多发性硬化19例临床分析并文献复习
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摘要
目的探讨多发性硬化(MS)的临床特点及诊治要点。
方法对我院2001年12月-2009年1月收治的19例多发性硬化患者临床表现、实验室检查及治疗方法进行回顾性分析,并复习国内外相关文献报道。
结果本组患者发病年龄范围18-61岁,平均年龄35.6岁。其中有1例患者小于20岁。急性和亚急性起病者14例(73.7%),慢性起病者5例(26.3%)。首次发病者5例,复发-缓解型12例,原发进展型2例。首发症状：视力减退5例,复视3例,肢体无力11例,感觉障碍13例,共济失调4例,尿便障碍4例,头晕5例,头晕伴恶心呕吐1例,言语不利4例。辅助检查：脑脊液检查,压力正常者15例,4例脑脊液蛋白轻度增高,17例脑脊液IgG增高,行寡克隆区带检查6例,异常2例,行抗髓鞘碱性蛋白检查6例,增高4例。14例行诱发电位检查,VEP异常6例(42.9%),BAEP异常4例(21.1%),SEP异常9例(47.3%),VEP异常中有1例(16.7%)为亚临床病灶。磁共振成像检查,16例头颅MRI检查结果有异常,表现为白质内多发长T1、长T2点状、斑片状或类圆形病灶,位于侧脑室周围、额顶叶白质、脑干、小脑等处。其中6例行颈、胸椎MRI检查,4例脊髓有点状或条索状病灶。脑活检1例。入院前误诊3例,均误诊为脑梗死,经完善相关辅助检查明确诊断为多发性硬化。急性期治疗以激素冲击治疗为主。临床疗效评价采用EDSS,19例患者中基本治愈3例,明显缓解11例,总有效率73.7。
结论1.多发性硬化多发生于青壮年女性,详细询问病史,结合症状、体征以及脑脊液、诱发电位、MRI等检查,严格按照McDonald诊断标准确诊不难。2.对于急性期MS激素冲击治疗疗效肯定,对缓解期预防复发尚无很好的治疗方法。3.诱发电位、MRI等检查对于MS的早期诊断有很重要的价值。
Objective To investigate clinical features and treatment points Of multiple sclerosis (MS).
Methods Reviewing medical records of ten cases at our hospital over a period of 8 years and combinating with reported literature at home and abroad.
Results Age of onset in this group of patients ranged from 18 to 61 years, mean age 35.6 years. Among them,1 patient less than 20 years old. Acute and subacute onset in 14 cases (73.7%), chronic onset in 5 cases (26.3%).5 patients the first attack, relapsing-remitting in 12 cases,2 cases of primary progressive. Initial symptoms:5 cases of vision loss, diplopia in 3 cases, 11 cases of limb weakness, sensory disturbance in 13 cases,4 cases of ataxia, urine will be obstacles in 4 cases,5 cases of dizziness, dizziness associated with nausea and vomiting in 1 case, negative speech 4 cases. Supplementary examination:cerebrospinal fluid, normal pressure in 15 cases,4 cases of CSF protein elevation,17 patients with elevated CSF IgG, oligoclonal bands inspection line 6 cases, abnormal in 2 cases, anti-myelin basic protein examination in 6 cases increased in 4 cases.14 cases evoked potential, VEP abnormalities in 6 cases (42.9%), BAEP abnormalities in 4 cases (21.1%), SEP abnormalities in 9 cases (47.3%), VEP abnormalities in 1 case (16.7%) were subclinical lesions. Magnetic resonance imaging scan,16 cases with cranial MRI scans were abnormal, showing multiple white matter long T1, long T2 punctate, patchy or oval lesion in the periventricular, frontal white matter, brain stem, cerebellum etc..6 cases in which cervical, thoracic MRI, four cases of spinal cord or cords a little bit like lesions. Brain biopsy in 1 case.3 cases were misdiagnosed before admission, were misdiagnosed as cerebral infarction, by improving the relevant auxiliary examination diagnosed as multiple sclerosis. Acute treatment with corticosteroid therapy. Clinical Evaluation of use of EDSS,19 patients were cured in 3 cases, relieved in 11 cases, the total effective rate of 73.7.
