摘要
目的探讨复发性Fisher综合征(FS)叠加Bickerstaff脑干脑炎(BBE)的临床特点和疾病命名。方法分析2例复发性Fisher-Bickerstaff综合征的患者的临床资料,并复习相关文献。结果例1表现为复发性睡眠增多、走路不稳、视物成双,肌电图检查提示下肢神经源性损害;例2表现为复发性视物成双、走路不稳,第2次发病出现四肢无力,查体双下肢远端肌力3级,双侧Babinski征阳性,肌电图提示四肢神经源性损害;2例患者除具有眼肌麻痹和共济失调的症状外,都有中枢和周围神经受损的表现;依据患者的病史、查体、临床检测和疾病经过,诊断为复发性Fisher-Bickerstaff综合征,第2例患者叠加吉兰-巴雷综合征。结论具有周围神经损害的BBE和具有中枢神经损害的FS从临床表型上不能截然分开,Fisher-Bickerstaff综合征的概念更能准确地表述这类患者的表型特点。
Objective To explore the clinical manifestation and nosological status of Fisher syndrome(FS) plus Bickerstaff brainstem encephalitis(BBE).Methods We reported two cases of recurrent Fisher-Bickerstaff syndrome(FBS).The related literatures have been reviewed.Results Case 1 presented with recurrent somnolence,ataxia and diplopia.The EMG showed neurogenic lesions of two lower limbs.Case 2 presented with recurrent diplopia and ataxia.He also presented with weakness of four limbs at the second episode,and neurological examination showed distal lower limb strength with 3 on the Medical Research Council scale,hyporeflexia and bilateral extensor plantar response.The two patients had other manifestations of both CNS and PNS involvements as well as ophthalmoplegia and ataxia.They were diagnosed as recurrent FBS based on the clinical features and relative examination.The presentation of the second case overlapped with GBS.Conclusion BBE with peripheral lesion is difficult to be differentiated from FS with CNS lesion,and vice versa.Proposed Fisher-Bickerstaff syndrome may be useful for understanding the clinical continuity of FS and BBE.
出处
《脑与神经疾病杂志》
2012年第5期332-336,共5页
Journal of Brain and Nervous Diseases