Clinical presentation and pharmacological therapy in corticobasal degeneration
Clinical presentation and pharmacological therapy in corticobasal degeneration
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DOI：
10.1001/archneur.55.7.957
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发表时间：
1998-07-01
影响因子：
--
通讯作者：
Riley, DE
中科院分区：
文献类型：
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作者：
Kompoliti, K;Goetz, CG;Riley, DE
背景资料：迄今为止，据我们所知，在临床诊断为皮质基底节变性的大量患者中，尚无系统的治疗结果报告。目的：评价临床诊断为皮质基底节变性患者的临床表现和治疗结果。我们收集了过去5年中在8个主要运动障碍诊所就诊的被诊断为皮质基底节细胞性运动障碍的病例。方法：采用回顾性分析的方法，对147例患者的临床表现、用药情况、药物反应及不良反应进行分析。所有患者均存在帕金森病特征，89%存在其他运动障碍，93%存在较高的皮质功能障碍。最常见的帕金森症状是僵硬（92%），其次是运动迟缓（80%），步态障碍（80%）和震颤（55%）。其他运动障碍为肌张力障碍71%，肌阵挛5%。较高的皮质功能障碍包括运动障碍（82%）、异肢（42%）、皮质感觉丧失（33%）和痴呆（25%）。92%的病例患者接受了多巴胺能药物治疗，24%的患者获得了有益效果。帕金森症状改善最多，左旋多巴是最有效的药物。47例患者服用苯二氮卓类药物，主要是氯硝西泮，导致23%的肌阵挛和9%的肌张力障碍改善。在这些病例患者中，药物试验最常见的致残性不良反应是嗜睡（n = 24）、胃肠道不适（n = 23）、意识模糊（n = 16）、头晕（n = 12）、幻觉（n = 5）和口干（n = 5）。药物干预在皮质基底节变性的治疗中基本无效，需要新的治疗方法来改善这种综合征的症状。
Background: To date, to our knowledge, there is no systematic presentation of treatment outcome in large series of patients clinically diagnosed as having corticobasal degeneration.Objective: To evaluate the clinical presentation and treatment outcome of patients clinically diagnosed as having corticobasal degeneration.Subjects: We gathered case patients seen in 8 major movement disorder clinics during the last 5 years who were diagnosed as having corticobasal ganglionic degeneration.Methods: Using a chart review method, we recorded the clinical presentation, medications used, response to medications, and adverse effects.Results: A total of 147 case patients were reviewed, 7 were autopsy proven. Parkinsonian features were present in all, other movement disorders in 89%, and higher cortical dysfunction in 93%. The most common parkinsonian sign was rigidity (92%), followed by bradykinesia (80%), gait disorder (80%), and tremor (55%). Other movement disorders were dystonia in 71% and myoclonus in 5%. Higher cortical dysfunction included dyspraxia (82%), alien limb (42%), cortical sensory loss (33%), and dementia (25%). Ninety-two percent of the case patients received dopaminergic drugs, which resulted in a beneficial effect for 24%. Parkinsonian signs were the elements improving the most and levodopa was the most effective drug. Benzodiazepines, primarily clonazepam, were administered to 47 case patients, which resulted in improvement of myoclonus in 23% and dystonia in 9%. The most frequent disabling adverse effects of drug trials in these case patients were somnolence (n = 24), gastrointestinal complaints (n = 23), confusion (n = 16), dizziness (n = 12), hallucinations (n = 5), and dry mouth (n = 5).Conclusions: Pharmacological intervention was largely ineffective in the management of corticobasal degeneration, and new treatments are needed for ameliorating the symptoms of this syndrome.