文摘
摘要目的:本研究的目标是确定病理异质性,clinico-radiological发现与不同的潜在的病理,阳性预测值(PPV)当前的CBD在哥伦比亚广播公司(CBS)患者的诊断标准。
背景:Corticobasal综合征(CBS)的特点是不对称震颤麻痹,刚性,肌阵挛,失用症。虽然最初认为是运动障碍次要corticobasal变性(CBD),对认知的影响,行为,语言是现在公认——以及它的异构基础神经病理学。
设计/方法:临床、neuropathologic,大脑核磁共振和摄影成像数据的患者临床诊断与治疗综述了哥伦比亚广播公司根据神经病理学尸检确定。
结果:组包括113例,61例(54%)。主要neuropathologic诊断是:43 (38%)CBD, 27(23.9%)进行性核上的麻痹(PSP), 17(15%)阿尔茨海默病(AD) 10(8.8%)额颞叶大叶性变性TDP-43夹杂物(FTLD-TDP), 7(6.2%)扩散路易身体疾病/广告(DLBD /广告),并与其他诊断9例(7.9%)。CBS-AD或CBS-DLBD / AD患者的死亡((差)中位数:64[13],[11]64年)而CBS-PSP患者古老(77 [12.5],P= 0.024)。CBS-DLBD / AD患者疾病持续时间最长(9[6]年)而CBS-other子群的最短(3 (4.25),P= 0.04)。后皮质迹象和肌阵挛CBS-AD和CBS-DLBD / AD患者的特征。
分布形态测量学显示广泛的皮质亏损CBS-AD的特征,而CBS-CBD和CBS-PSP主要涉及运动区域与更大的白质损失。CBS-DLBD /广告显示萎缩在焦parieto-occipital地区,和CBS-FTLD-TDP主要前额叶皮质的损失。例CBS-PSP病理学、中脑、脑桥比例最低(P= 0.012)。
谁见过标准的67例病例中可能的CBD表示,27日是pathology-proven CBD情况下,收益率PPV的40.3%。
结论:各种神经退行性疾病可以确定在哥伦比亚广播公司(CBS)患者中,但临床和区域成像差异可能有助于预测潜在的神经病理学。敏感和特定的生物标志物基础CBD是必要的。
披露:烤博士没有披露。芽庄(Pham星期四收到个人赔偿作为梅奥诊所的一名雇员。博塔博士收到NIH的研究支持。甲贺博士没有披露。拿俄米Kouri没有披露。阿里博士没有披露。Knopman博士已经收到个人薪酬在5000 - 9999美元的范围为在科学咨询服务或数据安全监测委员会殿你研究。Knopman博士的机构收到NIH的研究支持。彼得森博士已经收到个人薪酬在500 - 4999美元的范围为罗氏公司担任顾问。彼得森博士已经收到个人薪酬在500 - 4999美元的范围为默克公司担任顾问。 Dr. Petersen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. Dr. Petersen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Nestle. Dr. Petersen has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech, Inc.. Dr. Petersen has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche, Inc.. Dr. Petersen has received publishing royalties from a publication relating to health care. Dr. Petersen has received publishing royalties from a publication relating to health care. Dr. Boeve has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for Rainwater Charitable Foundation. The institution of Dr. Boeve has received research support from Alector. The institution of Dr. Boeve has received research support from GE Healthcare. The institution of Dr. Boeve has received research support from Transposon. The institution of Dr. Boeve has received research support from Cognition Therapeutics. Dr. Boeve has received publishing royalties from a publication relating to health care. The institution of Walter Kremers has received research support from Biogen. Aivi Nguyen has nothing to disclose. Dr. Murray has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Avid Radiopharmaceuticals. The institution of Dr. Murray has received research support from National Institute on Aging. The institution of Dr. Murray has received research support from Alzheimer’s Association. The institution of Dr. Murray has received research support from Chan Zuckerberg Initiative. The institution of Ross Reichard has received research support from NIH. An immediate family member of Ross Reichard has received publishing royalties from a publication relating to health care. Dr. Dickson has nothing to disclose. 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. Josephs has nothing to disclose. Dr. Whitwell has nothing to disclose. Dr. Graff-Radford has received personal compensation for serving as an employee of Mayo Clinic. Dr. Graff-Radford has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for NINDS/NIH. Dr. Graff-Radford has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for American Academy of Neurology. The institution of Dr. Graff-Radford has received research support from NIH.