Conclusion 1. Multiple sclerosis occurs in many young women, detailed history, combined with symptoms, signs, and cerebrospinal fluid, evoked potentials, MRI, etc., and strictly in accordance with the McDonald diagnostic criteria for diagnosis is not difficult.2. For the acute phase of MS steroid pulse therapy is effective and, on remission to prevent relapse is no good treatment.3. Evoked potential, MRI and other tests for early diagnosis of MS has important value.
方法对我院2001年12月-2009年1月收治的19例多发性硬化患者临床表现、实验室检查及治疗方法进行回顾性分析,并复习国内外相关文献报道。
结果本组患者发病年龄范围18-61岁,平均年龄35.6岁。其中有1例患者小于20岁。急性和亚急性起病者14例(73.7%),慢性起病者5例(26.3%)。首次发病者5例,复发-缓解型12例,原发进展型2例。首发症状：视力减退5例,复视3例,肢体无力11例,感觉障碍13例,共济失调4例,尿便障碍4例,头晕5例,头晕伴恶心呕吐1例,言语不利4例。辅助检查：脑脊液检查,压力正常者15例,4例脑脊液蛋白轻度增高,17例脑脊液IgG增高,行寡克隆区带检查6例,异常2例,行抗髓鞘碱性蛋白检查6例,增高4例。14例行诱发电位检查,VEP异常6例(42.9%),BAEP异常4例(21.1%),SEP异常9例(47.3%),VEP异常中有1例(16.7%)为亚临床病灶。磁共振成像检查,16例头颅MRI检查结果有异常,表现为白质内多发长T1、长T2点状、斑片状或类圆形病灶,位于侧脑室周围、额顶叶白质、脑干、小脑等处。其中6例行颈、胸椎MRI检查,4例脊髓有点状或条索状病灶。脑活检1例。入院前误诊3例,均误诊为脑梗死,经完善相关辅助检查明确诊断为多发性硬化。急性期治疗以激素冲击治疗为主。临床疗效评价采用EDSS,19例患者中基本治愈3例,明显缓解11例,总有效率73.7。
结论1.多发性硬化多发生于青壮年女性,详细询问病史,结合症状、体征以及脑脊液、诱发电位、MRI等检查,严格按照McDonald诊断标准确诊不难。2.对于急性期MS激素冲击治疗疗效肯定,对缓解期预防复发尚无很好的治疗方法。3.诱发电位、MRI等检查对于MS的早期诊断有很重要的价值。
Objective To investigate clinical features and treatment points Of multiple sclerosis (MS).
Methods Reviewing medical records of ten cases at our hospital over a period of 8 years and combinating with reported literature at home and abroad.
Results Age of onset in this group of patients ranged from 18 to 61 years, mean age 35.6 years. Among them,1 patient less than 20 years old. Acute and subacute onset in 14 cases (73.7%), chronic onset in 5 cases (26.3%).5 patients the first attack, relapsing-remitting in 12 cases,2 cases of primary progressive. Initial symptoms:5 cases of vision loss, diplopia in 3 cases, 11 cases of limb weakness, sensory disturbance in 13 cases,4 cases of ataxia, urine will be obstacles in 4 cases,5 cases of dizziness, dizziness associated with nausea and vomiting in 1 case, negative speech 4 cases. Supplementary examination:cerebrospinal fluid, normal pressure in 15 cases,4 cases of CSF protein elevation,17 patients with elevated CSF IgG, oligoclonal bands inspection line 6 cases, abnormal in 2 cases, anti-myelin basic protein examination in 6 cases increased in 4 cases.14 cases evoked potential, VEP abnormalities in 6 cases (42.9%), BAEP abnormalities in 4 cases (21.1%), SEP abnormalities in 9 cases (47.3%), VEP abnormalities in 1 case (16.7%) were subclinical lesions. Magnetic resonance imaging scan,16 cases with cranial MRI scans were abnormal, showing multiple white matter long T1, long T2 punctate, patchy or oval lesion in the periventricular, frontal white matter, brain stem, cerebellum etc..6 cases in which cervical, thoracic MRI, four cases of spinal cord or cords a little bit like lesions. Brain biopsy in 1 case.3 cases were misdiagnosed before admission, were misdiagnosed as cerebral infarction, by improving the relevant auxiliary examination diagnosed as multiple sclerosis. Acute treatment with corticosteroid therapy. Clinical Evaluation of use of EDSS,19 patients were cured in 3 cases, relieved in 11 cases, the total effective rate of 73.7.
