Ja Young Kim, et al. Dysarthria and Cognitive Function in Parkinson s Disease 근육긴장이상및미간반사 (glabella reflexes) 등이관찰된다 (Jankovic, 2008). 비운동영역에서는자율신경계기능

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1 ISSN (Print) ISSN (Online) Commun Sci Disord 2014;19(4): Original Article Characteristics of Dysarthria and Cognitive Functions in Patients with Parkinson s Disease and Parkinsonplus Syndrome Ja Young Kim a, Sun Ju Chung b, Jae-Hong Lee b, Miseon Kwon b a Department of Neurology, Asan Medical Center, Seoul, Korea b Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea Correspondence: Miseon Kwon, PhD Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul , Korea Tel: Fax: mskwon@amc.seoul.kr Received: September 30, 2014 Revised: November 2, 2014 Accepted: November 28, 2014 This work was supported by Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Education, Science and Technology (No ). Objectives: This study was to investigate the characteristics of dysarthria and cognitive ability of patients with Parkinson s disease and Parkinson-plus syndrome to find the key components that can differentiate these diseases. Methods: Forty-one patients (11 patients with idiopathic Parkinson s disease [IPD], 10 with multiple systems atrophy with predominant cerebellar ataxia [MSA-c], 10 with multiple systems atrophy with predominant Parkinsonism [MSA-p], and 10 with progressive supranuclear palsy [PSP]) participated. After controlling the motor ability in rigidity, bradykinesia, and ataxia in the Unified Parkinson s Disease Rating Scale of 4 groups, dysarthria was assessed by performing tasks of prolonged phonation, diadochokinesis, and connected speech. In addition, cognitive function was measured by the Korean version of the Montreal Cognitive Assessment. Results: The age, education level, disease duration, and motor ability of patients were not significantly different. However, analysis of motor ability showed significant (p <.05) differences between IPD-MSA-p, and MSA-c-MSA-p group for rigidity, and between IPD-MSA-c, MSA-c- MSA-p, and MSA-p-PSP group for ataxia. There was no significant difference for bradykinesia. In addition, dysarthria evaluation showed that the hypokinetic component was more frequently observed in the IPD and MSA-p than the MSA-c group and the ataxic component was