Muscarinic receptors and nicotine receptors can increase free calcium ion levels in the facial nucleus via different channels following facial nerve injury. In addition, γ-aminobutyric acid A （GABAA） receptors have...Muscarinic receptors and nicotine receptors can increase free calcium ion levels in the facial nucleus via different channels following facial nerve injury. In addition, γ-aminobutyric acid A （GABAA） receptors have been shown to negatively regulate free calcium ion levels in the facial nucleus by inhibiting nicotine receptors. The present study investigated the influence of GABAA, γ-aminobutyric acid B （GABAB） and C （GABAc） receptors on muscarinic receptors in rats with facial nerve injury by confocal laser microscopy. GABAA and GABAB receptors exhibited significant dose-dependent inhibitory effects on increased muscarinic receptor-mediated free calcium ion levels following facial nerve injury. Results showed that GABAA and GABAB receptors negatively regulate muscarinic receptor effects and interplay with cholinergic receptors to regulate free calcium ion levels for facial neural regeneration.展开更多
Background： Autoimmune encephalitis associated with antibodies against γ-aminobutyric acid B receptor （GABABR） in patients with limbic encephalitis （LE） was first described in 2010. We present a series of klan C...Background： Autoimmune encephalitis associated with antibodies against γ-aminobutyric acid B receptor （GABABR） in patients with limbic encephalitis （LE） was first described in 2010. We present a series of klan Chinese patients tbr further clinical refinement. Methods： Serum and cerebrospinal fluid （CSF） samples from patients referred to the program of encephalitis and paraneoplastic syndrome of Peking Union Medical College Hospital were tested with indirect immunofluorescence. Clinical information of patients with anti-GABABR antibody positivity was retrospectively reviewed, and descriptive statistical analysis was performed. Results： All eighteen anti-GABABR antibody-positive cases had limbic syndromes, and electroencephalogram （EEG） or neuroimaging evidence fulfilled the diagnostic criteria of LE. Four patients had additional antibodies against Hu in serum and one had anti-N-methyl-d-aspartate receptor antibody in both sera and CSF. Seventeen （17/18） patients presented with new-onset refractory seizure or status epileptics. Twelve （12/18） patients had memory deficits, 11 （11/18） patients had personality change, 7 （7/18） patients had disturbance of consciousness, and 3 （3/18） patients showed cerebellar dysfunction. One patient with LE had progressive motor and sensory polyneuropathy. Lung cancer was detected in 6 （6/18） patients. Ten （10/18） patients showed abnormality in bilateral or unilateral mediotemporal region on magnetic resonance imaging. Ten （10/18） patients had temporal lobe epileptic activity with or without general slowing on EEG. Seventeen patients received immunotherapy and 15 of them showed neurological improvement. Four patients with lung cancer died within 1-12 months due to neoplastic complications. Conclusions： Our study demonstrates that most Han Chinese patients with anti-GABABR antibody-associated LE have prominent refractory epilepsy and show neurological improvement on immunotherapy. Patients with underlying lung tumor have a relatively poor prognosis. Testing for anti-GABABR antibodies is necessary for patients with possible LE or new-onset epilepsy with unknown etiology.展开更多
