BACKGROUND Miller fisher syndrome(MFS)is a variant of Guillain-Barrésyndrome,an acute immune-mediated peripheral neuropathy that is often secondary to viral infections.Anti-ganglioside antibodies play crucial rol...BACKGROUND Miller fisher syndrome(MFS)is a variant of Guillain-Barrésyndrome,an acute immune-mediated peripheral neuropathy that is often secondary to viral infections.Anti-ganglioside antibodies play crucial roles in the development of MFS.The positive rate of ganglioside antibodies is exceptionally high in MFS patients,particularly for anti-GQ1b antibodies.However,the presence of other ganglioside antibodies does not exclude MFS.CASE SUMMARY We present a 56-year-old female patient who suddenly developed right blepharoptosis and progressively worsening vision in both eyes.There were flu symptoms prior to onset,and a coronavirus disease 2019 test was positive.On physical examination,the patient exhibited bilateral extraocular muscle paralysis,weakened reflexes in both limbs,and impaired coordination.The cerebrospinal fluid examination results showed no obvious abnormalities.Bilateral peroneal nerve F-waves were not extracted.Serum anti-GD1b IgG and anti-GT1a IgG antibodies were positive.The patient received intravenous methylprednisolone(1000 mg/day),with the dosage gradually decreased.Additionally,intravenous high-dose immunoglobulin treatment was administered for 5 days(0.4 g/kg/day)from day 2 to day 6 of hospitalization.The patient’s symptoms improved after treatment with immunoglobulins and hormones.CONCLUSION Positive ganglioside antibodies may be used as supporting evidence for the diagnosis;however,the diagnosis of MFS is more reliant on clinical symptoms.展开更多
Severe acute respiratory syndrome coronavirus (SARS-CoV-2) is a newly identified member of the coronavirus family that has caused the coronavirus disease 2019 (COVID-19) pandemic. This rapidly evolving and unrelenting...Severe acute respiratory syndrome coronavirus (SARS-CoV-2) is a newly identified member of the coronavirus family that has caused the coronavirus disease 2019 (COVID-19) pandemic. This rapidly evolving and unrelenting SARS-CoV-2has disrupted the lives and livelihoods of millions worldwide. As of 23 August 2021, a total of 211,373,303 COVID-19cases have been confirmed globally with a death toll of 4,424,341. A strong understanding of the infection pathway of SARS-CoV-2, and how our immune system responds to the virus is highly pertinent for guiding the development and improvement of effective treatments. In this review, we discuss the current understanding of neutralising antibodies(NAbs) and their implications in clinical practice. The aspects include the pathophysiology of the immune response,particularly humoral adaptive immunity and the roles of NAbs from B cells in infection clearance. We summarise the onset and persistence of IgA, IgM and IgG antibodies, and we explore their roles in neutralising SARS-CoV-2, their persistence in convalescent individuals, and in reinfection. Furthermore, we also review the applications of neutralising antibodies in the clinical setting—from predictors of disease severity to serological testing to vaccinations, and finally in therapeutics such as convalescent plasma infusion.展开更多
Purpose To review recent knowledge on the clinical features, pathology and pathophysiology, diagnosis and treatment of Miller Fisher syndrome (MFS) Data sources Clinical and laboratory studies on MFS in the past 1...Purpose To review recent knowledge on the clinical features, pathology and pathophysiology, diagnosis and treatment of Miller Fisher syndrome (MFS) Data sources Clinical and laboratory studies on MFS in the past 10 years were included Results A viral infection preceded neurological symptoms in 71 8% of MFS patients Typical MFS consists of the triad of ataxia, areflexia and ophthalmoplegia Other cranial nerves are also involved, which may overlap with limb weakness in typical