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经尸检确诊的帕金森病中冻结步态的临床病理特征

Clinicopathological characteristics of freezing of gait in autopsy-confirmed Parkinson's disease.

作者信息

Virmani Tuhin, Moskowitz Carol B, Vonsattel Jean-Paul, Fahn Stanley

机构信息

Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, USA.

Department of Pathology, College of Physicians and Surgeons, Columbia University, New York, NY, USA.

出版信息

Mov Disord. 2015 Dec;30(14):1874-84. doi: 10.1002/mds.26346. Epub 2015 Aug 3.

Abstract

BACKGROUND

Twenty-five percent to sixty percent of Parkinson's disease (PD) patients reportedly have freezing of gait, leading to impaired mobility, falls, and decreased quality of life. Several factors have been associated with gait freezing in PD patients. We analyze for these factors in autopsy-proven PD patients.

METHODS

We performed a chart review of 58 patients with pathologically confirmed PD based on substantia nigra Lewy bodies. Freezing of gait was defined as a score of 1 or more on Item 14 of the Unified Parkinson's Disease Rating Scale or if documented on examination. Serial office notes and scales were used to determine onset and progression of motor and non-motor symptoms.

RESULTS

Patients had been followed up for an average of 20 visits over 9 y. The mean onset of gait freezing was 9.3 y from initial motor symptoms. Patients with earlier gait freezing more commonly had initial gait difficulties and developed postural instability, dyskinesias, memory impairment, hallucinations, and vivid dreams earlier during the disease course. Early onset of hallucinations was correlated with more rapid progression of gait freezing. Maximal equivalent levodopa dose was not correlated with earlier onset or progression of gait freezing. Progressive and more severe gait freezing trended toward higher-severity Lewy body disease on postmortem examination.

CONCLUSIONS

Early onset and rapid progression of freezing of gait in this cohort were correlated with early cognitive impairment and hallucinations that are potential clinical hallmarks of cortical Lewy bodies. The gradual worsening and severity of gait freezing correlated with the density of cortical Lewy body-containing neurons.

摘要

背景

据报道,25%至60%的帕金森病(PD)患者存在步态冻结,导致行动能力受损、跌倒及生活质量下降。PD患者的步态冻结与多种因素相关。我们对经尸检证实的PD患者的这些因素进行分析。

方法

我们对58例基于黑质路易小体病理确诊为PD的患者进行了病历回顾。步态冻结定义为统一帕金森病评定量表第14项得分为1分或更高,或检查记录中有步态冻结情况。使用系列门诊记录和量表来确定运动和非运动症状的发作及进展情况。

结果

患者平均随访9年,共就诊20次。步态冻结的平均发作时间距初始运动症状出现9.3年。步态冻结出现较早的患者更常出现初始步态困难,且在病程中更早出现姿势不稳、运动障碍、记忆障碍、幻觉及生动梦境。幻觉出现较早与步态冻结进展更快相关。最大等效左旋多巴剂量与步态冻结的较早发作或进展无关。尸检显示,进行性且更严重的步态冻结倾向于更高严重程度的路易体病。

结论

该队列中步态冻结的早发和快速进展与早期认知障碍及幻觉相关,而这些是皮质路易体的潜在临床特征。步态冻结的逐渐加重和严重程度与含皮质路易体神经元的密度相关。

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