吉兰-巴雷综合征临床分型与预后

Subtypes and prognosis of Guillain-Barré syndrome

目的：通过对吉兰-巴雷综合征的临床分型和预后进行分析,探讨吉兰-巴雷综合征各亚型临床特点、预后及预后相关因素。方法：收集本院2006年至2013年收治的170例吉兰-巴雷综合征患者的临床资料进行分析,并根据临床表现、电生理表现分为急性炎症性脱髓鞘性多发神经病（acute inflammatory demyelinating polyneuropathy,AIDP）、急性运动轴索性神经病（acute motor axonal neuropathy,AMAN）、Miller-Fisher综合征（Miller-Fisher syndrom,MFS）、脑神经型（cranial nerve variants,CNV）、Bickerstaff脑干脑炎叠加吉兰-巴雷综合征（Bickerstaff’s brainstem encephalitis overlaps with Guillain-Barre syndrome,BBE-GBS）和其他组。采用卡方检验分析各型GBS临床特点,对随访到的134例患者采用重复测量方差分析及多因素logistic回归分析进行预后及预后相关因素的分析。结果：AIDP组97例（57%）,AMAN组37例（22%）,MFS组12例（7%）,脑神经组8例（5%）,BBE-GBS组8例（5%）,其他组8例（5%）。据各组间3个月及6个月HFGS评分比较各组间预后,AMAN组与BBE-GBS组之间3个月（1.54±1.45,2.67±1.75,F=3.291,P=0.070）与6个月（1.21±1.17,2.00±1.67,F=1.973,P=0.161）均无统计学差异;AMAN组与AIDP组3个月（1.54±1.45,1.20±1.18,F=10.332,P=0.001）、6个月（1.21±1.17,0.62±0.88,F=15.264,P=0.000）有统计学差异;MFS组（0.72±0.79）、脑神经型（0.29±0.49）6个月预后均良好（HFGS评分≤1）。Logistic回归分析显示病情达峰时HFGS评分≥3分（P〈0.000 1,OR=6.650、95%CI=2.865~15.023）,自主神经功能障碍（P=0.0435,OR=2.820、95%CI=1.031~7.715）与预后不良（HFGS评分〉1分）有关。结论：AIDP为GBS主要亚型;AMAN组和BBE-GBS组3个月与6个月预后均较AIDP组差;脑神经型GBS与MFS预后良好;重型患者、自主神经功能障碍为GBS预后不良预测因素。

Objective：To analyze the clinical subtypes and prognosis of patients with Guillain-Barré syndrome（GBS）,and to explore the clinical features and prognosis of the different variants of GBS. Methods：Patients with GBS admitted to The First Affiliated Hospital of Chongqing Medical University from 2006 to 2013 were collected and were divided into acute inflammatory demyelinating polyneuropathy（AIDP） group,acute motor axonal neuropathy（AMAN） group,Miller-Fisher syndrome（MFS） group,cranial nerve variants（CNV）,Bickerstaff’s brainstem encephalitis overlaps with Guillain-Barre syndrome（BBE-GBS） group and unclassifiable group based on clinical features and electrophysiological findings,and patients were subdivided into two groups based on Hughes functional grading scale at nadir for different severities of GBS. One hundred and thirty-four patients were followed-up for 6 months.The clinical characteristics,prognosis of different subtypes and predictors of prognosis were analyzed. Results：There were 97 cases（57%）in AIDP group,37 cases（22%）in AMAN group,12 cases（7%）in MFS group,8 cases（5%）in CNV group,8 cases（5%）in BBE-GBS group and 8 cases（5%）in the other group. HFGS score was used to assess the prognosis at 3 and 6 months. The prognosis of AMAN and BBE-GBS group at 3 months（F=3.291,P=0.070）and 6 months（F=1.973,P=0.161）had no statistical significance. Prognosis of AMAN was worse than AIDP at 3 months（F=10.332,P=0.001）and 6 months（F=15.264,P=0.000）during the follow-up,with statistically significant differences. Outcome was good in both of MFS group and cranial nerve group at 6 months（HFGS≤1）. Logistic regression analysis revealed that the HFGS scores peaked at 3 points or more（P〈0.000 1,OR=6.650,95%CI=2.865 to 15.023）and autonomic nerve dysfunction（P =0. 0435,OR =2. 820,95 % CI =1. 031 to 7. 715） were associated with poor outcome at 6 months.Conclusion：AIDP is the main subtype of GBS. Prognosis of AMAN group and BBE-GBS group is poorer than that of AIDP group at3 months and 6 months during follow-up. Prognosis is good in both cranial nerve group and MFS group. The critical patients andautonomic nerve dysfunction are predictors of poor prognosis at6 months.