Guillain Barre syndrome (GBS) Lower cranial nerve variants of GBS, atypical MFS and ataxic neuropathies may overlap, and are thought of as variant forms of MFS Recurrence and CNS involvement is found more frequently in MFS than in GBS Antibody to GQ1b, a tetrasyaloganglioside (GQ1b antibody) which is found in close relation to ophthalmoplegia in MFS, is also associated with Campylobacter jejuni (C jejuni) serotype Penner 2 This suggests that C jejuni may induce MFS via the GQ1b structure The GQ1b antibody may lead to the failure of acetylcholine release from motor nerve terminals, which has been confirmed by clinical neurophysiological results Conclusions Many studies have shown similarities in the pathogenesis of MFS and GBS However, there are still some differences between them, especially in the areas of sensory and CNS involvement The GQ1b antibody is thought of as one of the key factors in the pathogenesis of MFS, especially with ophthalmoplegia, and it may prove a useful clinical marker in the diagnosis of MFS展开更多
目的:探讨着丝点蛋白B(centromere protein B,CENP-B)抗体阳性的原发性干燥综合征(primary Sj9gren’s syndrome,pSS)患者的临床和免疫学特征。方法:采用横断面研究的研究设计,回顾性纳入2016年1月至2022年8月就诊于北京大学口腔医院并...目的:探讨着丝点蛋白B(centromere protein B,CENP-B)抗体阳性的原发性干燥综合征(primary Sj9gren’s syndrome,pSS)患者的临床和免疫学特征。方法:采用横断面研究的研究设计,回顾性纳入2016年1月至2022年8月就诊于北京大学口腔医院并确诊为pSS的患者,收集分析患者的一般及临床特征、唾液腺造影、唇腺活检病理、血清免疫学及生化检查等资料。将pSS患者分为CENP-B抗体阳性组和阴性组,用SPSS 23.0软件对数据进行描述性分析、单因素及相关性分析,并将CENP-B抗体阳性组分为CENP-B抗体单阳性组及合并其他抗体阳性组进行亚组分析。结果:共纳入288例pSS患者,其中CENP-B抗体阳性组75例(26.0%),阴性组213例(74.0%)。CENP-B抗体阳性组的发病年龄较大,罹患自身免疫性肝病的比例较高,唾液腺肿大的比例较低,SSA/Ro60、Ro52、SSB抗体的阳性率均较低,免疫球蛋白G、类风湿因子的水平较低,免疫球蛋白M的水平较高,总蛋白水平较低,而白蛋白/球蛋白比值、谷丙转氨酶、谷草转氨酶、碱性磷酸酶、γ-谷氨酰转移酶、乳酸脱氢酶的水平较高。亚组分析显示,CENP-B抗体单阳性组的总蛋白、免疫球蛋白A水平低于合并其他抗体阳性组。结论:CENP-B抗体阳性的pSS患者具有独特的临床和免疫学特征,表现为疾病活动性较低,较少发生唾液腺肿大,但易罹患自身免疫性肝病,肝功能生化指标水平偏高,提示CENP-B抗体可能是pSS亚型分类的特异性标志物。展开更多
目的探讨血清抗Vinculin足细胞自身抗体在儿童特发性肾病综合征(INS)中的表达阳性率,及对INS诊断、预后的价值。方法采用ELISA方法检测80例INS患儿及320例健康儿童血清Vinculin-IgG抗体水平,根据抗体滴度水平将80例INS患儿分为抗体阳性...目的探讨血清抗Vinculin足细胞自身抗体在儿童特发性肾病综合征(INS)中的表达阳性率,及对INS诊断、预后的价值。方法采用ELISA方法检测80例INS患儿及320例健康儿童血清Vinculin-IgG抗体水平,根据抗体滴度水平将80例INS患儿分为抗体阳性组和阴性组,并对不同组病例的临床资料进行分析。结果与健康儿童相比较,INS患儿抗Vinculin抗体阳性率显著升高(30/80 vs 10/320,P<0.05),抗体阳性率与患儿激素敏感性无关(24/63 vs 6/13,P>0.05)。与抗体阴性INS患儿相比较,抗体阳性INS患儿24 h尿蛋白定量水平升高[(3200.7±1286.8)mg/24 h vs(1051.2±266.3)mg/24 h,P<0.05]。抗体阳性患儿组血清抗Vinculin抗体滴度与INS患儿24 h尿蛋白定量存在相关性(r=0.586,P<0.05),尿蛋白转阴后,抗体滴度较确诊时明显下降(P<0.05)。结论血清抗Vinculin足细胞抗体在INS早期诊断、疗效评估、预后判断中可能具有重要的临床意义。展开更多
文摘BACKGROUND Miller fisher syndrome(MFS)is a variant of Guillain-Barrésyndrome,an acute immune-mediated peripheral neuropathy that is often secondary to viral infections.Anti-ganglioside antibodies play crucial roles in the development of MFS.The positive rate of ganglioside antibodies is exceptionally high in MFS patients,particularly for anti-GQ1b antibodies.However,the presence of other ganglioside antibodies does not exclude MFS.CASE SUMMARY We present a 56-year-old female patient who suddenly developed right blepharoptosis and progressively worsening vision in both eyes.There were flu symptoms prior to onset,and a coronavirus disease 2019 test was positive.On physical examination,the patient exhibited bilateral extraocular muscle paralysis,weakened reflexes in both limbs,and impaired coordination.The cerebrospinal fluid examination results showed no obvious abnormalities.Bilateral peroneal nerve F-waves were not extracted.Serum anti-GD1b IgG and anti-GT1a IgG antibodies were positive.The patient received intravenous methylprednisolone(1000 mg/day),with the dosage gradually decreased.Additionally,intravenous high-dose immunoglobulin treatment was administered for 5 days(0.4 g/kg/day)from day 2 to day 6 of hospitalization.The patient’s symptoms improved after treatment with immunoglobulins and hormones.CONCLUSION Positive ganglioside antibodies may be used as supporting evidence for the diagnosis;however,the diagnosis of MFS is more reliant on clinical symptoms.
