bennett JL,et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders[J].Neurology,2015,85(2):177-189.
[5]Sun H,Sun X,Li J,et al. Gender differences among Chinese patients with neuromyelitis optica spectrum disorders[J].Mult Scler Relat Disord,2017,17(10):5-8.
[6]贺斌,邵福源.大剂量甲泼尼龙冲击疗法治疗视神经脊髓炎的疗效观察[J].世界临床药物,2003,24(3):141-144.
[7]Uzawa A,Mori M,Kuwabara S.Neuromyelitis optica:concept,immunology and treatment[J].J Clin Neurosci,2014,21(1):12-21.
[8]Jarius S,Wildemann B.Aquaporin-4 antibodies(NMO-IgG)as a serological marker of neuromyelitis optica:a critical review of the literature[J].Brain Pathol,2013,23(6):661-683.
[9]Ungureanu A,de Seze J,Ahle G,et al. Myelin oligodendrocyte glycoprotein antibodies in neuromyelitis optica spectrum disorder[J].Rev Neurol(Paris),2018,174(10):675-679.
[10]Zarei S,Eggert J,Franqui-Dominguez L,et al. Comprehensive review of neuromyelitis optica and clinical characteristics of neuromyelitis optica patients in Puerto Rico[J].Surg Neurol Int,2018,9(1):242-242.
[11]Viswanathan S,Arip M,Mustafa N,et al.The frequency of anti-aquaporin-4 IgG antibody in neuromyelitis optica and its spectrum disorders at a single tertiary referral center in malaysia[J].Mult Scler Int,2014,doi:10.11 5 5/2014/568254.
[12]Kang ES,Min JH,Lee KH,et al.Clinical usefulness of cellbased indirect immunofluorescence assay for the detection of aquaporin-4 antibodies in neuromyelitis optica spectrum disorder[J].Ann Lab Med,2012,32(5):331-338.
[13]Granieri L,Marnetto F,Valentino P, et al. Evaluation of a multiparametric immunofluorescence assay for standardization of neuromyelitis optica serology[J].PLoS One,2012,7(6):e38896-e38896.
[14]Marignier R,Bernard-Valnet R,Giraudon P,et al. Aquaporin-4antibody-negative neuromyelitis optica:distinct assay sensitivity-dependent entity[J].Neurology,201 3,80(24):2194-2200.
[15]Shahmohammadi S,Doosti R,Shahmohammadi A,et al.Autoimmune diseases associated with neuromyelitis optica spectrum disorders:a literature review[J].Mult Scler Relat Disord,2019,27:350-363.
[16] Chen C,Xiaobo S,Yuge W,et al. Multiple autoantibodiesandneuromyelitis optica spectrum disorders[J].Neuroimmunomodulation,2016,23(3):151-156.
[17]Freitas E,Guimaraes J.Neuromyelitis optica spectrum disorders associated with other autoimmune diseases[J].Rheumatol Int,2015,35(2):243-253.
[18]Bibic VC,Brust TB,Burton JM.Neuromyelitis optica spectrum disorder presenting with concurrent autoimmune diseases[J].Mult Scler Relat Disord,2019,28:125-128.
[19]Zhong YH,Zhong ZG,Zhou Z, et al.Comparisons of presentations and outcomes of neuromyelitis optica patients with and without Sjogren's syndrome[J].Neurol Sci,2017,38(2):271-277.
[20]Katsumata Y,Kawachi I,Kawaguchi Y,et al.Semiquantitative measurement of aquaporin-4 antibodies as a possible surrogate marker of neuromyelitis optica spectrum disorders with systemic autoimmune diseases[J].Mod Rheumatol,2012,22(5):676-684.
[21]Papadopoulos MC,Bennett JL,Verkman AS.Treatment of neuromyelitis optica:state-of-the-art and emerging therapies[J].Nat Rev Neurol,2014,10(9):493-506.
[2]Wingerchuk DM,Lennon VA,Lucchinetti CF,et al. The spectrum of neuromyelitis optica[J].Lancet Neurol,2007,6(9):805-815.