greater in the MSA-c than other groups. Moreover, cognitive ability was significantly (p <.05) more impaired in patients with PSP than the IPD & MSA-c groups. Conclusion: The characteristics of dysarthria and cognitive deficits may serve as useful factors in distinguishing IPD, MSA-c, MSA-p, and PSP. Further studies including large numbers of patients are warranted to confirm these results. Keywords: Parkinson s disease, Parkinson-plus syndrome, Dysarthria, Cognitive function, Differentiate diseases 퇴행성 이란이미성장한구조, 기능, 조직등이점차역행하는성질을의미하는말로, 신경계의퇴행성질환은말 / 언어장애의주요원인이된다. 퇴행성질환중파킨슨병 (Parkinson s disease, PD) 은중뇌흑질 (substantial nigra) 의신경학적손실과탈색소현상을보이며도파민생성이줄어들고루이체 (Lewy body) 가침착하여생기는질환으로, 운동을계획하고실행하는전두엽의기능장애가나타난다 (Gelb, Oliver, & Gilman, 1999; Jankovic, 2008). 이중특 발성파킨슨병 (idiopathic PD, IPD) 은원인불명의질환으로알려져있다 (Hughes, Daniel, Kilford, & Lees, 1992). 파킨슨병은운동영역과비운동영역기능에장애를일으키는데, 운동영역은떨림 (tremor), 운동느림증 (bradykinesia), 자세불안정 (postural instability), 경축 (rigidity) 으로대표되고, 이차적특징으로무표정 (hypomimia), 마비말장애 (dysarthria), 삼킴장애 (dysphagia), 과잉침분비, 소자증 (micrographia), 발끌기, 동결 (freezing), 가속보행 (festination), Copyright 2014 Korean Academy of Speech-Language Pathology and Audiology This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( by-nc/3.0) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited

2 Ja Young Kim, et al. Dysarthria and Cognitive Function in Parkinson s Disease 근육긴장이상및미간반사 (glabella reflexes) 등이관찰된다 (Jankovic, 2008). 비운동영역에서는자율신경계기능이상, 인지 / 신경행동장애, 수면장애및감각장애가관찰된다 (Jankovic, 2008). 파킨슨플러스증후군 (Parkinson-plus syndrome) 은 IPD와유사한파킨슨증을보이나그원인이나메커니즘은다른질환으로인한것이다. 그중진행성핵상마비 (progressive supranuclear palsy, 이하 PSP) 는 1964년에처음으로보고된희귀질환으로원인은잘알려져있지않지만기저핵, 대뇌피질, 소뇌치상핵에축적된타우단백질에의한신경교세포의괴사로발생한다 (Maher, Smith, & Lee, 1985; Rajput & Rajput, 2001; Verny, Duyckaerts, Agid, & Hauw, 1996). 안구운동장애가가장특징적인증상으로나타나며, 운동느림증, 균형상실등이관찰되나 IPD와는다르게경직된형태의운동불능 (akinetic-rigid form) 이관찰된다 (Hughes et al., 1992). 또치매가동반되기도하고, 쉽게잊어버리거나사고력이느려지며, 무감동 (apathy) 을보이기도하는데, 말측면에서는발성부전과마비말장애가나타난다. 또다른유형인다계통위축증 (multiple systems atrophy, MSA) 도명확한원인이밝혀지지않은퇴행성질환으로기저핵, 소뇌, 뇌간, 척수등다양한중추신경계에서산발적으로발생하며, 파킨슨증, 소뇌기능이상, 자율신경계이상, 추체로이상징후를보인다 (Wenning, Ben-Shlomo, Magalhaes, Daniel, & Quinn, 1994). MSA는이상징후에따라하위유형으로분류할수있는데, 자율신경계이상이주증상일경우샤이-드레거증후군 (Shy-Drager syndrome, MSA-a), 파킨슨증을주증상으로하는추체외로이상은선조흑질변성 (striatonigral degeneration; Parkinsonian variant, MSA-p), 소뇌기능이상은산발적올리브교소뇌위축 (sporadic olivopontocerebellar atrophy, MSA-c) 으로구분된다 (Wenning et al., 1994). 