노인퇴행성신경질환의마비말장애특성 김선우 a 김향희 a,b, a 연세대학교대학원언어병리학협동과정, b 연세대학교의과대학재활의학교실 교신저자김향희연세대학교대학원언어병리학협동과정교수서울시서대문구성산로 250번지연세의료원재활병원 3층 e-mail: h.kim@yonsei.ac.kr tel.: 02-2228-3900 배경및목적 : 고령화사회진입에따른퇴행성신경질환의유병률과발병률증가는임상에서효과적인질환중재의방향설정을위한감별진단의중요성을부각시켰다. 본연구는퇴행성신경질환의마비말장애를보고한국외문헌을수집하여각질환별로말특성을기술하고, 개별적말특성이질환간의감별에유용한요소인지를검토하였다. 방법 : 50 세를기준으로한준고령자이상의연령층에서뇌신경손상을동반하는대표적퇴행성신경질환인파킨슨병, 피질기저핵변성, 진행성핵상마비, 다계통위축증, 그리고전두측두엽치매분야에해당하는논문들을검색한뒤, 이자료에서마비말장애를보고한관련연구들을정리하였다. 결과 : 관련퇴행성신경질환군에서마비말장애특성이보고된논문의수는 1978 년에서 2008 년까지총 58 편이었다. 한편의논문에서두개이상의질환군이언급된경우는개별논문으로취급하여파킨슨병 42 편, 피질기저핵변성 6 편, 진행성핵상마비 5 편, 다계통위축증 3 편, 그리고전두측두엽치매 5 편으로분석되었다. 공통으로관찰되는저운동형마비말장애, 다양한혼합형마비말장애혼재, 발병초기에관찰되는파킨슨증, 병인과병소부위의유사성등으로인해퇴행성신경질환군간의감별진단에어려움이있음이재확인되었다. 논의및결론 : 비록선행연구들에서이들질환군을감별할수있는명확한기준은제시되지못했지만질환군에따른필수적으로관찰되는마비말장애유형과좀처럼관찰되지않는유형을제시하여말검사를통한퇴행성신경질환의변별가능성을시사하였다. 언어청각장애연구, 2009;14(1);82-95. 핵심어 : 퇴행성신경질환, 마비말장애, 파킨슨병, 피질기저핵변성, 진행성핵상마비, 다계통위축증, 전두녹두엽치매 Ⅰ. 서론 의료기술의발달, 생활수준의개선, 건강에대한관심및투자증가, 그리고충분한영양섭취등으로인해전세계적으로평균수명이날로증가하고있는추세이다. 2007년 7월 1일을기준으로발표된보건복지가족부의고령자통계에따르면, 우리나라전체인구중에 65세이상의노인은 4,810,000명으로총인구의 9.9 % 를차지하며, 2018년에는 14.3 %, 2026년에는 20.8 % 로급증하여초고령사회로의진입을예상했다. 노인인구는경제활동인구층인청 장년층에비해특히, 퇴행성신경질환의위험에노출되어있다. 퇴행성신경질환이란뇌와척수를포함한중추신경계의노화가진행됨에따라기능이상과장애를동반하는것을일컫는다. 공통된병인으로는신경세포내외에걸친단백질의비정상적축적과그에따른 단백질의응집및침착이확인되고있다 ( 백승렬 이정호, 2007). 퇴행성신경질환은신경말 언어측면에서볼때에높은빈도로마비말장애, 말실행증및실어증을동반한다. 이러한말 언어장애는퇴행성신경질환의초기증상 ( 예 : 진행성마비말장애 (progressive dysarthria); 원발성진행성실어증 (primary progressive aphasia)) 으로관찰되기도하며, 신경질환간의감별진단에이용되기도한다 (Duffy, 2008). 기존선행연구들에서퇴행성신경질환이말장애를동반하며, 병의진전에따라마비말장애의중증도가심해지며, 신경질환군에따른마비말장애하위유형간에차이가있다고보고되었다. 그럼에도불구하고, 노인성퇴행성신경질환의말특성을비교보고한연구는없는실정이다. 이에, 본연구는노인퇴행성신경질환과관련된국외마비말장애연구를종합하여각질환별로말특성을정리하고, 더나아가말특 게재신청일 : 2009 년 1 월 22 일 최종수정일 : 2009 년 3 월 6 일 게재확정일 : 2009 년 3 월 16 일 c 2009 한국언어청각임상학회 http://www.kasa1986.or.kr 82
김선우 김향희 / 노인퇴행성신경질환의마비말장애특성 성차이가유사질환인파킨슨증 (parkinsonism) 의감별진단에유용한요소인지를검토하고자한다. 1. 마비말장애 Darley, Aronson & Brown (1969a; 1969b; 1975) ( 이하 DAB) 에따르면, 마비말장애 (dysarthria) 란중추및말초신경계의손상에기인한말산출기제의근육조절장애를통칭하는말장애군으로서, 말조절근육의마비, 약화, 비협응으로인해구어의사소통에문제가동반된상태 라고정의했다. 이후, 후속연구들을통하여이전 DAB의정의에포함되지않았던요소들을포함하고, 개념을명확하고, 구체화하기위한목적으로아래와같이재수정된정의가제안되었다. 마비말장애란말산출에관여하는호흡, 발성, 공명, 조음및운율을조절하는과정에서힘, 속도, 항상성, 범위, 긴장및정확성에비정상적문제가수반된신경학적말장애를의미한다. 중추및말초신경계의이상이병리의원인이며, 약화, 경직, 비협응, 불수의적움직임, 근육긴장이상 ( 증가, 감소또는가변 ) 이높은빈도로관찰된다. (Duffy, 2005). 즉, 마비말장애란중추적으로는말산출을계획, 체계화, 그리고집행하는중추신경계의뇌손상과관련되며, 말초적으로는말산출명령을전달하고수행하는일련의과정에관여하는신경세포, 축삭, 신경근육연접및근육등의문제로인한부정확한말의표출인것이다. 2. 퇴행성질환군본문헌연구는노령화에따른퇴행성신경질환으로그범위를제한하기위해평균발병연령이준고령자 (50세이상성인 ) 인다음의 5개질환군을대상으로하였다. 첫째, 파킨슨병 (Parkinson s disease: PD, 이하 PD) 은기저핵 (basal ganglia) 의일부인흑색질 (substantial nigra) 의도파민생성저하로인하여, 운동을계획하고실행하는전두엽의기능장애를동반한중추신경계퇴행성운동질환이다. 대부분의파킨슨병이그원인을알수없는특발성파킨슨병 (idiopathic PD) 으로알려져있지만독성물질, 약물, 대뇌손상, 유전적변형등에의해이차적으로발병하기도한다. 파킨슨병은일반적으로 50세이상의인구에서발생하며, 운동 (motor) 과비운동 (non-motor) 에서기능장애를 초래한다. 운동영역의일차적특징으로는파킨슨증이라불리는떨림 (tremor), 운동느림 (bradykinesia), 자세불안정 (postural instability), 그리고경직 (rigidity) 이관찰된다. 이차적으로는무표정 (hypomimia), 마비말장애, 삼킴장애, 과잉침분비, 소자증 (micrographia), 발끌기, 고정 (freezing), 가속보행 (festination), 근육긴장이상및미간반사 (glabella reflexes) 등이있다. 비운동영역은자율계기능이상, 인지 신경행동장애, 수면장애및감각장애가동반된다 (Jankovic, 2008). 