Park Hee Kyung, Park Kee Hyung, Yoon Bora, Lee Jae-Hong, Choi Seong Hye, Joung Jee H, Yoon Soo Jin, Kim Byeong C, Kim Seung Hyun, Kim Eun-Joo, Na Duk L, Park Kyung Won
Department of Neurology, Inje University Ilsan-Paik Hospital, Goyang, Republic of Korea.
Department of Neurology, Gil Medical Center, Gachon University College of Medicine, Incheon, Republic of Korea.
J Neurol Sci. 2017 Jan 15;372:51-56. doi: 10.1016/j.jns.2016.11.033. Epub 2016 Nov 16.
We investigated the prevalence of parkinsonism in frontotemporal dementia (FTD) subtypes and the cognitive and behavioral differences between FTD with and without parkinsonism in a well-structured, prospective cohort.
One hundred and ninety-one FTD patients were enrolled and all patients underwent comprehensive neurological evaluations, neuropsychological tests, and the Unified Parkinson's Disease Rating Scale.
The prevalence of parkinsonism was 38.7% (74 patients), and included 33 (46.5%) behavioral variant FTD (bvFTD), 16 (24.2%) semantic dementia (SD), 19 (45.2%) progressive nonfluent aphasia (PNFA), and 6 (50%) FTD associated with motor neuron disease (FTD-MND). SD patients with parkinsonism had higher CDR sum of boxes scores (9.7±4.5 vs 6.2±4.5, p=0.024), frontal behavioral inventory total score (33.7±20.5 vs 24.3±14.5, p=0.045), and executive function score of frontal executive dysfunction, disinhibition, and apathy (28.9±13.7 vs 19.2±12.9, p=0.021) than those without parkinsonism. Seoul Instrumental Activities of Daily Living score (bvFTD: 23.5±11.7 vs 17.3±11.3, p=0.031, SD: 23.1±11.1 vs 11.3±9.3, p=0.005) was higher for bvFTD and SD with parkinsonism than for those without parkinsonism.
Parkinsonism is found to be more common in patients with bvFTD, PNFA, and FTD-MND patients than those with SD. Behavioral disturbances were more prominent in SD with parkinsonism than without. Additional studies are needed to determine the pathomechanism and optimal treatment of parkinsonism in different FTD subtypes.
我们在一个结构严谨的前瞻性队列中，研究了额颞叶痴呆（FTD）各亚型中帕金森综合征的患病率，以及伴有和不伴有帕金森综合征的FTD患者在认知和行为方面的差异。
纳入191例FTD患者，所有患者均接受了全面的神经学评估、神经心理学测试以及统一帕金森病评定量表评估。
帕金森综合征的患病率为38.7%（74例患者），其中包括33例（46.5%）行为变异型FTD（bvFTD）、16例（24.2%）语义性痴呆（SD）、19例（45.2%）进行性非流利性失语（PNFA）以及6例（50%）与运动神经元病相关的FTD（FTD-MND）。伴有帕金森综合征的SD患者在总体衰退量表（CDR）方框总和评分（9.7±4.5对6.2±4.5，p = 0.024）、额叶行为量表总分（33.7±20.5对24.3±14.5，p = 0.045）以及额叶执行功能障碍、脱抑制和淡漠的执行功能评分（28.9±13.7对19.2±12.9，p = 0.021）方面均高于不伴有帕金森综合征的患者。伴有帕金森综合征的bvFTD和SD患者的首尔日常生活活动能力量表评分（bvFTD：23.5±11.7对17.3±11.3，p = 0.031，SD：23.1±11.1对11.3±9.3，p = 0.005）高于不伴有帕金森综合征的患者。
发现帕金森综合征在bvFTD、PNFA和FTD-MND患者中比在SD患者中更常见。伴有帕金森综合征的SD患者的行为障碍比不伴有者更为突出。需要进一步研究以确定不同FTD亚型中帕金森综合征的发病机制和最佳治疗方法。