파킨슨병과파킨슨플러스증후군이보이는공통적특징으로인해감별진단이쉽지않지만이를위한시도가꾸준히이루어지고있다. Duffy (2005) 는퇴행성신경질환자들의마비말장애하위유형을분석하였는데, IPD 환자들에게는과소운동형 (hypokinetic) 이빈번하게관찰되었고, PSP 환자들에게는경직형 (spastic), 실조형 (ataxic), 과소운동형 (hypokinetic) 이관찰되었다. 또한 MSA-a, MSA-c 환자들에게는이완형 (flaccid), 경직형, 실조형, 과소운동형이, MSA-p 환자들에게는경직형, 실조형, 과소운동형, 과다운동형 (hyperkinetic) 마비말장애의특성이관찰된다고보고하였다 (Kim, 2012). Ackermann 과 Ziegler (1991) 도 IPD 환자들에게는주로과소운동형이관찰된다고하였고, 다른연구에서는 MSA환자들에게과소운동형-실조형-경직형, 과소운동형-실조형, 과소운동형-경직형의혼합형이관찰된다고하였다 (Kluin, Gilman, Lohman, & Junck, 1996). 또한, PSP 환자들에게는과소운동형-경직형 (Sachin et al., 2008) 과과소운동형-경직형 -실조형의혼합형 (Kluin et al., 2001) 이관찰된다고보고된연구도있었다. 위질환들은운동기능장애뿐아니라인지기능장애가동반되기도하는데, 파킨슨병은전전두엽의측면 (lateral surface) 에서꼬리핵의배외측머리부분을통해기저핵에이르는배외측전전두피질회로 (dorsolateral prefrontal cortex circuit) 의이상으로집행기능장애를보인다고보고되었다 (Piatt, Fields, Paolo, & Tröster, 1999; Piatt, Fields, Paolo, Koller, & Tröster, 1999; Tekin & Cummings, 2002). 세부질환별인지기능장애특성을살펴보기위한시도도이루어졌다. Lange 등 (2003) 은 IPD, MSA, PSP 환자집단과정상인집단의집행기능능력비교를위해신경심리검사를시행한결과, 모든환자집단에서구어유창성, 문제해결능력, 작업기억능력의저하가관찰되었다. 특히 PSP 환자들은 IPD, MSA 환자들보다구어유창성과제에서더어려움을보였다. 또 Robbins 등 (1994) 은 IPD, PSP, MSA 환자들에게 Cambridge Neuropsychological Test Automated Battery (CANTAB) 를이용하여전두엽기능과연관된인지기능평가를실시하였는데, 계획 (planning) 과제에서세집단모두어려움이관찰되었지만, IPD, PSP 환자들은처음행동하기전에걸리는시간이오래소요되었고, MSA 환자들은첫실행후다음실행을할때까지의소요시간만오래걸렸다. 주의전환능력과제에서도모든집단에서어려움이관찰되었지만 PSP 환자들의어려움이두드러졌다. 이상의결과처럼파킨슨증을공통적으로보이는질환들의세부적특성을밝히고자하는연구들이이어지고있으나대부분퇴행성신경질환군간마비말장애유형및인지능력을분석하거나, 병의진전에따른말장애의중증도및인지능력수준을분석한연구가주를이루고특정질환내의세부유형별특성연구는부족한실정이다. 따라서본연구에서는파킨슨병과파킨슨플러스증후군의 IPD, MSA-c, MSA-p, PSP 네질환환자를대상으로마비말장애의특성과인지기능에차이를보이는지를살펴보고자하였다. 연구방법연구대상본연구의대상은 2007년 1월부터 2014년 6월까지서울아산병원에입원또는외래로방문한환자중신경과전문의의진단을거쳐말장애평가에의뢰된 IPD 환자 11명 ( 남 : 여 = 8:3, 평균연령 64세 ), MSA-c 환자 10명 ( 남 : 여 = 7:3, 평균연령 63세 ), MSA-p 환자 10명 ( 남 : 여 = 3:7, 평균연령 62세 ), PSP 환자 10명 ( 남 : 여 = 7:3, 평균연령

3 특발성파킨슨병과파킨슨플러스증후군환자의마비말장애및인지기능특성비교 김자영외 세 ), 총 41명이었다. 다른뇌신경계질환이없고, 신경과전문의의진단을거쳐진단명이확정된환자만을선별하였고, 집단간운동기능장애의중증도를통제하기위해파킨슨병등급척도 (Unified Parkinson s Disease Rating Scale, UPDRS) 의세부항목중운동평가부분의경축 (rigidity), 운동느림증 (bradykinesia), 실조증 (ataxia) 의각점수를합친점수를산출하여 ( 운동기능장애점수 ) 네집단간정도의차이가나지않도록하였다 (Table 1). 자료수집환자의마비말장애와인지기능평가는외부의소음이차단된언어치료실에서검사자와일대일로시행되었고, 검사자료는디지털녹음기에기록하였다. 