BACKGROUND It is not uncommon to develop autoimmune encephalitis and paraneoplastic neurological syndromes(PNS).4 kinds of antibody-positive autoimmune paraneoplastic limbic encephalitis(PLE)have not been reported.CAS...BACKGROUND It is not uncommon to develop autoimmune encephalitis and paraneoplastic neurological syndromes(PNS).4 kinds of antibody-positive autoimmune paraneoplastic limbic encephalitis(PLE)have not been reported.CASE SUMMARY PNS are distant effects of cancer on the nervous system,rather than syndromes in which cancer directly invades and metastasizes to the nerves and/or muscle tissues.If the limbic lobe system of the brain is involved,this will result in PLE.The detection of patients with PNS is challenging since tumors that cause paraneoplastic neurologic disorders are often asymptomatic,obscure,and thus easily misdiagnosed or missed.Currently,single-or double-antibody-positive paraneoplastic marginal encephalitis has been reported.However,no cases of three or more-antibody-positive cases have been reported.Here,we report a case of PLE that is anti-collapsing response-mediator protein-5,anti-neuronal nuclear antibody-type 1,anti-aminobutyric acid B receptor,and anti-glutamate deglutase positive,and address relevant literature to improve our understanding of the disease.CONCLUSION This article reports on the management of a case of PLE with four positive antibodies,a review of the literature,in order to raise awareness among clinicians.展开更多
Encephalitis associated with antibodies to gamma-aminobutyric-acid B(GABA-B)is a subgroup of autoimmune synaptic encephalitis with typical features of limbic encephalitis and small cell lung cancer(SCLC).We report a c...Encephalitis associated with antibodies to gamma-aminobutyric-acid B(GABA-B)is a subgroup of autoimmune synaptic encephalitis with typical features of limbic encephalitis and small cell lung cancer(SCLC).We report a case of anti-GABA-B receptor encephalitis in a 57-year-old man who presented with seizures,memory loss,and abnormal behavior.He developed partially neurological responses to immunotherapy,but refused comprehensive tumor screening.The symptoms were aggravated again 4 months later.Workup showed antibodies to GABA-B receptors and tumor screening revealed SCLC.It highlights the importance of early screening of underlying tumor and anti-tumor treatment in paraneoplastic cases.展开更多
1例67岁男性患者因“阵发性抽搐发作2周余”入院,诊断为抗γ-氨基丁酸B型受体(GABA_(B)R)抗体相关脑炎。入院后给予免疫治疗,治疗过程中出现癫痫持续状态、肺部感染、低蛋白血症、凝血功能异常等。临床药师结合患者病情,在免疫治疗方案...1例67岁男性患者因“阵发性抽搐发作2周余”入院,诊断为抗γ-氨基丁酸B型受体(GABA_(B)R)抗体相关脑炎。入院后给予免疫治疗,治疗过程中出现癫痫持续状态、肺部感染、低蛋白血症、凝血功能异常等。临床药师结合患者病情,在免疫治疗方案的选择上给予甲泼尼龙联合静脉注射免疫球蛋白,在需要抗感染治疗时给予美罗培南(1 g q8h ivgtt);应用多烯磷脂酰胆碱护肝,给予氨溴索联合布地奈德改善肺功能,应用低分子量肝素钙预防血栓形成,对患者的营养和电解质平衡方面进行监护,给予患者全程化监护,最终患者病情好转出院。展开更多
目的探讨儿童抗γ-氨基丁酸B(GABAB)受体脑炎的发病机制、临床特点、辅助检查以及诊断与治疗方法。方法回顾性分析我院随诊15个月的1例抗GABAB受体(GABABR)脑炎患儿的临床资料,总结患儿临床特征、影像学改变、血清(脑脊液)免疫学检查及...目的探讨儿童抗γ-氨基丁酸B(GABAB)受体脑炎的发病机制、临床特点、辅助检查以及诊断与治疗方法。方法回顾性分析我院随诊15个月的1例抗GABAB受体(GABABR)脑炎患儿的临床资料,总结患儿临床特征、影像学改变、血清(脑脊液)免疫学检查及临床诊疗经过,并分别以“自身免疫性脑炎”“γ-氨基丁酸B受体”“autoimmune encephalitis”“anti-gamma-aminobutyric acid B receptor”为检索词,分别在中国知网、万方、Pubmed数据库查询建库至2023年1月的相关文献,总结分析儿童抗GABABR脑炎的临床特点及影像学改变。结果本研究中患儿急性起病,以肌张力障碍、情绪异常为主要表现,头颅MRI提示基底节区对称性损害,血清抗GABABR抗体阳性,脑脊液抗GABABR抗体阴性,诊断为“抗GABABR脑炎”,经丙球加类固醇免疫治疗后病情缓解。检索到8篇文献,结合本研究,共11例患儿,男性居多,一线治疗主要是皮质类固醇、静注免疫球蛋白、血浆置换。11例患儿中,除1例自发缓解外,其余患儿均接受一线治疗,2例死于严重感染,其余均预后良好。结论临床上对于单纯头颅MRI基底节区对称性损害的患者,排除感染、代谢、肿瘤等常见病因后需注意鉴别抗GABABR脑炎,尽早完善自身免疫性脑炎抗体检测,及早发现并积极治疗能改善患者预后。展开更多
基金the Youth Research Foundation of Qingdao University, No. 2007
文摘Muscarinic receptors and nicotine receptors can increase free calcium ion levels in the facial nucleus via different channels following facial nerve injury. In addition, γ-aminobutyric acid A （GABAA） receptors have been shown to negatively regulate free calcium ion levels in the facial nucleus by inhibiting nicotine receptors. The present study investigated the influence of GABAA, γ-aminobutyric acid B （GABAB） and C （GABAc） receptors on muscarinic receptors in rats with facial nerve injury by confocal laser microscopy. GABAA and GABAB receptors exhibited significant dose-dependent inhibitory effects on increased muscarinic receptor-mediated free calcium ion levels following facial nerve injury. Results showed that GABAA and GABAB receptors negatively regulate muscarinic receptor effects and interplay with cholinergic receptors to regulate free calcium ion levels for facial neural regeneration.
