Rationale:Guillain Barre syndrome(GBS)is an acute neurological illness leading to quadriparesis with respiratory involvement.It can be triggered by infections,vaccinations,surgery,trauma,transplantation and drugs.Anti...Rationale:Guillain Barre syndrome(GBS)is an acute neurological illness leading to quadriparesis with respiratory involvement.It can be triggered by infections,vaccinations,surgery,trauma,transplantation and drugs.Anti-rabies cell culture vaccines introduced to overcome the high rate of neurological complications associated with tissue based rabies vaccine,can be very rarely associated with GBS.Patient concerns:A 50-year-old female presented with acute severe upper back pain evolving into pure motor quadriparesis following administration of human diploid cell vaccine for rabies.Diagnosis:Acute motor axonal neuropathy variant of GBS following anti-rabies human diploid cell vaccine.Interventions:Intravenous high dose steroids.Outcomes:Patient recovered completely within 1 month.Lessons:Although anti-rabies cell culture vaccines are highly immunogenic and safe,they are rarely associated with GBS.Clinicians should be aware of this link because prompt diagnosis and treatment can result in complete recovery and avoid complications.展开更多
Surgery has been reported a rare cause of Guillain-Barré syndrome (GBS), but a recent retrospective study reported a much higher incidence rate for post-surgical patients. There are several case reports of GBS pr...Surgery has been reported a rare cause of Guillain-Barré syndrome (GBS), but a recent retrospective study reported a much higher incidence rate for post-surgical patients. There are several case reports of GBS presenting after cardiac surgical procedures. All these cases were diagnosed as acute inflammatory demyelinating polyradiculoneuropathy (AIDP). We described a case of acute motor axonal neuropathy (AMAN) after cardiac surgery. Clinical features were reviewed along with spinal magnetic resonance imaging (MRI) and cerebral spinal fluid (CSF) analysis. Sequential electrodiagnostic studies (EDx) were performed. This case represented a rare complication of AMAN with urinary retention after cardiac surgery.展开更多
目的为深入理解急性运动轴索型神经病(acute motor axonal neuropathy,AMAN)分子机制,以蛋白质组技术比较AMAN患者与正常对照组血清对培养的大鼠脊髓运动神经元的影响。方法原代培养脊髓运动神经元蛋白质以固相pH梯度等电聚焦为第一向,S...目的为深入理解急性运动轴索型神经病(acute motor axonal neuropathy,AMAN)分子机制,以蛋白质组技术比较AMAN患者与正常对照组血清对培养的大鼠脊髓运动神经元的影响。方法原代培养脊髓运动神经元蛋白质以固相pH梯度等电聚焦为第一向,SDS-PAGE垂直电泳为第二向进行双向电泳,图像分析软件Image Master2D Elite分析电泳图谱,MALDI-TOF/TOF串联质谱鉴定在AMAN患者血清的影响下,培养的神经元发生变化的蛋白质。结果 AMAN患者血清干预24h的脊髓运动神经元与正常对照组血清干预24h的脊髓运动神经元相比较,有13个蛋白的表达量降低,8个蛋白的表达量升高,这些蛋白与能为疾病血清中致病因子作用的靶蛋白。结论在急性运动轴索型神经病患者血清作用下,培养大鼠脊髓运动神经元的细胞凋亡相关蛋白、氧化应激蛋白、突触可塑性相关蛋白、细胞骨架蛋白表达发生变化,这些发现有助于我们深入理解该疾病的病理机制。通过进一步的研究,本文发现的差异表达蛋白质也可能对急性运动轴索型神经病的诊断治疗有一定的帮助。展开更多
急性运动轴索性神经病(Acute motor axonal neuropathy,AMAN)是吉兰-巴雷综合征(Guillain-Barre’0x09syndrome,GBS)中的一种主要分型,以脊神经前根和运动神经纤维轴索变性损害为主,属自身免疫病范畴。单唾液酸四己糖神经节苷脂钠注射...急性运动轴索性神经病(Acute motor axonal neuropathy,AMAN)是吉兰-巴雷综合征(Guillain-Barre’0x09syndrome,GBS)中的一种主要分型,以脊神经前根和运动神经纤维轴索变性损害为主,属自身免疫病范畴。单唾液酸四己糖神经节苷脂钠注射液长久以来作为神经营养药物广泛应用于神经系统疾病,该药在改善脑细胞功能,促进受损神经细胞恢复方面疗效显著,但近年来,其临床不良副反应逐渐显露,国内外陆续报道多例外源性神经节苷脂所致GBS病例。现报道2例因呼吸困难入住我院重症监护室的神经节苷脂相关性GBS患者,运动神经纤维轴索病变为其主要特点,旨在深化临床对药物性GBS的认识。展开更多