과제는말측면에서모음연장과제, 조음교대 / 일련운동과제, 그리고자발화및읽기과제를실시하였고, 인지측면에서 Korean version of Montreal Cognitive Assessment (MoCA- K; Rossetti, Lacritz, Cullum & Weiner, 2011) 과제를시행하였다. 자료분석말과제의분석은환자의의학적진단정보를모르는경력 10년이상의언어치료전문가와모음연장과제, 조음교대 / 일련운동과제, 읽기및자발화과제를분석하여마비말장애유형및중증도를분류하였다. 인지과제는 MoCA-K 검사의총점을사용하였다. 집단간피험자의차이를분석하기위해일원분산분석 (one-way ANO- VA) 을실시하였고, 유의한차이가있는경우 Tukey 사후검정을시 행하였다. 말과제분석은카이제곱검정 (chi-square test) 을실시하 였다. 인지기능은일반선형모형 (general linear model) 을사용하여 연령과교육의정도를공변량으로통제하고분산분석을시행하였 고, 집단간차이를보기위한대비분석을시행하였다. 연구결과 집단간피험자특성 분석항목중연령, 학력, 발병후의기간 ( 월 ), 마비말장애의중증 도, 운동기능장애정도는네집단간모두유의하지않았다 (Table 2). 그러나운동기능능력을경축 (rigidity), 운동느림증 (bradykinesia), 실조증 (ataxia) 항목으로세분화하여분석하였을때운동느림 증은집단간유의한차이를보이지않았지만경축과실조증은집 단간유의한차이를보였다 (Table 3). 사후분석결과경축은 IPD 와 MSA-p 집단과 MSA-c 와 MSA-p 집단간에차이를보였는데, IPD 보다 MSA-p 집단이높았고 MAS-c 보다 MSA-p 집단이높았다. 실 Table 2. Analysis of variance of subject characteristics (N= 41) Age Education POT Dysarthria severity Motor score of UPDRS MoCA-K F * POT= post onset time; UPDRS= Unified Parkinson s Disease Rating Scale; MoCA- K= Korean version of the Montreal Cognitive Assessment. *p <.05. Table 1. Characteristics of subjects Groups Male Genders Female Age (yr) Education (yr) POT (mo) Motor score of UPDRS IPD (N= 11) ± ± ± ± 4.61 MSA-c (N= 10) ± ± ± ± 5.51 MSA-p (N= 10) ± ± ± ± 5.40 PSP (N= 10) ± ± ± ± 8.46 Total (N= 41) ± ± ± ± 6.40 Values are presented as mean± SD. IPD = idiopathic Parkinson s disease; MSA-c = multiple systems atrophy with predominant cerebellar ataxia; MSA-p = multiple systems atrophy with predominant Parkinsonism; PSP= progressive supranuclear palsy; POT= post onset time; UPDRS= Unified Parkinson s Disease Rating Scale. Table 3. Analysis of variance & post-hoc analysis of motor dysfunction (N= 41) Group Rigidity.66.04* *.35.04*.00* Ataxia.00* *.00* *.05* * Group 1= idiopathic Parkinson s disease (IPD); Group 2= multiple systems atrophy with predominant cerebellar ataxia (MSA-c); Group 3= multiple systems atrophy with predominant Parkinsonism (MSA-p); Group 4= progressive supranuclear palsy (PSP). *p <