Conclusion 1. Multiple sclerosis occurs in many young women, detailed history, combined with symptoms, signs, and cerebrospinal fluid, evoked potentials, MRI, etc., and strictly in accordance with the McDonald diagnostic criteria for diagnosis is not difficult.2. For the acute phase of MS steroid pulse therapy is effective and, on remission to prevent relapse is no good treatment.3. Evoked potential, MRI and other tests for early diagnosis of MS has important value.
引文
[1]Poser CM,Party DW,Scheinber L,et al.New diagnostic criteria for multiple sclerosis:guidelines for research protocols.Ann Neurol,1983,33(2):227-231
[2]Stuve O,Youssef S,Steinman L,et al.Statins as potenttherapeutic agents in neuro-inflammatory.Curt Opin Neuro,2003,16(3):393
[3]Fisher JB,Jaeobs DA,Markowitz CE,et al.Relion of viual function to retinal nerve fiber layer thickness in multiple sclemsis.Ophthalmology,2006,113(2):324
[4]Krakauer M,Schaldemose NH,Jensen J,et al.Intrathecal synthesis of free immunoglobulin light chains in multiple sclerosis.Acta Neurol Scand,1998,98(3):161-165
[5]Grdinier MV, Amiguet P, Linington C, et al.Myelinoligodendrocyte glycoprotein is unique member of the immunoglibulin superfamily. J Neurosci Res,1992,33:177-87
[6]Egg R,Reindl M,Deisenhammer F,et al.Anti-MOG and anti-MBP antibody subclasses in multiple sclerosis.Multscler,2001,7(5):285-289
[7]McDonald WI,Compston A,Edan G,et al.Recommended dianostic criteria for multiple sclerosis:guidelines from the International Panel on the diagnosis of multiple sclerosis.Ann neurol,2001,50(1):121-127
[8]中华医学会神经病学分会.中国多发性硬化及相关中枢神经系统脱髓鞘疾病的诊断和治疗专家共识(草案).中华神经科杂志,2006,39：862-864
[9]陈海,江先娣,杨职,等.甲泼尼龙与丙种球蛋白联合治疗多发性硬化急性期的疗效.广东医学,2006,27(1)：121-123
[10]PRISMS(Prevention of Relapses and Disability by Interferon beta-la Subcutaneously in Multiple Sclerosis)Study Group. Randomised double blind placebo-controlled study of interferon beta-la in relapsing/remitting multiple sclerosis[J]. Lancet,1998,352(9139):1498-1504
[1]Belka C, BudachW, Kortmann RD, et a.l Radiation induced CNS toxicity-molecular and cellular mechanisms.BrJCancer,2001,85(9):1233-1239
[2]Baumann N, Pham-Dinh D. Biology of oligodendrocyte and myelin in the mammalian central nervous system.PhysiolRev,2001,81(2):871-927
[3]DawsonMR, PolitoA, Levine JM, et a.l NG2-expressing glial progenitor cells:an abundantand widespread population of cycling cells in the adult rat CNS.MolCellNeurosci,2003,24(2):476-488
[4]Sortwell CE, Daley BF, PitzerMR, et a.l Oligodendrocyte-type 2 astrocyte-derived trophic factors increase survival of developing do pamine neurons through the inhibition of apoptotic cell death.J CompNeurol,2000, 426(1):143-153
[5]Du Y, Dreyfus CF.Oligodendrocytes as providers of growth factors.JNeurosciRes,2002,68(6):647-654
[6]Wilkins A, Majed H, Layfield R, et al. Oligodendrocytes promote neuronal survival and axonal length by distinct intracellular mechanisms:a novel role for oligodendrocyte-derived glial cell line-derived neurotrophic factor. J Neurosci,2003,23:4967-4974
[7]Chen ZJ, UghrinY, Levine JM. Inhibition of axon growth by oligodendrocyte precursor
[8]cells.Mol CellNeurosci,2002,20 (1):125-139
[9]Mckerracher L, Winton MJ. Nogo on the go. Neuron,2002,36:345-348
[10]Hafler D A.Slavik J M.Anderson D E Multiple sclerosis,2005,204:208-31
