摘要
目的探讨GAD65抗体相关性小脑共济失调临床及眼震电图(ENG)特点。方法分析1例影像学阴性GAD65抗体相关性自身免疫性小脑共济失调的临床表现及实验室指标尤其是(ENG)检查特点,结合文献回顾对病例特点综合分析。结果患者老年女性,急性起病,以头晕行走不稳为主要表现,至入院时病程已9个月,逐渐进展,查体双眼左视水平凝视性眼震及左侧肢体共济失调为主。脑脊液(CSF)SOB阳性;CSF和血清GAD65抗体阳性。头颅MRI:小脑和脑干均未见异常。ENG提示脑干、小脑眼动调节中枢病变。激素治疗后患者病情趋于稳定。结论GAD65抗体相关性小脑共济失调急性起病,波动性进展,累及小脑及脑干,可表现为不对称性偏侧共济失调,影像学可无特异性表现,激素及免疫调剂治疗可稳定病情,不能完全功能恢复。影像表现阴性时ENG作为前庭小脑系统功能性检查手段可以辅助定位。
Objective To analysis the clinical and electronystagmography(ENG)features of GAD65 antibody-assosiated cerebellar ataxia.Methods We discuss the clinical manifestations,laboratory indexes and ENG of a case of GAD65 antibod-assosiated cerebellar ataxia,and reviewing the literatures.Results The patient presented with paroxysmal vertigo and asymmetric left-limbs ataxia for 9 months.Neurological examination found gaze-evoked nystagmus and left-limbs ataxia.Laboratory test for the serological anti-GAD65 antibody of serum and cerebrospinal fluid were positive.Cerebral MRI was negative.ENG indicated the cerebellar and brainstem damage.Conclusion GAD65 antibod-assosiated cerebellar ataxia is a special type of immunemediated cerebellar ataxias,which can be progressive vertigo or asymmetric ataxia with acute,subacute or chronic onset.Cerebral MRI can be negative,for those patient ENG will be helpful.Patient tends to be stable but can not completely relieved with immune treatment.
作者
刘静
樊春秋
刘爱华
杨冬菊
王中傲
陈芳
韩建
Liu Jing;Fan Chunqiu;Liu Aihua;Yang Dongju;Wang Zhongao;Chen Fang;Han Jan(Department of Neurology,the Xuanwu Hospital,Capital Medical University,Beijing 100053,China)
出处
《脑与神经疾病杂志》
2020年第9期574-577,共4页
Journal of Brain and Nervous Diseases