目的探讨中国南方儿童急性运动轴索性多发性神经病(acute motor axonal neuropathy,AMAN)的临床特征、电生理特点及其预后。方法对6例AMAN患儿的临床和神经电生理资料进行分析和随访。结果(1)患儿平均发病年龄为4.4岁,均来自农村,多有...目的探讨中国南方儿童急性运动轴索性多发性神经病(acute motor axonal neuropathy,AMAN)的临床特征、电生理特点及其预后。方法对6例AMAN患儿的临床和神经电生理资料进行分析和随访。结果(1)患儿平均发病年龄为4.4岁,均来自农村,多有前驱感染史(5例),发病时间无明显季节性差异。肌无力为主要首发症状,病情达高峰的平均时间为4.2 d。(2)神经电生理检查:所有患儿神经传导检测均表现为运动神经反应电位波幅显著减低,降低至(22.3%～73.4%)正常值低限,四肢远端神经受损程度较近端重(P<0.05),而上、下肢神经受损程度无明显差异(P>0.05),运动神经电位传导速度和感觉神经传导正常。全部患儿给予静脉丙种球蛋白(IVIG)治疗。(3)随访3个月至1年,4例患儿已能独立行走。结论儿童AMAN好发于农村,全年均可发病;最常见的首发症状为肌无力,多在病程早期达到疾病高峰;神经电生理对AMAN的诊断、鉴别诊断提供了客观依据;部分患儿起病后1年能恢复独立行走能力;尽早IVIG治疗有利于促进临床神经功能恢复。展开更多
文摘Rationale:Guillain Barre syndrome(GBS)is an acute neurological illness leading to quadriparesis with respiratory involvement.It can be triggered by infections,vaccinations,surgery,trauma,transplantation and drugs.Anti-rabies cell culture vaccines introduced to overcome the high rate of neurological complications associated with tissue based rabies vaccine,can be very rarely associated with GBS.Patient concerns:A 50-year-old female presented with acute severe upper back pain evolving into pure motor quadriparesis following administration of human diploid cell vaccine for rabies.Diagnosis:Acute motor axonal neuropathy variant of GBS following anti-rabies human diploid cell vaccine.Interventions:Intravenous high dose steroids.Outcomes:Patient recovered completely within 1 month.Lessons:Although anti-rabies cell culture vaccines are highly immunogenic and safe,they are rarely associated with GBS.Clinicians should be aware of this link because prompt diagnosis and treatment can result in complete recovery and avoid complications.
文摘Surgery has been reported a rare cause of Guillain-Barré syndrome (GBS), but a recent retrospective study reported a much higher incidence rate for post-surgical patients. There are several case reports of GBS presenting after cardiac surgical procedures. All these cases were diagnosed as acute inflammatory demyelinating polyradiculoneuropathy (AIDP). We described a case of acute motor axonal neuropathy (AMAN) after cardiac surgery. Clinical features were reviewed along with spinal magnetic resonance imaging (MRI) and cerebral spinal fluid (CSF) analysis. Sequential electrodiagnostic studies (EDx) were performed. This case represented a rare complication of AMAN with urinary retention after cardiac surgery.
文摘急性运动轴索性神经病(Acute motor axonal neuropathy,AMAN)是吉兰-巴雷综合征(Guillain-Barre’0x09syndrome,GBS)中的一种主要分型,以脊神经前根和运动神经纤维轴索变性损害为主,属自身免疫病范畴。单唾液酸四己糖神经节苷脂钠注射液长久以来作为神经营养药物广泛应用于神经系统疾病,该药在改善脑细胞功能,促进受损神经细胞恢复方面疗效显著,但近年来,其临床不良副反应逐渐显露,国内外陆续报道多例外源性神经节苷脂所致GBS病例。现报道2例因呼吸困难入住我院重症监护室的神经节苷脂相关性GBS患者,运动神经纤维轴索病变为其主要特点,旨在深化临床对药物性GBS的认识。
文摘目的探讨中国南方儿童急性运动轴索性多发性神经病(acute motor axonal neuropathy,AMAN)的临床特征、电生理特点及其预后。方法对6例AMAN患儿的临床和神经电生理资料进行分析和随访。结果(1)患儿平均发病年龄为4.4岁,均来自农村,多有前驱感染史(5例),发病时间无明显季节性差异。肌无力为主要首发症状,病情达高峰的平均时间为4.2 d。(2)神经电生理检查:所有患儿神经传导检测均表现为运动神经反应电位波幅显著减低,降低至(22.3%～73.4%)正常值低限,四肢远端神经受损程度较近端重(P<0.05),而上、下肢神经受损程度无明显差异(P>0.05),运动神经电位传导速度和感觉神经传导正常。全部患儿给予静脉丙种球蛋白(IVIG)治疗。(3)随访3个月至1年,4例患儿已能独立行走。结论儿童AMAN好发于农村,全年均可发病;最常见的首发症状为肌无力,多在病程早期达到疾病高峰;神经电生理对AMAN的诊断、鉴别诊断提供了客观依据;部分患儿起病后1年能恢复独立行走能力;尽早IVIG治疗有利于促进临床神经功能恢复。
