@article {GutierrezP5.303作者={乔尔·古铁雷斯Jose-Alberto帕尔马和卡洛斯Mendoza-Santiesteban霍雷肖Kaufmann}, title ={急性自主和感觉神经病变:一个案例系列(P5.303)},体积={88}={16}补充数量,elocation-id = {P5.303} ={2017},出版商= {Wolters Kluwer健康,公司代表美国神经病学学会},文摘={目的:描述3例急性自主和感觉神经病变(AASN)。首页背景:AASN被认为是非常罕见的变异的免疫介导的急性周围神经病变或ganglionopathy。只有少数病例记录在文献中。设计/方法:病例系列。结果:三个以前健康受试者(11岁的男性;11岁的女性;37岁的女性),所有的亚洲血统,出现急性和严重的感觉和自主赤字后不久,一个简短的胃肠道和呼吸道发热性疾病。自主障碍包括呕吐、腹泻,无汗症,腹部绞痛,神经性疼痛,口干和眼睛,无电抗中等学生,头晕站和晕厥。感觉障碍包括减少所有感官与知觉广泛分布不均衡,明显感觉性共济失调。受损的运动控制与吞咽困难解释为减少肌肉力量,促使格林-巴利综合征的早期诊断。症状进展了几天,后来稳定下来。神经系统检查发现非常沮丧或缺席深腱,角膜和呕吐反射保存肌肉力量。 Corneal exam showed paracentral and inferior corneal opacities due to exposure keratopathy and dry eye. Nerve conduction studies showed extremely reduced or absent sensory nerve action potentials with normal motor nerve conduction and F waves. Cardiovascular autonomic evaluation showed decreased heart rate variability, orthostatic hypotension without compensatory tachycardia and very low or absent plasma norepinephrine levels. Spinal cord MRI showed extensive T2 hyperintensities of the posterior cords. Patient $\#$1 had positive anti-sulfatide antibodies. One year after onset, recovery of sensory impairment was poor, and autonomic dysfunction was ameliorated with symptomatic treatment.Conclusions: This is the second largest case series ever reported of patients with AASN, an immune-mediated disorder mostly affecting Asian subjects. Patients are usually misdiagnosed, which delays the start of appropriate therapy.Study Supported by: Dysautonomia Foundation, Inc.Disclosure: Dr. Gutierrez has nothing to disclose. Dr. Palma has received personal compensation for activities with Lunbeck as a speaker and advisor. Dr. Mendoza-Santiesteban has nothing to disclose. Dr. Kaufmann received personal compensation from Lundbeck and Astra Zeneca for his membership on Scientific Advisory Board.. Dr. Kaufmann received personal compensation from Springer for serving as editor in Chief of Clinical Autonomic Research.. Dr. Kaufmann received support for conducting clinical trials of Droxidopa for Chelsea Therapeutics..}, issn = {0028-3878}, URL = {//www.ez-admanager.com/content/88/16_Supplement/P5.303}, eprint = {//www.ez-admanager.com/content}, journal = {Neurology} }