둘째, 피질기저핵변성 (corticobasal degeneration; cortico-striatal-nigral degeneration; cortico-basalganglionic degeneration: CBD, 이하 CBD) 은대뇌피질과피질하의조직이상으로발병하며, 레바도파약물에잘반응하지않으며, 비대칭적무운동성경직증후군, 근이긴장증, 실행증, 피질성감각소실, 통제불능손 (alien hand)( 김정은외, 2005) 의주증상에인지 기억력장애, 실어증, 말실행증및마비말장애가동반된다. 일반적으로 60세에서 80세사이에발병하며, 점차적으로악화되어발병후 5년에서 15년사이에사망하는것으로알려져있다. 셋째, 진행성핵상마비 (progressive supranuclear palsy: PSP, 이하 PSP) 는 1964년에 Steele, Richardson & Olszewski 에의해첫임상사례가보고된희귀퇴행성질환이다 (Litvan et al., 1996). 진행성핵상마비는기저핵, 뇌간, 대뇌피질및소뇌치상핵에타우단백질 (tau protein) 이축적되어형성된신경섬유에의한신경교세포 (neuroglial cells) 의점진적인괴사로발생한다. Verny et al. (1996) 은흑색질, 담창구 (globus pallidus) 및시상밑핵 (subthalamic nucleus) 에서심각한병리이상이발견된점을언급하면서이영역에서부터퇴행의시작가능성을주장하였다. 발병초기부터균형상실, 성격변화, 운동느림, 핵상안구운동마비및마비말장애의소견을보이며, 말기에는치매와삼킴장애를동반하는것으로알려져있다. 발병평균연령은 63세이며, 평균생존기간은 7년으로보고되었다. 넷째, 다계통위축증 (multiple system atrophy: MSA, 이하 MSA) 은기저핵, 소뇌, 뇌간및척수등의다양한중추신경계에서산발적으로발생하며, 점진적인신경소실과신경교증 (gliosis) 을동반하는퇴행성질환이다 ( 김용덕외, 1999). 자가면역, 독성물질또는외상의영향이제기되고있지만현재까지명확 83
언어청각장애연구 2009;14;82-95 한원인은밝혀지지않았다. 평균발병연령은 52세에서 55세이며, 평균생존기간은 6년에서 9년으로알려져있다. 일반적으로자율신경계징후및배뇨이상을첫증상으로하며, 레바도파약물에반응하지않는파킨슨증및소뇌기능이상을특징으로한다. 다계통위측증에는하위유형이있는데, 자율신경계이상이주증상인경우는샤이-드래거증후군 (Shy- Drager syndrome; MSA-A), 파킨슨증을특징으로하는추체외로이상은선조흑질변성 (striatonigral degeneration; parkinsonian variant; MSA-P), 소뇌기능장애는산발적올리브교소뇌위축 (sporadic olivopontocerebellar atrophy; MSA-C) 으로구분된다. 다섯째, 전두측두엽치매 (frontotemporal lobar degeneration: FTLD; dementia of frontal lobe type; frontal lobe degeneration of non-alzheimer type) 는 1892년 Arnold Pick에의해첫측두엽위축증례가보고되었으며, 1925년에픽병 (Pick s disease) 으로명명되었다 (Neuman & Cohn, 1967). 이후에치매를동반하는임상병리학적증례가점차적으로증가하면서용어의통일성이제기되어 1994년에전두측두엽치매로통합명명되었다. 대뇌전두엽과측두엽에서신경교증형, 전두엽퇴행형및운동신경원질환형의조직학적이상소견을보이는이질적장애집단을통칭하며, 평균 50대말에발병한다 ( 박기정 윤수진 나덕렬, 2005). 65세이하인구에서는알츠하이머병다음으로두번째의, 65세이상에서는네번째의치매발병원인으로알려져있다. 전두측두엽치매는전두측두치매 (frontotemporal dementia), 의미치매 (semantic dementia), 그리고진행비유창실어증 (progressive nonfluent aphasia) 의세하위유형으로분류된다. 3. 퇴행성질환과마비말장애 1987년에서 1990년, 그리고 1993년에서 2001년사이에 Mayo Clinic에서진단된퇴행성신경질환자들의마비말장애하위유형을분석한자료를아래 < 표 -1> 에제시하였다. 본자료에서혼합형은제외되었지만 Duffy (2005) 는아래유형들이결합한다양한마비말장애의관찰가능성을언급하였다. < 표 - 1> 병인에따른마비말장애하위유형의분류 질환 하위유형 이완형 경직형 실조형 저운동형 과운동형 일측상부운동신경세포형 PD a) - i) - - ++ k) - - CBD b) - + + + + - PSP c) - + + + - - Pick s D d) - - - ++ - - MSA e) f) SDS + j) + + + - - SND g) - + + + + - OPCA h) + + + + - - a) PD: Parkinson s Disease, 파킨슨병 ; b) CBD: cortico-basalganglionic degeneration, 피질기저핵변성 ; c) PSP: progressi ve supranuclear palsy, 진행성핵상마비 ; d) Pick s D: Pick s Disease, 픽병 ; e) MSA: multiple system atrophy, 다계통위축증 ; f) SDS: Shy-Drager syndrome, 샤이-드래거증후군 ; g) SND: striatonigral degeneration, 선조흑질변성 ; h) OPCA: olivopontocerebellar atrophy, 올리브교소뇌위축 ; i) -: 관찰불가능함 ; j) +: 관찰됨 ; k) ++ : 빈번하게관찰됨. * 본표는 Duffy, J. R. (2005) 의 Motor speech disorders: Substrates, differential diagnosis, and management (2nd ed.) 의내용중 15장의 differential diagnosis를재수정한것임. 1. 자료선정절차 Ⅱ. 연구방법 퇴행성신경질환과관련된마비말장애연구자료를수집한절차는다음과같다. PubMed, ProQuest, 그리고 EBSCOhost 의검색엔진을활용하여 neurodegenerative disorder, degenerative disorder, dysarthria, Parkinson s disease, corticobasal degeneration, progressive supranuclear palsy, multiple system atrophy, 그리고 frontotemporal lobar degeneration 등의핵심어로관련논문을검색하였다. 검색된논문중에서일어, 스페인어또는불어등으로기술되어해석이불가능한경우는자료에서제외하여최종적으로 1978년에서 2008년까지총 58 편의논문이수집되었다. 한편의논문이두개이상의관련질환군을연구한경우는각각을개별논문으로취급하였다. 84