4 * ** Ja Young Kim, et al. Dysarthria and Cognitive Function in Parkinson s Disease Table 4. Significant probability in dysarthria type (N= 41) Pearson chi-square value df Exact significant probability (2-sided) Hypokinetic a 3.003* Spastic a Flaccid a Ataxic a 3.002* a Four cells (50.0%) have expected counts less than 5. The minimum expected count is *p <.01. Table 5. Results of contrast test between groups in MoCA-K (N = 41) Group Contrast estimate SE p IPD - PSP * MSA-c - PSP * MSA-p - PSP IPD= idiopathic Parkinson s disease; MSA-c= multiple systems atrophy with predominant cerebellar ataxia; MSA-p = multiple systems atrophy with predominant Parkinsonism; PSP = progressive supranuclear palsy; MoCA-K = Korean version of the Montreal Cognitive Assessment. *p < ** * IPD MSA-c MSA-p PSP * 의미하게낮은인지기능을보였다 (Table 5). MoCA-K 세부항목별분석결과, 네집단모두주의력과지연회상력에서능력저하가관찰되었는데, 이는 MSA-p, PSP 집단에서두드러졌다. 이외에 PSP 집단에서는실행력, 추상력항목에서도두드러진능력저하가관찰되었다. 논의및결론 0 Figure 1. Frequency of dysarthria characteristics by groups. IPD = idiopathic Parkinson s disease; MSA-c = multiple systems atrophy with predominant cerebellar ataxia; MSA-p = multiple systems atrophy with predominant Parkinsonism; PSP= progressive supranuclear palsy. *p <.01, **p <.05. 조증은 IPD 와 MSA-c 집단, MSA-c 와 MSA-p 집단, 그리고 MSA-p 와 PSP 집단간에차이를보였는데 MSA-c 집단이 IPD, MSA-p 집 단보다높았으며, PSP 집단이 MSA-p 집단보다높았다. 마비말장애특성 마비말장애중증도는집단간유의하지않았지만 (Table 2), 마비 말장애유형중과소운동형과실조형특성은집단간유의미한차 이를보였다 (Table 4). 과소운동형은 IPD 와 MSA-c 집단그리고 MSA-c 와 MSA-p 집단간에유의미한차이를보였고, 실조형은 IPD 와 MSA-c 집단, MSA-c 와 MSA-p 집단, 그리고 MSA-c 와 PSP 집단간에유의미한차이를보였다 (Figure 1). 과소운동형의빈도 는 MSA-p, IPD, PSP, MSA-c 집단순으로관찰되었고, 실조형의빈 도는 MSA-c, MSA-p, IPD 와 PSP 집단순이었다. 인지기능특성 Hypo Spastic Flaccid Ataxic 집단간 MoCA-K 점수도유의미한차이를보였는데 (p<.05)(table 2), 대비분석결과 IPD 와 PSP 집단과 MSA-c 와 PSP 집단간에 차이가나타났다. PSP 환자는 IPD 환자와 MSA-c 환자에비해유 본연구에서는공통적인임상적특징으로인해임상에서감별진단이어려운파킨슨병과파킨슨플러스증후군환자들의말장애및인지기능의특성을살펴봄으로써감별진단에유용한요소를찾아보고자하였다. 파킨슨병 (IPD) 집단과파킨슨플러스증후군으로임상에서자주관찰되는 MSA-c, MSA-p, PSP 집단을대상으로질환의중증도를통제하기위해우선 UPDRS의세부항목중경축 (rigidity), 운동느림증 (bradykinesia), 실조증 (ataxia) 의각점수를합친것을기준으로운동기능장애중증도에집단간차이가나지않도록하였다. 그러나운동기능세부항목별분석을시행한결과운동느림증은집단간유의하지않았지만경축과실조증은집단간유의미한차이를보였다. 특히경축은 IPD와 MSA-p 집단, 그리고 MSA-c와 MSA-p 집단간에유의미한차이를보였다. IPD와 MSA-p 집단이운동불능증 (akinesia) 및경축 (rigidity) 과같은중복되는운동기능특징으로인해임상에서초기진단시가장구분하기어렵다는기존의연구결과 (Jankovic, 2008) 와같이두집단은많은면에서유사성을보이나본연구에서는운동장애의중증도나 POT가비슷할때 MSA-p에서경축증상이더심한것으로나타났다. 하지만본연구의대상자수가제한적이고두집단의발병후경과일수가평균 3-5년정도라는요인이결과에영향을미쳤을수있는점을고려해야할것이다. MSA-c와 MSA-p 집단간차이는운동불능증과경축과같이파킨슨증을주된특징으로하는 MSA-p 집단과소뇌기능이상으로인해실조증을주특징으로하는 MSAc 집단간의차이로인해나타나는결과로이는기존의연구와일치 554

