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皮质基底节变性和进行性核上性麻痹中失用症的比较。

Comparison of apraxia in corticobasal degeneration and progressive supranuclear palsy.

作者信息

Pharr V, Uttl B, Stark M, Litvan I, Fantie B, Grafman J

机构信息

Cognitive Neuropharmacology Unit, Defense & Veteran Head Injury Program, Henry M. Jackson Foundation, Rockville, MD, USA.

出版信息

Neurology. 2001 Apr 10;56(7):957-63. doi: 10.1212/wnl.56.7.957.

Abstract

OBJECTIVE

To describe ideomotor apraxia in patients with corticobasal degeneration and those with progressive supranuclear palsy, two parkinsonian disorders that are often misdiagnosed due to the overlap in their clinical features, and to determine whether systematic apraxia testing is useful for differential diagnosis.

METHODS

Fourteen patients fulfilling National Institute of Neurological Disorders and Stroke-Society for Progressive Supranuclear Palsy clinical criteria for progressive supranuclear palsy, 13 patients fulfilling modified Lang criteria for corticobasal degeneration, and 12 normal healthy control subjects were given the Test of Oral and Limb Apraxia, which was scored according to the Florida Apraxia Battery for occurrence of various types of apraxic errors.

RESULTS

Both patients with progressive supranuclear palsy and corticobasal degeneration committed a greater number of apraxic errors than normal healthy control subjects on both transitive and intransitive tasks (p < 0.001 in both cases), but apraxia was much more severe in patients with corticobasal degeneration than progressive supranuclear palsy (p < 0.001). The index of apraxia severity, in combination with the assessment of the two key features of progressive supranuclear palsy (falls and vertical gaze palsy), correctly classified all patients.

CONCLUSIONS

Patients with corticobasal degeneration show more severe ideomotor apraxia than patients with progressive supranuclear palsy, and systematic assessment of ideomotor apraxia facilitates the differential diagnosis between patients with progressive supranuclear palsy and those with corticobasal degeneration.

摘要

目的

描述皮质基底节变性患者和进行性核上性麻痹患者的观念运动性失用症,这两种帕金森氏症由于临床特征重叠常被误诊,并确定系统的失用症测试是否有助于鉴别诊断。

方法

14名符合美国国立神经疾病和中风研究所-进行性核上性麻痹协会进行性核上性麻痹临床标准的患者、13名符合改良朗氏标准的皮质基底节变性患者以及12名正常健康对照者接受了口腔和肢体失用症测试,该测试根据佛罗里达失用症量表对各种类型的失用错误的发生情况进行评分。

结果

进行性核上性麻痹患者和皮质基底节变性患者在及物和不及物任务中出现的失用错误均多于正常健康对照者(两种情况p均<0.001),但皮质基底节变性患者的失用症比进行性核上性麻痹患者严重得多(p<0.001)。失用症严重程度指数,结合对进行性核上性麻痹两个关键特征(跌倒和垂直凝视麻痹)的评估,正确分类了所有患者。

结论

皮质基底节变性患者比进行性核上性麻痹患者表现出更严重的观念运动性失用症,对观念运动性失用症的系统评估有助于进行性核上性麻痹患者和皮质基底节变性患者之间的鉴别诊断。

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