김선우 김향희 / 노인퇴행성신경질환의마비말장애특성 2. 자료분석수집된자료들은말산출하위체계인호흡, 발성, 공명, 조음그리고운율별로말특성을기술하였다. Ⅲ. 연구결과 최종적으로각질환별논문편수는파킨슨병 42편, 피질기저핵변성 6편, 진행성핵상마비 5편, 다계통위축증 3편, 전두측두엽치매 5편으로서, 파킨슨병이가장많았다 (< 그림 -1>). 각질환별로논문에포함된총환자수를살펴보면, 파킨슨병은 788명 ( 편당평균 18.76명 ), 피질기저핵변성은 59명 ( 편당평균 9.83명 ), 진행성핵상마비는 100명 ( 편당평균 20.0명 ), 다계통위축증은 101명 ( 편당평균 33.66명 ), 그리고전두측두엽치매는 9명 ( 편당평균 1.80명 ) 이었다. 이로써, 총논문수 42편, 총환자수 788명으로파킨슨병에집중된퇴행성신경질환마비말장애의연구동향이확인되었다. 1. 파킨슨병파킨슨병의마비말장애하위유형은주로저운동형으로보고되었으며 (Ackermann & Ziegler, 1991; Adams, 1994; Caligiuri, 1989; Goberman & Elmer, 2005; Ho, Iansek & Bradshaw, 2001; Kent et al., 2003; Letter, Santens & Borsel, 2005; Murdoch et al., 1997; Ozsancak et al., 2006; Sachin et al., 2008; Tjaden, 2003; Yorkston, 2007), 말산출의하위체계별말특징은다음과같았다. 먼저, 호흡에있어서는성문저항증가, 성문하압저하, 짧은평균발화길이, 짧은발화내쉼등이보고되었으나, 발성호기율은정상이었다 (Murdoch et al., 1997). 발성의음량 (loudness) 면에서는평이음량 (monoloudness), 평균음량의저하 (Tjaden & Wilding, 2004; 2005), 저하된음량변이 (Letter et al., 2007), 그리고말운동하부체계의불안정이반영된점차적이며뚜렷한말음량의감소 (Ho, Iansek & Bradshaw, 2001) 가보고되었다. 음도 (pitch) 는기본주파수의편차는증가 (Zwirner & Barnes, 1992) 했으며, 남성의기본주파수는증가 (Hertrich & Ackermann, 1995) 한반면에여성의기본주파수범위는감소 (Doyle et al., 1995) 하였다. 조음기관의불안정을반영하는제 l음형대주파수의변이증가 (Doyle et al., 1995), 제 2음형대의범위감소, 최대주파수범위의감소 (Holmes et al., 2000), 그리고평이음도등이관찰되었다. 그리고, 정상군에비해청지각적모음변별에서기본주파수의중요성증가 (Bunton, 2006) 가보고되었다. < 그림 - 1> 1978 년에서 2008 년에발표된퇴행성신경질환의마비말장애논문수 a) PD: Parkinson s Disease, 파킨슨병 ; b) CBD: cortico-basal-ganglionic degeneration, 피질기저핵변성 ; c) PSP: progressive sup ranuclear palsy, 진행성핵상마비 ; d) MSA: multiple system atrophy, 다계통위축증 ; e) FTLD: frontotemporal lobar degeneratio n, 전두측두엽치매. 85
언어청각장애연구 2009;14;82-95 음질 (voice quality) 은음성떨림, 약한발성지수 (soft phonation index) 증가, 배음대소음비율저하, 숨찬, 쉰또는거친음성이보고되었다. 공명은과비성이일부연구에서만확인되었다 (Kempler & Lancker 2002; Tjaden, 2003). 조음은부정확한자음, 상대적으로성도의긴장을요구하는폐쇄자음에서오류증가 (Ozsancak et al., 2001), 감소되었지만정상군과유의한차이를보이지않는모음길이, 정상군에비해상대적으로감소한음절길이에비해서증가한쉼길이 (Hammen & Yorkston, 1996), 말중증도가심해질수록감소하는음향구간 (McRae, Tjaden & Schoonings, 2002), 뭉치는듯한말소리 (Weismer et al., 2001), 가변적인말명료도 (Miller et al., 2007), 자발화과제에서현저하게저하된말명료도 (Kempler & Lancker, 2002), 일상발화속도과제에서동시조음의증가 (Tjaden, 2000b), 동일연령군에비해빠른말속도 (Weismer, 1984), 변이성을보이는말속도, 조음교대운동속도과제에서음절간의좁은간격, 정상교대운동반복횟수및느린일련운동반복횟수 (Tjaden & Watling, 2003), 교대운동반복에서자가간격조절력의결함 (Ackermann, Konczak & Hertrich, 1997), 그리고제한된조음기관의움직임 (Ackermann & Ziegler, 1991) 등을특징으로하였다. 마지막으로, 운율은강세꼭지점의높이저하, 강세음절로기본주파수정점의치우침 (Penner et al., 2001) 및강세및대조저하가관찰되었다. 그리고동반된인지와감정장애의영향으로인한운율장애의가능성도제기되었다 (Caekebeke et al., 1991). 2. 피질기저핵변성피질기저핵변성의하위유형은저운동형, 경직형, 그리고혼합형으로보고되었다 (Frattali & Sonies, 2000). 저운동형은얕은흡기, 감소된호기조절능력, 음량감소, 쉰소리, 변이성이있는부정확한말명료도, 전반적으로느리지만간헐적으로관찰되는빠른말터짐, 그리고단조로운강세등이보고되었다. 공명은정상으로언급되었다. 경직형은호흡의압력생성력저하, 쥐어짜는듯한음성, 과비성, 부정확한조음, 느린말속도, 노력성발화및과도한음절강세를특징으로하였다. 이밖에도 2명을대상으로한임상사례보고 (Thűmler et al., 2003) 에서는단조로운말소리, 경직되고눌린듯한조음, 그리고떨리는듯한혀의움직임이언급되었다. 발병부위의산재로인해혼합형마비말장애가보고되었으며 (Ozsancak et al., 2006), 저운동형우세, 과운동형우세, 그리고경직형우세로세부분류되었다 (Frattali & Sonies, 2000). 이중에서저운동형우세는평이음도및평이음량등의저운동형의말특성에과비성및쥐어짜는음성이관찰되었다. 이밖에도느린말속도및일관적운율장애 (global prosody) 가함께보고되었다. 과운동형우세는불규칙하며갑작스럽게힘이들어간호흡, 쥐어짜는듯한거친음성, 음성떨림, 과비성, 조음부정확및소리연장이관찰되었다. 경직형우세는경직형마비말장애의특성에발성부전과정상공명이보고되었다. 