p.A53T PD 患者出现选择性认知障碍和嗅觉减退，而典型 PD 患者则没有。
Selective cognitive impairment and hyposmia in p.A53T PD vs typical PD.
机构信息
From the 2nd Neurology Clinic (C.K., M.S., A.S., I.B., N.P., S.F., D.K., S.G.P., L.S.), Attikon Hospital, National and Kapodistrian University of Athens Medical School, Greece; Neurology Clinic (M.S.), Philipps University, Marburg, Germany; Parkinson's Disease and Movement Disorders Department (M.S.), HYGEIA Hospital; and Neurology Clinic (D.P.), Henry Dunan Hospital, Athens, Greece.
出版信息
Neurology. 2018 Mar 6;90(10):e864-e869. doi: 10.1212/WNL.0000000000005063. Epub 2018 Feb 7.
OBJECTIVE
To evaluate nonmotor symptoms in early /p.A53T Parkinson disease (PD) (A53T PD) compared to typical PD (tPD).
METHODS
The presence of hyposmia, neuropsychiatric, dysautonomic, and sleep disturbances was assessed by standardized questionnaires and validated scales in 18 patients with A53T PD and 18 patients with tPD, matched for age, sex, and disease duration. All patients were enrolled into the Parkinson's Progression Markers Initiative study.
RESULTS
The levodopa equivalent daily dose was higher in the A53T PD ( = 0.018) group vs the tPD group. Scores on the University of Pennsylvania Smell Identification Test ( = 0.001), Benton Judgement of Line Orientation test ( = 0.001), Letter Number Sequencing Test ( = 0.002), and phonemic verbal fluency ( = 0.002) were lower in the A53T PD group vs the tPD group. In contrast, overall cognition, verbal memory, and semantic fluency were similar between groups.
CONCLUSION
The observed selective cognitive impairment reflecting frontal-parietal network dysfunction, together with impaired olfaction, define a set of nonmotor dysfunctions related to A53T PD. These results have implications for the prognosis of patients with A53T PD. Moreover, as the archetypal α-synucleinopathy, such results may give insights into tPD.
目的
评估早发性 /p.A53T 帕金森病（PD）（A53T PD）与典型 PD（tPD）的非运动症状。
方法
通过标准化问卷和经过验证的量表评估 18 例 A53T PD 患者和 18 例年龄、性别和病程匹配的 tPD 患者的嗅觉减退、神经精神、自主神经和睡眠障碍。所有患者均被纳入帕金森进展标志物倡议研究。
结果
A53T PD 组的左旋多巴等效日剂量较高（=0.018）。宾夕法尼亚大学嗅觉识别测试（=0.001）、本顿判断线定向测试（=0.001）、字母数字序列测试（=0.002）和语音流畅性测试（=0.002）的得分低于 tPD 组。相比之下，两组的总体认知、言语记忆和语义流畅性相似。
结论
观察到的选择性认知障碍反映了额顶叶网络功能障碍，加上嗅觉受损，定义了一组与 A53T PD 相关的非运动功能障碍。这些结果对 A53T PD 患者的预后有影响。此外，作为典型的α-突触核蛋白病，这些结果可能为 tPD 提供一些见解。
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