文摘
摘要目的:提高识别的潜在治疗的患者快速进行性痴呆的原因(RPD)。
背景:RPD包括不到两年后患者认知障碍的发病无能力由于痴呆。尽管RPD常与总是致命的神经退行性疾病如克雅二氏症,治疗形式的RPD越来越认识到在临床实践中。早期识别的治疗反应的原因RPD与早期治疗和更好的结果。
设计/方法:154 RPD患者前瞻性研究2016年2月至2022年8月的RPD两家三级医疗中心。两个神经病学家独立确定病原学诊断。RPD原因被进一步分为treatment-responsive或没有响应。人口统计学、临床和paraclinical特点与治疗相关的原因RPD被确定使用逐步多元逻辑回归。
结果:82/154例(53.2%)有RPD的潜在治疗的原因,包括自身免疫性条件(n = 51岁,62.2%),血管病变(n = 17日20.7%),精神疾病(n = 4, 4.8%),和营养不良(n = 4, 4.8%)。年轻在出现症状(或每十年= 1.22,95%置信区间ci: 1.11 - -1.36),癫痫发作在演讲(或= 6.86,95% ci: 2.27—-20.68), CSF脑脊液细胞增多(或= 6.47,95%置信区间ci: 2.36 - -17.84),和MRI特征暗示自身免疫性脑炎(或= 6.19,95% ci: 1.21—-31.79)与更大的概率有关RPD的治疗的原因。模型性能很好(曲线下的面积= 0.81;95%置信区间:0.75—-0.87;p < 0.001),对应> 80%的精度检测RPD的潜在患者治疗的原因。
结论:在我们系列Treatment-responsive RPD很常见的原因。年轻时代,早期癫痫发作,CSF -炎症或成像证据应该促使我们考虑治疗的原因,与早期开始治疗时表示。
披露:Satyadev先生没有披露。已收到博士蒂普顿个人薪酬在500 - 4999美元的范围为AbbVie担任顾问。已收到博士蒂普顿个人薪酬在500 - 4999美元的范围作为演讲者田纳西州与阿尔茨海默氏症。已收到博士蒂普顿个人薪酬在500 - 4999美元的范围与夏洛特县医疗社会作为一个演讲者,马顿斯博士没有披露。邓纳姆博士没有披露。Geschwind博士已经收到个人薪酬在500 - 4999美元的范围为LifeSci Capital LLC担任顾问。Geschwind博士已经收到个人薪酬在500 - 4999美元的范围作为顾问叶片疗法,Inc .。Geschwind博士已经收到个人薪酬在500 - 4999美元的范围作为号角的顾问咨询服务。Geschwind博士已经收到个人薪酬在5000 - 9999美元的范围作为专家证人MedConnect ProLLC。Geschwind博士的机构已收到从NIH / NIA R01 AG AG031189研究支持。Geschwind博士的机构已收到从NIH / NIA R01 AG AG062562研究支持。 The institution of Dr. Geschwind has received research support from Michael J. Homer Family Fund. The institution of Dr. Geschwind has received research support from NIH/NIA CADASIL RF1-R01. Dr. Geschwind has a non-compensated relationship as a Editorial Board with Dementia & Neuropsychologia that is relevant to AAN interests or activities. Dr. Morris has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for CBR International Advisory Board. Dr. Morris has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cure Alzheimers Fund. Dr. Morris has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for LEADS Steering Commitee. The institution of Dr. Morris has received research support from NIH grants. Dr. Morris has received intellectual property interests from a discovery or technology relating to health care. Dr. Morris has received intellectual property interests from a discovery or technology relating to health care. The institution of Dr. Graff-Radford has received research support from Biogen. The institution of Dr. Graff-Radford has received research support from Lilly. The institution of Dr. Graff-Radford has received research support from Novartis. The institution of Dr. Graff-Radford has received research support from AbbVie. Dr. Graff-Radford has received publishing royalties from a publication relating to health care. Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Parabon Nanolabs. The institution of Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Eli Lilly. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for DynaMed (EBSCO Health). Dr. Day has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Barrow Law. Dr. Day has stock in ANI Pharmaceuticals. The institution of Dr. Day has received research support from National Institutes of Health / NIA. The institution of Dr. Day has received research support from Chan Zuckerberg Initiative. The institution of Dr. Day has received research support from Alzheimer’s Association. The institution of Dr. Day has received research support from National Institutes of Health / NINDS. The institution of Dr. Day has received research support from Horizon Therapeutics. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Presenter at Annual Meeting (CME) with American Academy of Neurology. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Content Development (CME) with PeerView, Inc. Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving as a Content Development (CME) with Continuing Education, Inc. Dr. Day has a non-compensated relationship as a Clinical Director with AntiNMDA Receptor Encephalitis Foundation that is relevant to AAN interests or activities.