5 특발성파킨슨병과파킨슨플러스증후군환자의마비말장애및인지기능특성비교 김자영외 하였다 (Wenning et al., 1994). 또한, 실조증은 IPD와 MSA-c 집단, MSA-c와 MSA-p 집단, 그리고 MSA-p와 PSP 집단간에유의미한차이를보였는데이는앞서언급한바와같이파킨슨증을주특징으로하는 IPD와 MSA-p 집단과실조증을주된특징으로하는 MSA-c 집단간유의미한차이를보인것으로이전연구와일치하는결과이다. 단, MSA-p와 PSP 집단간차이는 PSP 집단의가장특징적인증상이경직된형태의운동장애 (akinetic-rigid form) 라는기존의연구가있으나본연구에서두집단간에는균형상실등의실조증이유의한특징인것으로관찰되었다 (Hughes et al., 1992). 본연구결과, 운동느림증은기존의연구에서와같이세집단에서모두관찰되는특성으로감별진단을위한유의한요소는아닌것으로관찰되었다. 말측면에서살펴보면, 집단간마비말장애중증도에는차이를보이지않았지만그특성은다른것으로나타났다. 우선, 네집단간에유의미한차이를보인마비말장애특성은과소운동형 (hypokinetic) 과실조형 (ataxic) 특성인것으로나타났다. 과소운동형은 IPD와 MSA-c 집단, 그리고 MSA-c와 MSA-p 집단간에차이를보였는데 IPD와 MSA-p 집단은파킨슨증을주증상으로하는집단으로기존의연구와마찬가지로과소운동형특성이빈번하게관찰되었고그빈도또한비슷하였다. 실조형은 IPD와 MSA-c 집단, MSA-c와 MSA-p 집단, 그리고 MSA-c와 PSP 집단간에유의미한차이를보였다. MSA-c 집단에서소뇌이상으로인해실조형이빈번하게관찰된다고보고한선행연구와같이 IPD, MSA-p와 MSA-c 집단간차이는과소운동형과함께실조형에서도유의미하게나타났다. 또한 PSP 환자들에게과소운동형-경직형의혼합형마비말장애나과소운동형-경직형- 실조형의특성이혼합되어나타난다는기존의연구 (Ackermann & Ziegler, 1991; Duffy, 2004; Kluin et al., 1996, 2001; Sachin et al., 2008) 가있으나 MSA-c 집단과비교했을때실조형특성은상대적으로약한것으로나타났다. 과소운동형의빈도는 MSA-p, IPD, PSP, MSA-c 집단순으로관찰되었는데, MSA-c 집단에서는현저하게낮은빈도가관찰되었으나나머지세집단간의빈도는큰차이를보이지않았다. 또한, 실조형은 MSA-c 집단에서만두드러졌고, 나머지세집단간빈도차이는크지않았다. 그결과, 소뇌기능이상으로나타나는두드러진특성을가진 MSA-c 집단을제외하고나머지집단에서는기존의연구에서와같이집단간마비말장애유형의중복되는특성으로여전히감별진단이어렵다는점이시사되었다. 인지능력도네집단을감별진단하기위한유용한요소였는데, IPD와 PSP 집단간에그리고 MSA-c와 PSP 집단간에차이가나타났다. IPD와 PSP 집단간차이는두집단의전두엽기능을비교한 연구에서 PSP 집단의수행결과가유의하게낮은것으로보고된연구와일치하였다 (Dubois, Pillon, Legault, Agid, & Lhermitte, 1988). 또한, 정상인과 MSA-p, MSA-c 집단간신경심리검사를실시한결과 MSA-c 집단은정상인집단과큰차이를보이지않았지만 MSAp 집단에서는인지기능능력의심한저하가관찰되었다는연구에기초할때 MSA-c와 PSP 집단간차이는위와같은맥락으로해석될수있다 (Kawai et al., 2008). 이로인해 PSP 집단이보이는전두엽-선조체기능의심한결함이재확인되었다. 본연구의제한점은집단별대상자수가적어일반화에제한이있을수있고, 하위검사에대한분석이나환자수행에대한질적분석을포함하지못했다는점이다. 그러나본연구에서는 IPD, MSAc, MSA-p, PSP 환자들의말, 인지기능측면을살펴봄으로써네집단을감별진단하는요소가무엇인지알아보고자하는시도를하였다는것에의의가있다. 그결과기존의연구와일치하거나다소차이를보이는항목이모두관찰되었는데향후본연구를토대로많은수의환자와증례를수집하고진단하부유형을세분화하여감별진단을위한요소를알아보는데도움이되길기대해본다. REFERENCES Ackermann, H., & Ziegler, W. (1991). Articulatory deficits in parkinsonian dysarthria: an acoustic analysis. Journal of Neurology, Neurosurgery & Psychiatry, 54, Dubois, B., Pillon, B., Legault, F., Agid, Y., & Lhermitte, F. (1988). Slowing of cognitive processing in progressive supranuclear palsy: a comparison with Parkinson s disease. Archives of Neurology, 45, Duffy, J. R. (2005). Motor speech disorders: substrates, differential diagnosis, and management (2nd ed.). St. Louis, Mo: