Autoimmune Dementia: Clinical Course and Predictors of Immunotherapy Response
Autoimmune Dementia: Clinical Course and Predictors of Immunotherapy Response
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DOI：
10.4065/mcp.2010.0326
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发表时间：
2010-10-01
影响因子：
8.9
通讯作者：
Pittock, Sean J.
中科院分区：
文献类型：
--
作者：
Flanagan, Eoin P.;McKeon, Andrew;Pittock, Sean J.
目的：为了确定诊断特征和治疗反应的预测因素在疑似自身免疫性dementia.PATIENTS和METHODS患者：2002年1月1日至2009年1月1日，连续72例患者接受免疫治疗疑似自身免疫性痴呆。他们的基线临床，放射学和血清学特征进行了审查，并比较了免疫治疗有反应的患者和那些没有反应的患者。如果治疗医生报告免疫治疗后有改善，则将患者归类为应答者（通过Kokmen简短的精神状态测试、神经心理学测试或两者结合，记录了80%的患者）。初始免疫治疗方案包括56例患者（78%）的甲基强的松龙，12例患者（17%）的强的松，2例患者（3%）的地塞米松，静脉注射免疫球蛋白1例（1%），血浆置换1例（1%）。46名患者（64%）改善，大多数在治疗的第一周。这些免疫治疗应答者中有35%最初被诊断为患有神经退行性疾病或朊病毒疾病。治疗前和治疗后的神经心理学评分比较显示，几乎所有的认知领域，最显着的学习和记忆的改善。39例患者中有22例（56%）报告了影像学或脑电图改善。亚急性发作可预测免疫治疗反应性（P
OBJECTIVE: To define the diagnostic characteristics and predictors of treatment response in patients with suspected autoimmune dementia.PATIENTS AND METHODS: Between January 1, 2002, and January 1, 2009, 72 consecutive patients received immunotherapy for suspected autoimmune dementia. Their baseline clinical, radiologic, and serologic characteristics were reviewed and compared between patients who were responsive to immunotherapy and those who were not. Patients were classified as responders if the treating physician had reported improvement after immunotherapy (documented in 80% by the Kokmen Short Test of Mental Status, neuropsychological testing, or both).RESULTS: Initial immunotherapeutic regimens included methyl-prednlsolone in 56 patients (78%), prednlsone in 12 patients (17%), dexamethasone in 2 patients (3%), intravenous immune globulin In 1 patient (1%), and plasma exchange In 1 patient (1%). Forty-six patients (64%) Improved, most in the first week of treatment. Thirty-five percent of these immunotherapy responders were Initially diagnosed as having a neurodegenerative or prion disorder. Pretreatment and posttreatment neuropsychological score comparisons revealed improvement in almost all cognitive domains, most notably learning and memory. Radiologic or electroencephalographic improvements were reported in 22 (56%) of 39 patients. Immunotherapy responsiveness was predicted by a subacute onset (P