关鸿志;陈琳;崔丽英;管宇宙;李秀丽;钱敏;任海涛;赵燕环;
GUAN Hong-zhi;CHEN Lin;CUI Li-ying;GUAN Yu-zhou;LI Xiu-li;QIAN Min;REN Hai-tao;ZHAO Yan-huan;Department of Neurology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences &Peking Union Medical College;

• 1:中国医学科学院 北京协和医学院 北京协和医院神经科

摘要(Abstract)：

目的探讨吉兰-巴雷综合征(Guillain-Barrésyndrome,GBS)的脑脊液(cerebrospinal fluid,CSF)细胞学特点及其诊断意义。方法回顾性分析北京协和医院2010年1月至2012年12月收治的GBS患者的临床、神经电生理和CSF检查资料。入组病例均符合《中国吉兰-巴雷综合征诊治指南》(2010年)诊断标准。CSF细胞学检查采用CSF细胞沉淀器(自然沉淀法)制片,MGG法染色。采用χ2检验比较CSF常规计数和CSF细胞学的阳性率。结果共28例GBS患者纳入研究,其中男19例,女9例,平均年龄39岁(8⁶9岁)。均急性起病,肢体无力22例,感觉减退13例,感觉过敏3例,球麻痹7例,需要呼吸机辅助通气3例,尿潴留5例,体位性低血压1例,眼肌麻痹5例,面瘫10例,共济失调4例。临床分型:急性炎性脱髓鞘性多发神经根神经病20例,急性运动轴索性神经病1例,急性运动感觉轴索性神经病1例,急性感觉神经病1例,Miller Fisher综合征4例,抗GQ1b抗体阳性的眼肌麻痹1例。CSF蛋白0.3969岁)。均急性起病,肢体无力22例,感觉减退13例,感觉过敏3例,球麻痹7例,需要呼吸机辅助通气3例,尿潴留5例,体位性低血压1例,眼肌麻痹5例,面瘫10例,共济失调4例。临床分型:急性炎性脱髓鞘性多发神经根神经病20例,急性运动轴索性神经病1例,急性运动感觉轴索性神经病1例,急性感觉神经病1例,Miller Fisher综合征4例,抗GQ1b抗体阳性的眼肌麻痹1例。CSF蛋白0.39⁴.23 g/L,蛋白升高26例,其中11例蛋白>1.0 g/L;CSF白细胞计数:(04.23 g/L,蛋白升高26例,其中11例蛋白>1.0 g/L;CSF白细胞计数:(0⁵)×106/L 26例,(65)×106/L 26例,(6¹0)×106/L 2例。CSF寡克隆区带阳性者14例,髓鞘碱性蛋白升高者18例。抗GM1抗体阳性3例,抗GQ1b抗体阳性2例。CSF细胞学检查异常12例,其中9例为淋巴细胞为主的炎症,3例为单核细胞与淋巴细胞为主的炎症,1例见2%的中性粒细胞,6例可见激活的淋巴细胞,2例可见激活的单核吞噬细胞,3例可见浆细胞。病例中CSF细胞数超过5/μl者2例(7.1%),CSF细胞学提示炎性改变者12例(42.9%),CSF细胞学阳性率高于常规细胞计数(P<0.01)。结论 GBS的CSF细胞学检查可见淋巴细胞性炎症等炎性改变,与GBS的多发性神经根炎的病理机制相一致。与CSF常规细胞计数相比,CSF细胞学能够更敏感地显示CSF的炎性状态,因此对于GBS也更具诊断意义。
Objective To investigate the cytological features of cerebrospinal fluid( CSF) in Guillain-Barrésyndrome( GBS) and the role of the cytological features in the diagnosis of GBS. Methods We reviewed the clinical,neurophysiological,and CSF cytological findings of GBS patients treated in Peking Union Medical College Hospital from January 2010 to December 2012. The included patients all met the diagnostic criteria in 2010 China Guidelines for Diagnosis and Treatment of GBS. CSF cytological tests were performed using sedimentation chamber and MGG staining. CSF routine cell count and CSF cytological-finding positive-rate were compared with Chi-squaretest. Results Twenty-eight cases of GBS were included,at a mean age of 39 years( 8- 69 years),including 19 males and 9 females. The 28 patients all had acute onset,with 22 demonstrating limb weakness,13 hypoesthesia,3 hyperesthesia,7 bulbar paralysis,3 with the need of assisted ventilation,5 urinary retention,1 positional hypotension,5 ophthalmoplegia,10 facial paralysis,and 4 ataxia. The patients were clinically classified to acute inflammatory demyelinating polyneuropathy( 20 cases),acute motor axonal neuropathy( 1 case),acute motor-sensory axonal neuropathy( 1 case),acute sensory neuropathy( 1 case),Miller Fisher syndrome( 4 cases),and GQ1 b antibody-positive ophthalmoplegia( 1 case). CSF protein was 0. 39- 4. 23 g / L,increased in 26 cases,and > 1. 0 g / L in 11 cases. CSF white blood cell count was 0 × 106- 5 × 106/ L in 26 cases,and 6 × 106- 10 × 106/ L in 2 cases. Oligoclonal band test of CSF produced positive results in 14 cases,and myelin basic protein was found increased in 18. AntiGM1 antibodies were positive in 3 cases and anti-GQ1 b antibodies in 2 cases. Abnormal CSF cytological results were found in 12 patients,including lymphocytic inflammation in 9 and monocyte-lymphocytic inflammation in 3.The percentage of neutrophils reached 2% in 1 case. Activated lymphocytes were present in 6 cases,activated mononuclear macrophages in 2,and plasma cells in 3. The CSF cell count was over 5 / μl in 2 patients( 7. 1%),significantly lower than the positive rate of CSF cytological test( 12 cases,42. 9%,P < 0. 01). Conclusions CSF cytological test in GBS could detect inflammatory changes such as lymphocyte inflammation,consistent with the pathological mechanism of polyradiculitis in GBS. Compared with CSF routine cell count,cytological test is more sensitive in revealing the inflammatory status of CSF,showing larger diagnostic value for GBS.

