항상 웃고 다니는 73세 할머니 창원 파티마 병원 신경과 기억 장애/치매 클리닉 권재철, 안선영

73세 여자 환자, 2002년 4월 첫 방문 C.C. : 웃을 일이 아닌데 웃는다. (onset ; 6개월전) Right handedness 학력 : 초졸 딸과 살고 있음. 천주교 신자. FHx : Non-specific PmHx : Hypertension – well controlled

N/Ex : No remarkable finding No parkinsonian feature Lab. finding : no remarkable findings Brain MRI finding : Mild diffuse brain atrophy esp. bilateral frontal area

Neuropsychological Test 2002-4-26 Attention Digit span forward/backward 6/2 Language&related function Fluency Fluent Auditory comprehension Normal Repetition 15/15 K-BNT 37/60 (30 percentile) Reading/Writing Normal/ Normal Calculation Finger naming Right-Left orientation Praxis(Limb/buccofacial) Borderline/Normal Visuospatial Interlocking pentagon Abnormal Copy of Ret-CFT 25/36 (<1 percentile) Memory Seoul Verbal Learning Test free recall(1st, 2nd, 3rd trial) 4+5+5 (total 14) 20-minute delayed recall 4 Recognition(TP-FP) 11-2=9 Recall of Rey-CFT Immediate / Delayed 5/7.5 8-5=3 Frontal/executive Fist-edge-palm Alt. square & triangle Word fluency ‘ㄱ’,ㅇ’,’ㅅ’ 2+7+4 animal, supermarket 11/15 K-MMSE 26/30

Progress Note 2002.08.05 : 식사 잘 함 2002.10.14 : 다리에 힘이 없다 함 2002.12.06 : 며느리가 방문 – 이상행동은 없다 2003.09.08 : 여전히 잘 웃는다. 2004.02.06 : 며느리 상담 행동이 많이 줄었다. 잠을 많이 잔다. 손자들과 리모콘 때문에 싸운다. 하루 종일 TV만 본다.

2004.10.29 : 며느리 videotaping 미사 강론시간에 예의 없는 행동을 한다. 레지오 도중에 집에 간다(참을성 저하). 폭언과 폭력은 없다. 여름에는 속옷만 입고 돌아다닌 적이 있다. 2004.11.4. : 같이 사는 딸과 상담(videotaping) 모든 동작을 하기 전에 말로 표현하거나 물어봄 (verbal tagging) TV 시간표는 신문 없이도 잘 외운다.

2005.01.17 : 언어적 표현력이 빈약해짐 “감기약 도라” “두달분 도라” “열흘분 도라 감기약” “여기다” “밥 묵고 왔다.” “응, 응” “버스타고 왔다. 38번 버스” “이것도 가지고 갈까.” (옷입고 갈까) “이것도 하고 갈까.” (목도리 하고 갈까) “이것도 할까.” (모자 쓸까)

2005년 3월 호기심이 너무 많다. (남의 집을 기웃거려서 112신고로 경찰서에 간적 있다.) K-MMSE : 16/30 (Videotaping)

Video Presentation

Behavior Summary Personality change Language change decreased fluency dysnomia nearly normal comprehension Compulsive behavior No motor abnormalities

Compulsive behaviors as presenting symptoms of frontotemporal dementia Compulsive behaviors as presenting symptoms of frontotemporal dementia. Mendez MF, Perryman KM, Miller BL, Swartz JR, Cummings JL. Frontotemporal dementia (FTD) is a common neurodegenerative dementia syndrome. Compulsive behaviors frequently occur in FTD and may be presenting symptoms of this disorder. This study evaluated compulsive behaviors as presenting symptoms in 29 patients with FTD compared to 48 patients with Alzheimer's disease (AD) enrolled in the UCLA Alzheimer's Disease Center. The FTD patients met the Lund and Manchester criteria for FTD and had predominant frontal hypoperfusion on single-photon emission computer tomography neuroimaging. The AD patients met National Institute of Neurological and Communicative Disorders-Alzheimer's Disease and Related Disorders criteria for clinically probable AD. Compulsive behaviors occurred in 11 FTD patients (38%) versus 5 AD patients (10%) (chi2 = 6.73, P < .01). This difference persisted after controlling for the younger age of the FTD group. There was a range of compulsive behaviors, with the most frequent being repetitive checking activities. Compulsive behaviors are common presenting symptoms among FTD patients and may result from an inability to inhibit urges to perform compulsive movements from damage to frontal-striatal circuits. J Geriatr Psychiatry Neurol. 1997 Oct;10(4):154-7.

Speaking while gesturing - The relationship between speech and limb praxis AM. Barrett et al. Neurology 2002;58(3):499-500. Verbal tagging Clinico-Anatomical correlation