[11]Lucchinetti CF, BruckW, Parisi J, et al. A quantitative analysis of oligodendrocytes in multiple sclerosis lesions:a study of 113 cases. Brain,2000,122 2279-2295
[12]Kurosinski P, G"otz J. Glial cells under physiologic and pathologic conditions.ArchNeurol,2002,59(10): 1524-1528
[13]Wolswijk G. Oligodendrocyte precursor cells in the demylinated multiple sclerosis spinal cord.Brain,2002, 125(2):338-349
[14]Dharmasaroja P. Specificity of autoantibodies to epitopes of myelin proteins in multiple sclerosis. J Neurol Sci,2003,206:7-16
[15]Panoutsakopoulou V, Huster KM, McCarty N,etal.Suppression of autoimmune disease after vaccination with autoreactive T cells that express Qa-1 peptide complexes. J Clin Invest,2004,113(8):1218-1224
[16]Stienekemeier M, Falk K, Rotzschke O,et al. Vaccination, prevention, and treatment of experimental autoimmune neuritis (EAN) by an oligomerized T cell epitope. Proc Natl Acad Sci USA, 2001;98(24):13872-13877
[17]Levince MC,Lee SM,Kalume F,et al.Autoimmunity due to molecular mimicry as a cause of neurological disease.Nat Med 2002;8(5):509-13
[18]Buntinx M,Ameloot M,Steels P,et al.Interferon-gamma-induced calcium influx in T lymphocytes of multiple sclerosis and rheumatoid arthritis patients:a complementary mechanism for T cell activation?J Neuroimmunol 2002;124(1-2):70-82
[19]T. Zeis & N. Schaeren-Wiemers Lame Ducks or Fierce Creatures?-The Role of Oligodendrocytes in Multiple Sclerosis J Mol Neurosci 2008; 35:91-100
[20]Zipp F.Apoptosis in multiple sclerosis.Cell Tissue Res 2000;301(1):163-71
[21]Gutcher I, BecherB.APC-derived cytokines andT cellpolarization in autoimmune inflammation. J Clin Invest,2007,117 (5):1119-1127
[22]Hartung HP,Pollard JD,Harvey GK, et al. Immunopathogenesis and treatment of the Guillain-Barresyndrome. Part Ⅰ, Ⅱ. Muscle Nerve 1995;18:137-164
[23]Gardinier MV, Amiguet P, Linington C, et al.Myelinoligodendrocyte glycoprotein is unique member of the immunoglibulin superfamily. J Neurosci Res,1992,33:177-87
[2]Stuve O,Youssef S,Steinman L,et al.Statins as potenttherapeutic agents in neuro-inflammatory.Curt Opin Neuro,2003,16(3):393
[3]Fisher JB,Jaeobs DA,Markowitz CE,et al.Relion of viual function to retinal nerve fiber layer thickness in multiple sclemsis.Ophthalmology,2006,113(2):324
[4]Krakauer M,Schaldemose NH,Jensen J,et al.Intrathecal synthesis of free immunoglobulin light chains in multiple sclerosis.Acta Neurol Scand,1998,98(3):161-165
[5]Grdinier MV, Amiguet P, Linington C, et al.Myelinoligodendrocyte glycoprotein is unique member of the immunoglibulin superfamily. J Neurosci Res,1992,33:177-87
[6]Egg R,Reindl M,Deisenhammer F,et al.Anti-MOG and anti-MBP antibody subclasses in multiple sclerosis.Multscler,2001,7(5):285-289
[7]McDonald WI,Compston A,Edan G,et al.Recommended dianostic criteria for multiple sclerosis:guidelines from the International Panel on the diagnosis of multiple sclerosis.Ann neurol,2001,50(1):121-127
[8]中华医学会神经病学分会.中国多发性硬化及相关中枢神经系统脱髓鞘疾病的诊断和治疗专家共识(草案).中华神经科杂志,2006,39：862-864
[9]陈海,江先娣,杨职,等.甲泼尼龙与丙种球蛋白联合治疗多发性硬化急性期的疗效.广东医学,2006,27(1)：121-123
[10]PRISMS(Prevention of Relapses and Disability by Interferon beta-la Subcutaneously in Multiple Sclerosis)Study Group. Randomised double blind placebo-controlled study of interferon beta-la in relapsing/remitting multiple sclerosis[J]. Lancet,1998,352(9139):1498-1504