基金supported by the National Medical Research Council,Singapore (NMRC COVID19RF2-0002)。
文摘Severe acute respiratory syndrome coronavirus (SARS-CoV-2) is a newly identified member of the coronavirus family that has caused the coronavirus disease 2019 (COVID-19) pandemic. This rapidly evolving and unrelenting SARS-CoV-2has disrupted the lives and livelihoods of millions worldwide. As of 23 August 2021, a total of 211,373,303 COVID-19cases have been confirmed globally with a death toll of 4,424,341. A strong understanding of the infection pathway of SARS-CoV-2, and how our immune system responds to the virus is highly pertinent for guiding the development and improvement of effective treatments. In this review, we discuss the current understanding of neutralising antibodies(NAbs) and their implications in clinical practice. The aspects include the pathophysiology of the immune response,particularly humoral adaptive immunity and the roles of NAbs from B cells in infection clearance. We summarise the onset and persistence of IgA, IgM and IgG antibodies, and we explore their roles in neutralising SARS-CoV-2, their persistence in convalescent individuals, and in reinfection. Furthermore, we also review the applications of neutralising antibodies in the clinical setting—from predictors of disease severity to serological testing to vaccinations, and finally in therapeutics such as convalescent plasma infusion.
文摘Purpose To review recent knowledge on the clinical features, pathology and pathophysiology, diagnosis and treatment of Miller Fisher syndrome (MFS) Data sources Clinical and laboratory studies on MFS in the past 10 years were included Results A viral infection preceded neurological symptoms in 71 8% of MFS patients Typical MFS consists of the triad of ataxia, areflexia and ophthalmoplegia Other cranial nerves are also involved, which may overlap with limb weakness in typical Guillain Barre syndrome (GBS) Lower cranial nerve variants of GBS, atypical MFS and ataxic neuropathies may overlap, and are thought of as variant forms of MFS Recurrence and CNS involvement is found more frequently in MFS than in GBS Antibody to GQ1b, a tetrasyaloganglioside (GQ1b antibody) which is found in close relation to ophthalmoplegia in MFS, is also associated with Campylobacter jejuni (C jejuni) serotype Penner 2 This suggests that C jejuni may induce MFS via the GQ1b structure The GQ1b antibody may lead to the failure of acetylcholine release from motor nerve terminals, which has been confirmed by clinical neurophysiological results Conclusions Many studies have shown similarities in the pathogenesis of MFS and GBS However, there are still some differences between them, especially in the areas of sensory and CNS involvement The GQ1b antibody is thought of as one of the key factors in the pathogenesis of MFS, especially with ophthalmoplegia, and it may prove a useful clinical marker in the diagnosis of MFS
文摘目的探讨血清抗Vinculin足细胞自身抗体在儿童特发性肾病综合征(INS)中的表达阳性率,及对INS诊断、预后的价值。方法采用ELISA方法检测80例INS患儿及320例健康儿童血清Vinculin-IgG抗体水平,根据抗体滴度水平将80例INS患儿分为抗体阳性组和阴性组,并对不同组病例的临床资料进行分析。结果与健康儿童相比较,INS患儿抗Vinculin抗体阳性率显著升高(30/80 vs 10/320,P<0.05),抗体阳性率与患儿激素敏感性无关(24/63 vs 6/13,P>0.05)。与抗体阴性INS患儿相比较,抗体阳性INS患儿24 h尿蛋白定量水平升高[(3200.7±1286.8)mg/24 h vs(1051.2±266.3)mg/24 h,P<0.05]。抗体阳性患儿组血清抗Vinculin抗体滴度与INS患儿24 h尿蛋白定量存在相关性(r=0.586,P<0.05),尿蛋白转阴后,抗体滴度较确诊时明显下降(P<0.05)。结论血清抗Vinculin足细胞抗体在INS早期诊断、疗效评估、预后判断中可能具有重要的临床意义。