文摘Background： Autoimmune encephalitis associated with antibodies against γ-aminobutyric acid B receptor （GABABR） in patients with limbic encephalitis （LE） was first described in 2010. We present a series of klan Chinese patients tbr further clinical refinement. Methods： Serum and cerebrospinal fluid （CSF） samples from patients referred to the program of encephalitis and paraneoplastic syndrome of Peking Union Medical College Hospital were tested with indirect immunofluorescence. Clinical information of patients with anti-GABABR antibody positivity was retrospectively reviewed, and descriptive statistical analysis was performed. Results： All eighteen anti-GABABR antibody-positive cases had limbic syndromes, and electroencephalogram （EEG） or neuroimaging evidence fulfilled the diagnostic criteria of LE. Four patients had additional antibodies against Hu in serum and one had anti-N-methyl-d-aspartate receptor antibody in both sera and CSF. Seventeen （17/18） patients presented with new-onset refractory seizure or status epileptics. Twelve （12/18） patients had memory deficits, 11 （11/18） patients had personality change, 7 （7/18） patients had disturbance of consciousness, and 3 （3/18） patients showed cerebellar dysfunction. One patient with LE had progressive motor and sensory polyneuropathy. Lung cancer was detected in 6 （6/18） patients. Ten （10/18） patients showed abnormality in bilateral or unilateral mediotemporal region on magnetic resonance imaging. Ten （10/18） patients had temporal lobe epileptic activity with or without general slowing on EEG. Seventeen patients received immunotherapy and 15 of them showed neurological improvement. Four patients with lung cancer died within 1-12 months due to neoplastic complications. Conclusions： Our study demonstrates that most Han Chinese patients with anti-GABABR antibody-associated LE have prominent refractory epilepsy and show neurological improvement on immunotherapy. Patients with underlying lung tumor have a relatively poor prognosis. Testing for anti-GABABR antibodies is necessary for patients with possible LE or new-onset epilepsy with unknown etiology.
文摘BACKGROUND It is not uncommon to develop autoimmune encephalitis and paraneoplastic neurological syndromes(PNS).4 kinds of antibody-positive autoimmune paraneoplastic limbic encephalitis(PLE)have not been reported.CASE SUMMARY PNS are distant effects of cancer on the nervous system,rather than syndromes in which cancer directly invades and metastasizes to the nerves and/or muscle tissues.If the limbic lobe system of the brain is involved,this will result in PLE.The detection of patients with PNS is challenging since tumors that cause paraneoplastic neurologic disorders are often asymptomatic,obscure,and thus easily misdiagnosed or missed.Currently,single-or double-antibody-positive paraneoplastic marginal encephalitis has been reported.However,no cases of three or more-antibody-positive cases have been reported.Here,we report a case of PLE that is anti-collapsing response-mediator protein-5,anti-neuronal nuclear antibody-type 1,anti-aminobutyric acid B receptor,and anti-glutamate deglutase positive,and address relevant literature to improve our understanding of the disease.CONCLUSION This article reports on the management of a case of PLE with four positive antibodies,a review of the literature,in order to raise awareness among clinicians.
文摘Encephalitis associated with antibodies to gamma-aminobutyric-acid B(GABA-B)is a subgroup of autoimmune synaptic encephalitis with typical features of limbic encephalitis and small cell lung cancer(SCLC).We report a case of anti-GABA-B receptor encephalitis in a 57-year-old man who presented with seizures,memory loss,and abnormal behavior.He developed partially neurological responses to immunotherapy,but refused comprehensive tumor screening.The symptoms were aggravated again 4 months later.Workup showed antibodies to GABA-B receptors and tumor screening revealed SCLC.It highlights the importance of early screening of underlying tumor and anti-tumor treatment in paraneoplastic cases.
文摘1例67岁男性患者因“阵发性抽搐发作2周余”入院,诊断为抗γ-氨基丁酸B型受体(GABA_(B)R)抗体相关脑炎。入院后给予免疫治疗,治疗过程中出现癫痫持续状态、肺部感染、低蛋白血症、凝血功能异常等。临床药师结合患者病情,在免疫治疗方案的选择上给予甲泼尼龙联合静脉注射免疫球蛋白,在需要抗感染治疗时给予美罗培南(1 g q8h ivgtt);应用多烯磷脂酰胆碱护肝,给予氨溴索联合布地奈德改善肺功能,应用低分子量肝素钙预防血栓形成,对患者的营养和电解质平衡方面进行监护,给予患者全程化监护,最终患者病情好转出院。
文摘目的探讨儿童抗γ-氨基丁酸B(GABAB)受体脑炎的发病机制、临床特点、辅助检查以及诊断与治疗方法。方法回顾性分析我院随诊15个月的1例抗GABAB受体(GABABR)脑炎患儿的临床资料,总结患儿临床特征、影像学改变、血清(脑脊液)免疫学检查及临床诊疗经过,并分别以“自身免疫性脑炎”“γ-氨基丁酸B受体”“autoimmune encephalitis”“anti-gamma-aminobutyric acid B receptor”为检索词,分别在中国知网、万方、Pubmed数据库查询建库至2023年1月的相关文献,总结分析儿童抗GABABR脑炎的临床特点及影像学改变。结果本研究中患儿急性起病,以肌张力障碍、情绪异常为主要表现,头颅MRI提示基底节区对称性损害,血清抗GABABR抗体阳性,脑脊液抗GABABR抗体阴性,诊断为“抗GABABR脑炎”,经丙球加类固醇免疫治疗后病情缓解。检索到8篇文献,结合本研究,共11例患儿,男性居多,一线治疗主要是皮质类固醇、静注免疫球蛋白、血浆置换。11例患儿中,除1例自发缓解外,其余患儿均接受一线治疗,2例死于严重感染,其余均预后良好。结论临床上对于单纯头颅MRI基底节区对称性损害的患者,排除感染、代谢、肿瘤等常见病因后需注意鉴别抗GABABR脑炎,尽早完善自身免疫性脑炎抗体检测,及早发现并积极治疗能改善患者预后。