[1]Belka C, BudachW, Kortmann RD, et a.l Radiation induced CNS toxicity-molecular and cellular mechanisms.BrJCancer,2001,85(9):1233-1239
[2]Baumann N, Pham-Dinh D. Biology of oligodendrocyte and myelin in the mammalian central nervous system.PhysiolRev,2001,81(2):871-927
[3]DawsonMR, PolitoA, Levine JM, et a.l NG2-expressing glial progenitor cells:an abundantand widespread population of cycling cells in the adult rat CNS.MolCellNeurosci,2003,24(2):476-488
[4]Sortwell CE, Daley BF, PitzerMR, et a.l Oligodendrocyte-type 2 astrocyte-derived trophic factors increase survival of developing do pamine neurons through the inhibition of apoptotic cell death.J CompNeurol,2000, 426(1):143-153
[5]Du Y, Dreyfus CF.Oligodendrocytes as providers of growth factors.JNeurosciRes,2002,68(6):647-654
[6]Wilkins A, Majed H, Layfield R, et al. Oligodendrocytes promote neuronal survival and axonal length by distinct intracellular mechanisms:a novel role for oligodendrocyte-derived glial cell line-derived neurotrophic factor. J Neurosci,2003,23:4967-4974
[7]Chen ZJ, UghrinY, Levine JM. Inhibition of axon growth by oligodendrocyte precursor
[8]cells.Mol CellNeurosci,2002,20 (1):125-139
[9]Mckerracher L, Winton MJ. Nogo on the go. Neuron,2002,36:345-348
[10]Hafler D A.Slavik J M.Anderson D E Multiple sclerosis,2005,204:208-31
[11]Lucchinetti CF, BruckW, Parisi J, et al. A quantitative analysis of oligodendrocytes in multiple sclerosis lesions:a study of 113 cases. Brain,2000,122 2279-2295
[12]Kurosinski P, G"otz J. Glial cells under physiologic and pathologic conditions.ArchNeurol,2002,59(10): 1524-1528
[13]Wolswijk G. Oligodendrocyte precursor cells in the demylinated multiple sclerosis spinal cord.Brain,2002, 125(2):338-349
[14]Dharmasaroja P. Specificity of autoantibodies to epitopes of myelin proteins in multiple sclerosis. J Neurol Sci,2003,206:7-16
[15]Panoutsakopoulou V, Huster KM, McCarty N,etal.Suppression of autoimmune disease after vaccination with autoreactive T cells that express Qa-1 peptide complexes. J Clin Invest,2004,113(8):1218-1224
[16]Stienekemeier M, Falk K, Rotzschke O,et al. Vaccination, prevention, and treatment of experimental autoimmune neuritis (EAN) by an oligomerized T cell epitope. Proc Natl Acad Sci USA, 2001;98(24):13872-13877
[17]Levince MC,Lee SM,Kalume F,et al.Autoimmunity due to molecular mimicry as a cause of neurological disease.Nat Med 2002;8(5):509-13
[18]Buntinx M,Ameloot M,Steels P,et al.Interferon-gamma-induced calcium influx in T lymphocytes of multiple sclerosis and rheumatoid arthritis patients:a complementary mechanism for T cell activation?J Neuroimmunol 2002;124(1-2):70-82
[19]T. Zeis & N. Schaeren-Wiemers Lame Ducks or Fierce Creatures?-The Role of Oligodendrocytes in Multiple Sclerosis J Mol Neurosci 2008; 35:91-100
[20]Zipp F.Apoptosis in multiple sclerosis.Cell Tissue Res 2000;301(1):163-71
[21]Gutcher I, BecherB.APC-derived cytokines andT cellpolarization in autoimmune inflammation. J Clin Invest,2007,117 (5):1119-1127
[22]Hartung HP,Pollard JD,Harvey GK, et al. Immunopathogenesis and treatment of the Guillain-Barresyndrome. Part Ⅰ, Ⅱ. Muscle Nerve 1995;18:137-164
[23]Gardinier MV, Amiguet P, Linington C, et al.Myelinoligodendrocyte glycoprotein is unique member of the immunoglibulin superfamily. J Neurosci Res,1992,33:177-87
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