GAD-65抗体相关性小脑性共济失调病例报道及文献回顾
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脑与神经疾病杂志 2017 年第 25 卷第 7 期
Hale Waihona Puke Baidu
397
·论 著·
GAD-65 抗体相关性小脑性共济失调病例报道 及文献回顾
刘 峥 董会卿 邱占东 段云云 黄 靖 刘亚欧
【摘要】 目的 探讨 GAD-65 抗体相关性小脑性共济失调的临床、血清学特点和预后。方法 报道 1 例抗 GAD-65 抗体相关性小脑性共济失调患者， 分析其临床和血清学特征。结果 患者中年女性， 急性起病， 波动性病程， 阶段性加重， 主要表现为间断发作的眩晕， 以及持续存在的复视、 不对称性的右侧肢体共济失调， 共济失调在头晕发作时加重。查体： 右睑下垂， 双眼上视受限， 下视时垂直眼震， 右下肢肌张力略低， 肌力 5级，右手指鼻稍欠稳准，右侧跟膝胫试验欠稳准，直线行走不能完成，双侧 Barbinski 征阴性。头颅 MRI 示 右侧小脑蚓部和半球萎缩 ； 脑脊液（CSF）SOB 阳性 ； 脑脊液和血清 GAD-65 抗体强阳性 ； 激素免疫治疗后 患者头晕消失，眼动异常和共济失调部分好转。结论 GAD-65 抗体相关性共济失调是免疫介导性小脑性共 济失调的一种特殊类型，表现为急性或亚急性起病的波动性进展的小脑性共济失调，可累及脑干。并可合 并其他自身免疫病，很少与肿瘤共病。血清学检测和尽早免疫治疗可使患者受益。 【关键词】 共济失调 ； 小脑 ； 谷氨酸脱羧酶 ； 抗体 中图分类号 ： R744.7 文献标识码 ： A 文章编号 ： 1006-351X（2017）07-0397-05 GAD-65 antibody-related cerebellar ataxia: a case report and review of the literature Medical University, Beijing 100053, China Corresponding author: LIU Zheng , Email: lzwcy2003@aliyun.com 【Abstract】 Objective To explore the clinical and serological features of GAD-65 antibody-related cerebellar ataxia. Method An rare case of a forty-five year-old female with GAD-65 antibody-positive cerebellar ataxia was reported. Her clinical presentation and serological findings were discussed. The related literatures have been reviewed. Results The patient presented with paroxysmal vertigo for 42 months and asymmetric right-limbs ataxia and diplopia for 36 month. Neurological examination found limited movements of bilateral upward looking and downbeat vertical nystagmus, mildly decreased muscle tone and strength of right lower limb, slight dysmetria while performing rightsided finger-nose and heel-knee-shin tests and unbalanced tandem gait. Laboratory test for the serological anti-GAD-65 antibody of serum and cerebrospinal fluid（CSF）were positive. Cerebral MRI revealed slightly atrophy of right cerebellar hemisphere and vermis. She was diagnosed as GAD-65 antibody-related cerebellar ataixa, and got partial relief after steroid treatment. Conclusion GAD-65 antibody-related cerebellar ataixa is a special type of immunemediated cerebellar ataxias, which can be progressive ataxia or fluctuatedly progressive ataxia with acute, subacute or chronic onset. It can be asymmetric, affect brainstem and combine other autoimmune disorders. Immune treatment can make patients partially relieved. 【Key words】 Ataxia; Cerebella; Glutamic acid decarboxylase; Antibody LIU Zheng, DONG Hui-qing,
QIU Zhan-dong, DUAN Yun-yun, HUANG Jing, LIU Ya-ou. Department of Neurology, the Xuanwu Hospital, Capital
小 脑 是 自 身 免 疫 病 的 靶 器 官， 发 生 免 疫 介 导 的 小 脑 性 共 济 失 调［1］ 。2016 年 日 本、 印 度、 英
Hale Waihona Puke Baidu
397
·论 著·
GAD-65 抗体相关性小脑性共济失调病例报道 及文献回顾
刘 峥 董会卿 邱占东 段云云 黄 靖 刘亚欧
【摘要】 目的 探讨 GAD-65 抗体相关性小脑性共济失调的临床、血清学特点和预后。方法 报道 1 例抗 GAD-65 抗体相关性小脑性共济失调患者， 分析其临床和血清学特征。结果 患者中年女性， 急性起病， 波动性病程， 阶段性加重， 主要表现为间断发作的眩晕， 以及持续存在的复视、 不对称性的右侧肢体共济失调， 共济失调在头晕发作时加重。查体： 右睑下垂， 双眼上视受限， 下视时垂直眼震， 右下肢肌张力略低， 肌力 5级，右手指鼻稍欠稳准，右侧跟膝胫试验欠稳准，直线行走不能完成，双侧 Barbinski 征阴性。头颅 MRI 示 右侧小脑蚓部和半球萎缩 ； 脑脊液（CSF）SOB 阳性 ； 脑脊液和血清 GAD-65 抗体强阳性 ； 激素免疫治疗后 患者头晕消失，眼动异常和共济失调部分好转。结论 GAD-65 抗体相关性共济失调是免疫介导性小脑性共 济失调的一种特殊类型，表现为急性或亚急性起病的波动性进展的小脑性共济失调，可累及脑干。并可合 并其他自身免疫病，很少与肿瘤共病。血清学检测和尽早免疫治疗可使患者受益。 【关键词】 共济失调 ； 小脑 ； 谷氨酸脱羧酶 ； 抗体 中图分类号 ： R744.7 文献标识码 ： A 文章编号 ： 1006-351X（2017）07-0397-05 GAD-65 antibody-related cerebellar ataxia: a case report and review of the literature Medical University, Beijing 100053, China Corresponding author: LIU Zheng , Email: lzwcy2003@aliyun.com 【Abstract】 Objective To explore the clinical and serological features of GAD-65 antibody-related cerebellar ataxia. Method An rare case of a forty-five year-old female with GAD-65 antibody-positive cerebellar ataxia was reported. Her clinical presentation and serological findings were discussed. The related literatures have been reviewed. Results The patient presented with paroxysmal vertigo for 42 months and asymmetric right-limbs ataxia and diplopia for 36 month. Neurological examination found limited movements of bilateral upward looking and downbeat vertical nystagmus, mildly decreased muscle tone and strength of right lower limb, slight dysmetria while performing rightsided finger-nose and heel-knee-shin tests and unbalanced tandem gait. Laboratory test for the serological anti-GAD-65 antibody of serum and cerebrospinal fluid（CSF）were positive. Cerebral MRI revealed slightly atrophy of right cerebellar hemisphere and vermis. She was diagnosed as GAD-65 antibody-related cerebellar ataixa, and got partial relief after steroid treatment. Conclusion GAD-65 antibody-related cerebellar ataixa is a special type of immunemediated cerebellar ataxias, which can be progressive ataxia or fluctuatedly progressive ataxia with acute, subacute or chronic onset. It can be asymmetric, affect brainstem and combine other autoimmune disorders. Immune treatment can make patients partially relieved. 【Key words】 Ataxia; Cerebella; Glutamic acid decarboxylase; Antibody LIU Zheng, DONG Hui-qing,
QIU Zhan-dong, DUAN Yun-yun, HUANG Jing, LIU Ya-ou. Department of Neurology, the Xuanwu Hospital, Capital
小 脑 是 自 身 免 疫 病 的 靶 器 官， 发 生 免 疫 介 导 的 小 脑 性 共 济 失 调［1］ 。2016 年 日 本、 印 度、 英