[3]Ciccarelli O,Cohen JA,Reingold SC,et al. Spinal cord involvement in multiple sclerosis and neuromyelitis optica spectrum disorders[J].Lancet Neurol,2019,18(2):185-197.
[4]Wingerchuk DM,Banwell B,Bennett JL,et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders[J].Neurology,2015,85(2):177-189.
[5]Sun H,Sun X,Li J,et al. Gender differences among Chinese patients with neuromyelitis optica spectrum disorders[J].Mult Scler Relat Disord,2017,17(10):5-8.
[6]贺斌,邵福源.大剂量甲泼尼龙冲击疗法治疗视神经脊髓炎的疗效观察[J].世界临床药物,2003,24(3):141-144.
[7]Uzawa A,Mori M,Kuwabara S.Neuromyelitis optica:concept,immunology and treatment[J].J Clin Neurosci,2014,21(1):12-21.
[8]Jarius S,Wildemann B.Aquaporin-4 antibodies(NMO-IgG)as a serological marker of neuromyelitis optica:a critical review of the literature[J].Brain Pathol,2013,23(6):661-683.
[9]Ungureanu A,de Seze J,Ahle G,et al. Myelin oligodendrocyte glycoprotein antibodies in neuromyelitis optica spectrum disorder[J].Rev Neurol(Paris),2018,174(10):675-679.
[10]Zarei S,Eggert J,Franqui-Dominguez L,et al. Comprehensive review of neuromyelitis optica and clinical characteristics of neuromyelitis optica patients in Puerto Rico[J].Surg Neurol Int,2018,9(1):242-242.
[11]Viswanathan S,Arip M,Mustafa N,et al.The frequency of anti-aquaporin-4 IgG antibody in neuromyelitis optica and its spectrum disorders at a single tertiary referral center in malaysia[J].Mult Scler Int,2014,doi:10.11 5 5/2014/568254.
[12]Kang ES,Min JH,Lee KH,et al.Clinical usefulness of cellbased indirect immunofluorescence assay for the detection of aquaporin-4 antibodies in neuromyelitis optica spectrum disorder[J].Ann Lab Med,2012,32(5):331-338.
[13]Granieri L,Marnetto F,Valentino P, et al. Evaluation of a multiparametric immunofluorescence assay for standardization of neuromyelitis optica serology[J].PLoS One,2012,7(6):e38896-e38896.
[14]Marignier R,Bernard-Valnet R,Giraudon P,et al. Aquaporin-4antibody-negative neuromyelitis optica:distinct assay sensitivity-dependent entity[J].Neurology,201 3,80(24):2194-2200.
[15]Shahmohammadi S,Doosti R,Shahmohammadi A,et al.Autoimmune diseases associated with neuromyelitis optica spectrum disorders:a literature review[J].Mult Scler Relat Disord,2019,27:350-363.
[16] Chen C,Xiaobo S,Yuge W,et al. Multiple autoantibodiesandneuromyelitis optica spectrum disorders[J].Neuroimmunomodulation,2016,23(3):151-156.
[17]Freitas E,Guimaraes J.Neuromyelitis optica spectrum disorders associated with other autoimmune diseases[J].Rheumatol Int,2015,35(2):243-253.
[18]Bibic VC,Brust TB,Burton JM.Neuromyelitis optica spectrum disorder presenting with concurrent autoimmune diseases[J].Mult Scler Relat Disord,2019,28:125-128.
[19]Zhong YH,Zhong ZG,Zhou Z, et al.Comparisons of presentations and outcomes of neuromyelitis optica patients with and without Sjogren's syndrome[J].Neurol Sci,2017,38(2):271-277.
[20]Katsumata Y,Kawachi I,Kawaguchi Y,et al.Semiquantitative measurement of aquaporin-4 antibodies as a possible surrogate marker of neuromyelitis optica spectrum disorders with systemic autoimmune diseases[J].Mod Rheumatol,2012,22(5):676-684.
[21]Papadopoulos MC,Bennett JL,Verkman AS.Treatment of neuromyelitis optica:state-of-the-art and emerging therapies[J].Nat Rev Neurol,2014,10(9):493-506.