세명의환자를대상으로한임상증례 (Bergeron et al., 1996) 에따르면, 구체적으로마비말장애의하위유형은기술되지않았지만저운동형의특징인발성부전과평이음도가대상환자의말특성으로보고되었다. 3. 진행성핵상마비진행성핵상마비는저운동-경직혼합형 (Sachin et al., 2008) 과저운동-경직-실조혼합형 (Kluin et al., 2001) 의마비말장애로보고되었다. 혼합형의저운동형특징으로는지시에의한발성강도의증가는가능하지만일반적으로저하된음량, 음량의점차적감소, 발성시작의어려움, 발성부전, 평이음도, 지속적인숨찬음성, 부정확한조음, 말뭉침, 말속도의증가, 부적절한쉼, 음절, 단어및구의반복현상, 그리고강세감소가언급되었다. 경직형의특색으로는저음도, 평이음도, 평이음량, 쥐어짜는듯한음성, 지속적인쉰음성, 부정확한조음, 음소와쉼의연장, 느린말속도, 지속적과비성및감소된강세가관찰되었다. 실조적특징으로는가청흡기 (audible inspiration), 음량과음도변이, 일시적으로관찰되는거칠거나숨찬음성, 음성떨림, 불규칙한조음오류, 말속도의변이, 일시적인과비성, 그리고과도한동일강세의사용이보고되었다 (Kluin et al., 1993; 2001). 86
김선우 김향희 / 노인퇴행성신경질환의마비말장애특성 4. 다계통위축증다계통위축증은저운동형-실조형-경직형, 저운동형-실조형, 저운동형-경직형의혼합형으로보고되었다 (Kluin et al., 1996). 저운동형특징은지시에의해말소리의증가가가능한저하된음량, 점차적음량의감소, 지속적인숨찬음성, 부정확한조음, 말뭉침, 말속도의증가, 부적절한쉼, 음절, 단어및구의반복현상, 그리고강세감소가관찰되었다. 경직형은저음도, 평이음도, 평이음량, 쥐어짜는듯한음성, 지속적인쉰음성, 부정확한조음, 음소와쉼의연장, 저하된말속도, 지속적과비성및강세감소가보고되었다. 실조형은청지각적으로인식되는흡기, 음량과음도의변이, 일시적으로관찰되는거칠거나숨찬음성, 음성떨림, 불규칙한조음오류, 말속도의변이, 일시적인과비성, 그리고과도한동일강세가특징으로언급되었다. 마비말장애의하위유형이구체적으로서술되지는않았지만한연구 (Sachin et al., 2008) 에의하면, 다계통위축증은음량저하, 음량변이, 평이음도, 쥐어짜는음성, 거친음성, 조음정확성저하, 말속도변이및강세감소를보였다. 그리고하위유형중에서선조흑질변성 (SND; MSA-P) 은저운동형우세로, 올리브교소뇌위축 (OPCA; MSA-C) 은실조형우세의마비말장애로보고되었다. 샤이-드래거증후군을대상으로한연구는실조형, 저운동형, 그리고혼합형으로마비말장애를분류하였다 (Linebaugh, 1979). 실조형은음소오류의불규칙성, 조음기관의운동범위와시간의불규칙, 비운율을동반한빠른 퍼, 터, 커 수행력및전사발화 (scanning speech) 가보고되었다. 저운동형은음량저하, 빈번한음소오류, 말뭉침, 과도하게빠른음절반복속도, 저하된조음기관의움직임, 조음근육의경직, 그리고강세저하를특징으로하였다. 혼합형은저운동형-실조형, 실조형-양측추체로형, 그리고경직형-실조형-저운동형으로분류되어언급되었다. 저운동-실조혼합형은저운동형의특징인음도와음량의범위감소, 숨찬음성, 말뭉침, 과도하게빠른음절반복속도, 조음기관의움직임범위감소, 뚜렷한강세감소와함께실조성의특징인불규칙한조음오류, 음절반복과제에서비운율의증가가보고되었다. 실조-양측추체로혼합형은쥐어짜는듯한음성, 불규칙한조음의깨어짐, 음소연장, 느린말속도, 동일강세및비운율적음절반복을특징으로하였다. 경직- 실조-저운동혼합형은경직형의특징인쥐어짜는듯한음성, 과비성및느린음절반복속도가보고되었다. 여기에실조형의특징인조음의불규칙한깨어짐, 발화내의동일한강세에저운동형의특징인음도및음량범위제한, 말뭉침및조음기관의움직임제한이더불어관찰되었다. 5. 전두측두엽치매전두측두엽치매는마비말장애의하위유형이문헌내에서구체적으로언급되지않았다. 한명의피험자를대상으로한 Ihori et al. (2006) 에의하면짧은최대발성시간, 발성부전, 장모음의단모음화, 조음기관의정상운동범위, 경미하게저하된교대운동반복횟수, 단조로운억양, 불필요한모음연장과운율이상, 그리고선율과리듬이단조로운노래가특징으로보고되었다. 역시, 한명을대상으로한 Fukui et al. (1996) 에따르면평이음도, 거친음성, 자음과모음의왜곡, 단어내음소위치변경, 느린말속도, 노력성발화및비음화등이특징으로관찰되었다. 전두측두엽치매를임상에서진단하기위한기준합의 (Neary et al., 1998) 에의하면, 이들의말특성으로자발성이감소된간결한말산출과억눌린듯한말소리가보고되었다. Ⅳ. 논의및결론 본연구의대상이었던다섯개의질환군중에서전두측두엽치매를제외한파킨슨병, 피질기저핵변성, 진행성핵상마비, 그리고다계통위축증은파킨슨증을동반하는퇴행성신경운동장애로분류된다 (Litvan et al., 2003). 현재까지사전 (antemortem) 에퇴행성신경질환인파킨슨증을감별하는생물학적인표지 (biological markers) 가존재하지않아병력, 관찰되는임상특성, 병의진전양상, 그리고뇌자기공명영상 (magnetic resonance imaging:mri) 에의존한진단방법이보편적으로사용되고있다. 이로인해, 다계통위축증의하위유형인선조흑질변성은다른하위유형인샤이-드래거증후군이나올리브교소뇌위축에비해파킨슨증이지배적으로관찰되어발병초기에파킨슨병으로오진되는빈도가높으며, 사후부검을통해서확진되기도한다. 87
언어청각장애연구 2009;14;82-95 그렇다면, 유사신경질환이병리적사지증세 (limb symptom) 에만국한되지않고말특성에근거한감별또는확진이가능하게된다면진단은치료의방향설정과예후의예측잣대로서의기능은더욱확립될것이다. 이에대한가능성을살펴보기위해질환별로관찰되는말특색을살펴보는것뿐만아니라, 파킨슨증을보이는유사질환간의말차이점을비교하여궁극적으로는유사질환간의감별가능성을모색하고자한다. 파킨슨병은중간뇌의흑색질에서생성되는도파민의분비저하로인한저운동형마비말장애이다. 이에반하여, 피질기저핵변성, 진행성핵상마비, 그리고다계통위축증은타우단백질 ( 백승렬 이정호, 2007) 에의한대뇌손상에서기인한혼합형마비말장애이다. 보고된혼합형마비말장애의유형을살펴보면, 피질기저핵변성은저운동형-경직형-과운동형 (Frattali & Sonies, 2000) 으로, 진행성핵상마비는저운동형- 경직형-실조형 (Kluin et al., 2001) 으로, 그리고다계통위축증은저운동형-경직형- 실조형의혼합형 (Kluin et al., 1996) 으로보고되었다. 즉, 파킨슨증신경질환군은저운동형마비말장애의특성을공유하는것으로밝혀져비단사지에만국한되지않는유사한특성이말에도공존함이재확인되었다. 그러면파킨슨증을동반한퇴행성신경질환군간에말장애의출현시기, 개별적말특성, 그리고중증도면에서차이가있는지를알아보자. 