关键词(KeyWords)： 吉兰-巴雷综合征;脑脊液;细胞学
Guillain-Barré syndrome;cerebrospinal fluid;cytology

Abstract:

Keywords:

基金项目(Foundation):

作者(Authors): 关鸿志;陈琳;崔丽英;管宇宙;李秀丽;钱敏;任海涛;赵燕环;
GUAN Hong-zhi;CHEN Lin;CUI Li-ying;GUAN Yu-zhou;LI Xiu-li;QIAN Min;REN Hai-tao;ZHAO Yan-huan;Department of Neurology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences &Peking Union Medical College;

参考文献(References)：

• [1]中国吉兰-巴雷综合征诊治指南[J].中华神经科杂志,2010,43:583-586.
• [2]Asbury AK,Arnason BG,Adams RD.The inflammatory lesion in idiopathic polyneuritis[J].Medicine,1969,48:173-215.
• [3]关鸿志,王长华,郭玉璞,等.脑脊液细胞学的特异性发现[J].中华神经科杂志,2004,37:65-67.
• [4]Kanda T,Hayashi H,Tanabe H,et al.A fulminant case of Guillain-Barrésyndrome:topographic and fibre size related analysis of demyelinating changes[J].J Neurol Neurosurg Psychiatry,1989,52:857-864.
• [5]Fishman RA.Cerebrospinal fluid in diseases of the nervous system[M].Philadelphia:Saunders,1980:175-176.
• [6]于素贞,刘梅仕,邓小梅,等.多发性硬化和格林巴利综合征脑脊液细胞学及寡克隆区带测定的临床意义[J].脑与神经疾病杂志,1994,2:70-71.
• [7]候熙德,周善仁.临床脑脊液细胞学[M].南京:江苏科学技术出版社,1985:1-120.
• [8]Kowarik MC,Grummel V,Wemlinger S,et al.Immune cell subtyping in the cerebrospinal fluid of patients with neurological diseases[J].J Neurol,2014,261:130-143.
• [9]Irani DN.Cerebrospinal fluid in clinical practice[M].Philadelphia:Saunders,2009:121-125.
• [10]Mateen FJ,Cornblath DR,Jafari H,et al.Guillain-BarréSyndrome in India:population-based validation of the Brighton criteria[J].Vaccine,2011,29:9697-9701.
• [11]Rauschka H,Jellinger K,Lassmann H,et al.Guillain-Barrésyndrome with marked pleocytosis or a significant proportion of polymorphonuclear granulocytes in the cerebrospinal fluid:neuropathological investigation of five cases and review of differential diagnoses[J].Eur J Neurol,2003,10:479-486.
• [12]关鸿志,陈琳,郭玉璞,等.脑膜淋巴瘤的临床脑脊液细胞学研究[J].中华神经科杂志,2006,39:114-117.
• [13]关鸿志,陈琳,梁智勇,等.原发中枢神经系统淋巴瘤的脑脊液细胞学诊断[J].协和医学杂志,2012,3:273-278.
• [14]Machida H,Shinohara T,Hatakeyama N,et al.CD5-positive diffuse large B cell lymphoma infiltrating the central nervous system presenting Guillain-Barré-like syndrome after chemotherapy[J].J Clin Exp Hematop,2012,52:199-204.