Mosby. Gelb, D. J., Oliver, E., & Gilman, S. (1999). Diagnostic criteria for Parkinson disease. Archives of Neurology, 56, Hughes, A. J., Daniel, S. E., Kilford, L., & Lees, A. J. (1992). Accuracy of clinical diagnosis of idiopathic Parkinson s disease: a clinico-pathological study of 100 cases. Journal of Neurology, Neurosurgery & Psychiatry, 55, Jankovic, J. (2008). Parkinson s disease: clinical features and diagnosis. Journal of Neurology, Neurosurgery & Psychiatry, 79, Kawai, Y., Suenaga, M., Takeda, A., Ito, M., Watanabe, H., Tanaka, F.,... Sobue, G. (2008). Cognitive impairments in multiple system atrophy: MSA- C vs MSA-P. Neurology, 70(16 Part 2), Kim, H. (2012). Neurologic speech language disorders. Seoul: Sigmapress. Kluin, K. J., Gilman, S., Foster, N. L., Sima, A. A., D Amato, C. J., Bruch, L. A., 555

6 Ja Young Kim, et al. Dysarthria and Cognitive Function in Parkinson s Disease... Johanns, J. (2001). Neuropathological correlates of dysarthria in progressive supranuclear palsy. Archives of Neurology, 58, Kluin, K. J., Gilman, S., Lohman, M., & Junck, L. (1996). Characteristics of the dysarthria of multiple system atrophy. Archives of Neurology, 53, Lange, K. W., Tucha, O., Alders, G. L., Preier, M., Csoti, I., Merz, B.,... Naumann, M. (2003). Differentiation of parkinsonian syndromes according to differences in executive functions. Journal of Neural Transmission, 110, Maher, E. R., Smith, E. M., & Lees, A. J. (1985). Cognitive deficits in the Steele- Richardson-Olszewski syndrome (progressive supranuclear palsy). Journal of Neurology, Neurosurgery & Psychiatry, 48, Piatt, A. L., Fields, J. A., Paolo, A. M., & Tröster, A. I. (1999). Action (verb naming) fluency as an executive function measure: convergent and divergent evidence of validity. Neuropsychologia, 37, Piatt, A. L., Fields, J. A., Paolo, A. M., Koller, W. C., & Tröster, A. I. (1999). Lexical, semantic, and action verbal fluency in Parkinson s disease with and without dementia. Journal of Clinical and Experimental Neuropsychology, 21, Rajput, A., & Rajput, A. H. (2001). Progressive supranuclear palsy: clinical features, pathophysiology and management. Drugs & Aging, 18, Robbins, T. W., James, M., Owen, A. M., Lange, K. W., Lees, A. J., Leigh, P. N.,... Summers, B. A. (1994). Cognitive deficits in progressive supranuclear palsy, Parkinson