먼저, 마비말장애의출현시기에관하여살펴보면, 초기피질기저핵변성은운동과관련된사지증세를주증상으로하며, 병이진전함에따라마비말장애가빈번하게발생 (Ozsancak, Auzou & Hannequin, 2000; Wenning et al., 1998) 하는것으로보고되었다. 반면에, 파킨슨병은발병초기부터음성문제를동반한마비말장애를호소하는것으로알려져있다 (Adams, 1997; Ho et al., 1998; Logemann et al., 1978; Stewart et al., 1995). 개별적인말특성을고찰해보면, 파킨슨병, 진행성핵상마비, 그리고다계통위축증은공통적으로저운동형마비말장애의특징인발성부전이보고되었다 (Sachin et al., 2008). 하지만, 파킨슨병은단조로운말소리, 간헐적인말뭉침 (Ackermann & Ziegler, 1991; Ho, Iansek & Bradshaw, 2001; Kent et al., 2003; Murdoch et al., 1997; Ozsancak et al., 2006) 등의저운동형특징만을보인반면, 진행성핵상마비 는저하된음량, 음량의점차적감소및말뭉침등과같은저운동형특성뿐만아니라발성시작의어려움, 쥐어짜는듯한음성및지속적인과다비성 (Kluin et al., 2001) 이동반되었다. 다계통위축증은파킨슨병및진행성핵상마비와유사한저운동형의특징에더불어조음의불규칙한깨어짐및발화내동일한강세의사용 (Linebaugh, 1979) 에서차이가있었다. 진행성핵상마비는다른두장애군에비해중증의말장애로보고되었으며 (Sachin et al., 2008), 저운동 -경직혼합형의진행성핵상마비는파킨슨병이나다계통위축증에비해상대적으로뚜렷하게저하된최대발성시간이언급되었다 (Sachin et al., 2008). 즉, 진행성핵상마비는이들의말특징을반영하는조음과호흡능력을반영하는검사항목이평가에포함되어야함이확인되었다. 다계통위축증과파킨슨병을대상으로교대운동속도과제를비교한국내연구에따르면, 다계통위축증에서유의미하게저하된평균반복횟수가보고되었다. 이는상대적으로저하된조음기의반복속도차이를이용한이두집단의변별가능성을시사하였다 ( 김향희외, 2003). 질환별말특성을기술하려는본연구들의공헌에도불구하고본문헌고찰에서제기된연구들의한계점은다음과같았다. 첫째, 마비말장애는주관적및객관적접근방식을사용한호흡, 발성, 공명, 조음및운율에대한청지각적, 음향학적, 그리고생리적검사가종합적으로이루어질때에정확한평가가가능하다. 그럼에도불구하고, 기존의연구들은말산출의하위영역중에서도조음과관련된말명료도의청지각적평가에집중된연구경향을보였다. 이로인해, 파킨슨병의경우는청지각적으로는빠른말속도로인식되지만실제로는정상군과유사한속도로분석된연구들이보고되면서청지각적뿐만아니라생리적검사를결합한평가방법의사용이권고되었다 (Tjaden, 2000a). 또한, 제한된연구방법은퇴행성신경질환대상자들의말산출에직접또는간접적으로작용하는관련하위체계의종합적이해부족을초래하였다. 둘째, 퇴행성신경질환과관련된마비말장애는파킨슨병에집중된연구경향을보였다. 수집된논문편수가반영하듯이파킨슨병이전체논문의 68.86% 를차지하여과반수를웃도는비중을차지하였다. 즉, 파킨슨병을제외한기타퇴행성신경질환은상대적 88
김선우 김향희 / 노인퇴행성신경질환의마비말장애특성 으로비교연구자료가불충분하여양적뿐만아니라질적가중치의근거가불충분하였다. 셋째, 각각의말하부체계과제에서사용된통일되지못한연구방법을들수있다. 예를들면, Kluin et al. (1993) 은문단읽기와그림묘사하기과제를사용하여 0점 ( 정상 ) 에서 3점 ( 심도 ) 으로진행성핵상마비의말명료도를청지각적으로평가하였다. 반면에, Frattali & Sonies (2000) 는음소균형을맞춘말자료를사용하여 1점 ( 정상 ) 에서 4점 ( 심도 ) 으로피질기저핵변성환자들을평가하였다. 즉, 두연구는조음과관련된말산출능력을평가했지만, 평가자극및평가척도의차이로이들퇴행성신경질환군간의수행력비교에는제한점이따른다. Kent & Kent (2000) 는마비말장애의평가를위해서는말과제에따른말산출능력의변이성에대한이해가선행되어야하며, 신경병리학적증상과운동체계의수행력을고려한타당한평가과제의수립과이의실시를주장하였다. 위에서제기된연구의한계점은체계적인말평가도구의제작, 통일된검사절차의실시, 그리고주관적및객관적방법을사용한종합적접근에대한부단한노력이선행될때에점차적인개선이이루어질것으로기대된다. 우리는증거기반접근 (evidence-based practice) 의시대에살고있다. 축척된자료를통해증명된사실은실체의본질을가장근접하게반영하고설명한다고할수있다. 마비말장애의개념을확립한 DAB (1969a; 1969b; 1975) 도증거에기반한비정상적말특징의상세한기술과범주화의노력은병리기전에대한통찰력을제공하고, 발병부위를감별하는잠재적가능성을내포한다고언급하였다. 우리는말평가를담당하는전문가로서치료사일뿐만아니라진단가이기도하며, 동시에연구자이다. 임상현장에서발표된임상증례들은퇴행성신경질환의감별진단을가능하게하는근거자료로사용될뿐만아니라, 이들에게제공하는재활서비스의토대를마련하는틀로활용될것이다. 이러한노력은비록많은수의노인퇴행성신경질환자들이빠른속도로병이진전되어사망에이르기는하지만생존기간동안이들의삶의질을개선한다는사실에는의심의여지가없다. 마지막으로, 재활서비스를제공하는사회의일원으로서, 고령화사회로의진입을앞둔현시점에서국가가준비하는복지정책에참여하는정책반영자로서의자세도필요하다. 참고문헌 김용덕 양진우 이현정 류철형 김원찬 이명식 (1999). 치료를받은적이없는원발성파킨슨병과다계통위축증환자들의 single oral levodopa challenge test. 대한신경과학회지, 17(1), 53-62. 김정은 한문구 임준성 백민재 김상윤 (2005). 피질기저핵변성환자의신경심리검사및양전자단층촬영소견. 대한신경과학회지, 23(3), 341-347. 김향희 이미숙 김선우 이원용 (2003). 파킨슨병과다계통위축증환자군간의말속도비교평가. 음성과학, 10(4), 221-227. 박기정 윤수진 나덕렬 (2005). 전두측두엽치매의개관. 대한치매학회지, 4, 1-5. 백승렬 이정호 (2007). 단백질응집제어기술 : 퇴행성신경질환과아밀로이드. 