肌萎缩侧索硬化症/额颞叶痴呆的认知和行为症状：检测、鉴别与进展
Cognitive and Behavioral Symptoms in ALSFTD: Detection, Differentiation, and Progression.
作者信息
Hsieh Sharpley, Caga Jashelle, Leslie Felicity V C, Shibata Marlene, Daveson Naomi, Foxe David, Ramsey Eleanor, Lillo Patricia, Ahmed Rebekah M, Devenney Emma, Burrell James R, Hodges John R, Kiernan Matthew C, Mioshi Eneida
机构信息
Brain and Mind Research Institute, Sydney, New South Wales, Australia Neuroscience Research Australia, Sydney, New South Wales, Australia ARC Centre of Excellence in Cognition and its Disorders, University of New South Wales, Sydney, New South Wales, Australia
Brain and Mind Research Institute, Sydney, New South Wales, Australia.
出版信息
J Geriatr Psychiatry Neurol. 2016 Jan;29(1):3-10. doi: 10.1177/0891988715598232. Epub 2015 Aug 6.
Brief screening tools that detect and differentiate patients with amyotrophic lateral sclerosis and frontotemporal dementia (ALSFTD) from those more subtle cognitive or behavioral symptoms (ALS plus) and motor symptoms only (ALS pure) is pertinent in a clinical setting. The utility of 2 validated and data-driven tests (Mini-Addenbrooke's Cognitive Examination [M-ACE] and Motor Neuron Disease Behavioral Scale [MiND-B]) was investigated in 70 ALS patients (24 ALSFTD, 19 ALS plus, and 27 ALS pure). More than 90% of patients with ALSFTD scored at or below the cutoff on the M-ACE, whereas this was seen in only about 20% of ALS patients without dementia. The MiND-B differentiated between ALS pure and ALS plus diagnostic categories. Rasch modeling of M-ACE and MiND-B items revealed early cognitive (fluency, memory recall) and behavioral (apathy) symptoms in ALSFTD. The combined use of the M-ACE and MiND-B detects patients with ALSFTD, differentiates along the ALS continuum, and offers insight into the progression of nonmotor symptomatology in ALSFTD.
在临床环境中，能够检测并区分肌萎缩侧索硬化症和额颞叶痴呆（ALSFTD）患者与那些具有更细微认知或行为症状（ALS加）以及仅具有运动症状（单纯ALS）患者的简易筛查工具具有重要意义。我们对70例ALS患者（24例ALSFTD、19例ALS加和27例单纯ALS）进行了两项经过验证且基于数据的测试（简易Addenbrooke认知检查 [M-ACE] 和运动神经元疾病行为量表 [MiND-B]）的效用研究。超过90%的ALSFTD患者在M-ACE上的得分处于或低于临界值，而在无痴呆的ALS患者中只有约20%出现这种情况。MiND-B区分了单纯ALS和ALS加的诊断类别。对M-ACE和MiND-B项目的Rasch模型分析揭示了ALSFTD患者早期的认知（流畅性、记忆回忆）和行为（冷漠）症状。联合使用M-ACE和MiND-B可检测出ALSFTD患者，在ALS连续体中进行区分，并深入了解ALSFTD中非运动症状的进展情况。
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