s disease, and multiple system atrophy in tests sensitive to frontal lobe dysfunction. Journal of Neurology, Neurosurgery & Psychiatry, 57, Rossetti, H. C., Lacritz, L. H., Cullum, C. M., & Weiner, M. F. (2011). Normative data for the Montreal Cognitive Assessment (MoCA) in a populationbased sample. Neurology, 77, Sachin, S., Shukla, G., Goyal, V., Singh, S., Aggarwal, V., & Behari, M. (2008). Clinical speech impairment in Parkinson s disease, progressive supranuclear palsy, and multiple system atrophy. Neurology India, 56, Tekin, S., & Cummings, J. L. (2002). Frontal-subcortical neuronal circuits and clinical neuropsychiatry: an update. Journal of Psychosomatic Research, 53, Verny, M., Duyckaerts, C., Agid, Y., & Hauw, J. J. (1996). The significance of cortical pathology in progressive supranuclear palsy Clinico-pathological data in 10 cases. Brain, 119, Wenning, G. K., Shlomo, Y. B., Magalhaes, M., Danie, S. E., & Quinn, N. P. (1994). Clinical features and natural history of multiple system atrophy: an analysis of 100 cases. Brain, 117,

7 특발성파킨슨병과파킨슨플러스증후군환자의마비말장애및인지기능특성비교 김자영외 국문초록 특발성파킨슨병과파킨슨플러스증후군환자의마비말장애및인지기능특성비교 김자영 1 정선주 2 이재홍 2 권미선 2 1 서울아산병원신경과, 2 울산대학교의과대학서울아산병원신경과 배경및목적 : 본연구는공통적특징으로감별진단이어려운특발성파킨슨병 (idiopathic Parkinson s disease, IPD) 과파킨슨플러스증후군환자의말 / 인지기능특징을살펴보고, 감별진단을위한유용한요소인지알아보고자하였다. 방법 : IPD 환자 11명과파킨슨플러스증후군중산발적올리브교소뇌위축 (sporadic olivopontocerebellar atrophy, MSA-c) 환자 10명, 선조흑질변성 (Striatonigral degeneration; Parkinsonian variant; MSA-p) 환자 10명, 진행성핵상마비 (Progressive supranuclear palsy, PSP) 환자 10명 ( 총 41명 ) 을대상으로파킨슨병등급척도 (Unified Parkinson s Disease Rating Scale, UPDRS) 의세부항목중경축, 운동느림증, 실조증의각점수를합친것을기준으로네집단의운동기능장애중증도를통제한후마비말장애유형및인지능력을평가하였다. 결과 : UPDRS 운동기능능력을세분화하여분석한결과, 경축은 IPD-MSA-p 집단과 MSA-c-MSA-p 집단간, 실조증은 IPD-MSA-c 집단, MSA-c-MSA-p 집단, MSA-p-PSP 집단간유의미한차이를보였다 (p<.05). 또한, 마비말장애유형중과소운동형은 IPD-MSA-c 집단과 MSA-c-MSA-p 집단간유의하였고, 실조형은 IPD-MSA-c 집단과 MSA-c-MSA-p 집단, MSA-c-PSP 집단간유의했다 (p<.01). Korean version of Montreal Cognitive Assessment (MoCA-K) 점수에서 PSP 환자는 IPD, MSA-c 환자에비해유의미하게낮은인지기능을보였다 (p<.05). 논의및결론 : 마비말장애유형과인지능력특성이네집단을감별진단하는데유용할수있음을시사하였는데더많은수의증례를포함해진단세부유형별연구가이루어지길기대해본다. 핵심어 : 특발성파킨슨병, 파킨슨플러스증후군, 마비말장애, 인지기능, 감별진단본연구는 2010년도정부 ( 교육과학기술부 ) 의재원으로한국연구재단의지원을받아수행된기초연구사업임 (No ). 참고문헌 김향희 (2012). 신경언어장애. 서울 : 시그마프레스

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