생화학분자생물학뉴스, 27(1), 1-7. Ackermann, H., Konczak, J., & Hertrich, I. (1997). The temporal control of repetitive articulatory movements in Parkinson s disease. Brain and Language, 56, 312-319. Ackermann, H., & Ziegler, W. (1991). Articulatory deficits in Parkinson dysarthria: An acoustic analysis. Journal of Neurology, Neurosurgery, and Psychiatry, 54, 1093-1098. Adams, S. G. (1994). Accelerating speech in a case of hypokinetic dysarthria: Descriptions and treatment. In J. S. Till, K. M. Yorkston & D. R. Beukelman (Eds.). Motor speech disorders: Advances in assessment and treatment (pp. 213-228). Baltimore, MD: Brookes. Adams, S. G. (1997). Hypokinetic dysarthira in Parkinson s disease. In M. R. McNeil (Ed.). Clinical management of sensorimotor speech disorders (pp. 261-285). NewYork: Thieme. Bergeron, C., Pollanen, M. S., Weyer, L., Black, S. E., & Lang, A. E. (1996). Unusual clinical presentations of corticobasal ganglionic degeneration. Annals of Neurology, 40(6), 893-900. Bunton, K. (2006). Fundamental frequency as a perceptual cue for vowel identification in speakers with Parkinson s disease. Folia Phoniatrica et Logopaedica, 58, 323-339. Caekebeke, J. F. V., Jennekens-Schinkel, A., van der Linden, M. E., Buruma, O. J. S., & Roos, R. A. (1991). The interpretation of dysprosody in patients with Parkinson s disease. Journal of Neurology, Neurosurgery, and Psychiatry, 54, 145-148. Caliguiri, M. P. (1989). The influence of speaking rate on articulatory hypokinesia in Parkinson dysarthria. Brain and Language, 36, 493-502. Darley, F. L., Aronson, A. E., & Brown, J. R. (1969a). Differential diagnostic patterns of dysarthria. Journal of Speech and Hearing Research, 12, 246-269. 89
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김선우 김향희 / 노인퇴행성신경질환의마비말장애특성 McRae, P. A., Tjaden, K., & Schoonings, B. (2002). Acoustic and perceptual consequences of articulatory rate change in Parkinson disease. Journal of Speech, Language, and Hearing Research, 45, 35-50. Miller, M., Allcock, L., Jones, D., Noble, E., Hildreth, A. J., & Burn, D. (2007). Prevalence and pattern of perceived intelligibility of changes in Parkinson s disease. Journal of Neurology, Neurosurgery, and Psychiatry, 78, 1188-1190. Murdoch, B. E., Manning, C. Y., Theodoros, D. G., & Thompson, E. C. (1997). Laryngeal and phonatory dysfunction in Parkinson s disease. Clinical Linguistics & Phonetics, 11(3), 245-266. Neary, D., Snowden, J. S., Gustafson, L., Passant, U., Stuss, D., Black, S., Freedman, M., Kertesz, A., Robert, P. H., Albert, M., Boone, K., Miller, B. L., Cummings, J., & Benson, D. F. (1998). Frontotemporal lobe degeneration: A consensus on clinical diagnostic criteria. Neurology, 51, 1546-1554. Neuman, M. A., & Cohn, R. (1967). Progressive subcortical gloisis, a rare form of presenile dementia. Brain, 90, 405-418. Ozsancak, C., Auzou, P., & Hannequin, D. (2000). Dysarthria and orofacial apraxia in corticobasal degeneration. Movement Disorders, 15, 905-910. Ozsancak, C., Auzou, P., Jan, M., Derambure, Ph., & Destee, A. (2006). The place of perceptual analysis of dysarthria in the differential diagnosis of corticobasal degeneration and Parkinson s disease. Journal of Neurology, 253, 92-97. Ozsancak, C., Auzou, P., Jan, M., & Hannequin, D. (2001). Measurement of voice onset time in dysarthric patients: Methodological considerations. Folia Phoniatrica et Logopaedica, 53, 48-57. Penner, H., Miller, N., Hertrich, I., Ackermann, H., & Schumm, F. (2001). Dysprosody in Parkinson s disease: An