PT -期刊文章盟Jose-Alberto帕尔马AU -卡洛斯Mendoza-Santiesteban AU -露西Norcliffe-Kaufmann盟霍雷肖Kaufmann TI -家族性神经异常:一种疾病与隐藏的眼泪(P5.305) DP - 2017年4月18日TA -神经病学PG - P5.305 VI - 88 IP - 16补充4099 - //www.ez-admanager.com/content/88/16_首页Supplement/P5.305.short 4100 - //www.ez-admanager.com/content/88/16_Supplement/P5.305.full所以Neurology2017 4月18日;88 AB -目的:确定政府的毒蕈碱的受体激动剂能刺激分泌眼泪家族性神经异常患者(FD)。背景:FD经常被称为一种疾病,没有眼泪,但根本原因alacrima是未知的。通常情况下,角膜神经刺激生产的眼泪泪腺体的眼睛。FD的缺失或减弱的眼泪是否由于去神经或异常泪腺体本身尚不清楚。设计/方法:我们应用毛果芸香碱(4%,副交感神经M3受体激动剂),局部16基因证实FD患者的眼睛刺激泪液分泌腺体在泪中直接绕过了神经通路。我们评估使用Cochet-Bonnet触觉器角膜敏感性。眼泪与Schirmer体积估计测试(按比例缩小的纸条放在下眼睑和水分测量5分钟后的长度)。Schirmer的测试进行了四次:基线;30分钟后滴注法的生理盐水(安慰剂,2滴);在坏死; and 3-hours after pilocarpine instillation (2 drops).Results: Basal tear secretion was 6.3 ± 2.6 mm (mean ± SD), and 6.9 ± 3.0 mm 30 min after placebo (p=0.395). Thirty minutes after the instillation of piloparpine, tear volume more than doubled to 19.6 ± 8.3 mm (p<0.001); and the increased tear production persisted at 3 hours (12.6 ± 5.1 mm; p<0.001). There was a significant positive correlation between corneal sensitivity and tear secretion at baseline (p<0.0001; R2=0.74).Conclusions: Patients with FD have functional lacrimal glands, which can be stimulated with an M3 agonist to produce tears. Basal tear secretion was directly related to corneal sensitivity. The findings suggest for the first time that tear production in patients with FD can be restored pharmacologically.Study Supported by: Dysautonomia Foundation, Inc.Disclosure: Dr. Palma has received personal compensation for activities with Lunbeck as a speaker and advisor. Dr. Mendoza-Santiesteban has nothing to disclose. Dr. Norcliffe-Kaufmann 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..