investigation of intonation patterns. Clinical Linguistics and Phonetics, 15(7), 551-566. Sachin, S., Shukla, G., Goyal, V., Singh, S., Aggarwal, V., Gureshkumar, & Behari, M. (2008). Clinical speech impairment in Parkinson s disease, progressive supranuclear palsy, and multiple system atrophy. Neurology India, 56(2), 122-126. Stewart, C., Winfield, L., Hunt, A., Bressman, S. B., Fahn, S., Blitzer, A., & Brin, M. F. (1995). Speech dysfunction in early Parkinson s disease. Movement Disorders, 10, 562-565. Thűmler, B. H., Urban, P. P., Davids, E., Siessmeier, M., Schreckenberger, T., Benz, P., Stoeter, P., Bartenstein, P., & Hopf, H. C. (2003). Dysarthria and pathological laughter/crying as presenting symptoms of corticobasalganglionic degeneration syndrome. Journal of Neurology, 250, 1107-1108. Tjaden, K. (2000a). A preliminary study of factors influencing perception of articulation rate in Parkinson disease. Journal of Speech, Language, and Hearing Research, 43, 997-1010. Tjaden, K. (2000b). An acoustic study of coarticulation in dysarthric speakers with Parkinson disease. Journal of Speech, Language, and Hearing Research, 43, 1466-1480. Tjaden, K. (2003). 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Articulation characteristics of parkinsonian dysarthria: Segmental and phrase-level timing spirantization and glottal-supraglottal coordination. In M. R. McNeil, J. C. Rosenbek & A. E. Aronson (Eds.). The dysarthrias. San Diego, CA: College-Hill Press. Weismer, G., Jeng, J-Y., Laures, J. S., Kent, R. D., & Kent, J. F. (2001). Acoustic and intelligibility characteristics of sentence production in neurogenic speech disorders. Folia Phoniatrica et Logopaedica, 53, 1-18. Wenning, G. K., Litvan, I., Jankovic, J. J., Granata, R., Mangone, C. A., McKee, A., Poewe, W., Jellinger, K., Ray Chaudhuri, K., D Olhaberriague, L., & Pearce, R. K. B. (1998). Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination. Journal of Neurology, Neurosurgery and Psychiatry, 64, 184-189. Yorkston, K. M. (2007). The degenerative dysarthrias: A window into critical clinical and research issues. Folia Phoniatrica et Logopaedica, 59, 107-117. 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Korean Journal of Communication Disorders 2009;14;82-95 ABSTRACT The Senile Neurodegenerative Dysarthrias Sun Woo Kim a HyangHee Kim a,b, a Graduate Program in Speech and Language Pathology, Yonsei University, Seoul, Korea b Department of Rehabilitation Medicine, Yonsei University College of Medicine, Seoul, Korea Correspondence to Prof. HyangHee Kim, PhD, Rehabilitation hospital 3 rd FL. Yonsei University College of Medicine, 250 Sungsanro, Seodaemun-gu, Seoul, Korea e-mail: h.kim@yonsei.ac.kr tel.: + 82 2 2228 3900a Background & Objectives: The diagnosis of neurodegenerative dysarthria is an important step indifferential diagnosis and treatment planning. The purpose of this article was to review research literature on selected senile neurodegenerative dysarthrias, including those associated with Parkinson's disease, corticobasal degeneration, progressive supranuclear palsy, multiple system atrophy, and frontotemporal lobar degeneration. Methods: A review of this research was conducted by searching electronic databases. Results: Literature searches between the years of 1978 and 2008 yielded 58 different studies reporting data related to senile neurodegenerative dysarthrias. Discussion & Conclusion: Although it is evident from the review that studies on this area are sparse, they provide a window into a variety of speech characteristics that may potentially influence the differential diagnosis of senile neurodegenerative disorders. (Korean Journal of Communication Disorders 2009;14;82-95) Key Words: neurodegenerative disorder, dysarthria, Parkinson s Disease, cortirobasal degeneration, progressive supraliuclear palsy, multiple system atrophy, frontotemporal lobar degeneration REFERENCES Ackermann, H., & Ziegler, W. (1991). Articulatory deficits in Parkinson dysarthria: An acoustic analysis. Journal of Neurology, Neurosurgery, and Psychiatry, 54, 1093-1098. Ackermann, H., Konczak, J., & Hertrich, I. (1997). The temporal control of repetitive articulatory movements in Parkinson s disease. Brain and Language, 56, 312-319. Adams, S. G. (1994). Accelerating speech in a case of hypokinetic dysarthria: Descriptions and treatment. In J. S. Till, K. M. Yorkston & D. R. Beukelman (Eds.). Motor speech disorders: Advances in assessment and treatment (pp. 213-228). Baltimore, MD: Brookes. Adams, S. G. (1997). Hypokinetic dysarthira in Parkinson s disease. In M. R. McNeil (Ed.). Clinical management of sensorimotor speech disorders (pp. 261-285). NewYork: Thieme. Association of parents with disabled children in Kyungsangnamdo (2007). The plan of health support program for the children with disability. Unpublished manuscript. Association of parents with disabled children in Kyungsangnamdo (2007). The plan of health support program for the children with disability. Unpublished manuscript. Association of parents with disabled children in Kyungsangnamdo (2007). The plan of health support program for the children with disability. Unpublished manuscript. Bergeron, C., Pollanen, M. S., Weyer, L., Black, S. E., & Lang, A. E. (1996). Unusual clinical presentations of corticobasal ganglionic degeneration. Annals of Neurology, 40(6), 893-900. Bunton, K. (2006). Fundamental frequency as a perceptual cue for vowel identification in speakers with Parkinson s disease. Folia Phoniatrica et Logopaedica, 58, 323-339. Caekebeke, J. F. V., Jennekens-Schinkel, A., van der Linden, M. E., Buruma, O. J. S., & Roos, R. A. (1991). The interpretation of dysprosody in patients with Parkinson s disease. Journal of Neurology, Neurosurgery, and Psychiatry, 54, 145-148. Caliguiri, M. P. (1989). The influence of speaking rate on articulatory hypokinesia in Parkinson dysarthria. Brain and Language, 36, 493-502. Darley, F. L., Aronson, A. E., & Brown, J. R. (1969a). Differential diagnostic patterns of dysarthria. Journal of Speech and Hearing Research, 12, 246-269. Darley, F. L., Aronson, A. E., & Brown, J. R. (1969b). Clusters Received Octorber 22, 2008 Final revision received December 10, 2008 Accepted December 11, 2008. c 2009 The Korean Academy of Speech-Language Pathology and Audiology http://www.kasa1986.or.kr 92
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