A Case of Frontotemporal Dementia with Motor Neuron Disease

J Korean Neurol Assoc > Volume 16(4); 1998 > Article

Journal of the Korean Neurological Association 1998;16(4): 557-562.

운동신경원성 질환을 동반한 전측두엽 치매 1례

최성혜, 나덕열, 김병준, 이정일, 서연림, 김상은

성균관대학교 의과대학 삼성서울병원 신경과. 신경외과*. 진단병리과**. 핵의학과***

A Case of Frontotemporal Dementia with Motor Neuron Disease

Seong-Hye Choi, M.D., Duk L Na, M.D., Byoung-Joon Kim, M.D., Jung-Il Lee, M.D.*, Yeon-Lim Suh, M.D.**., Sang Eun Kim, M.D.***

Department of Neurology, Neurosurgery*, Pathology** and Nuclear Medicine*** , Samsung Medical Center, College of Medicine, Sung Kyun Kwan University

Abstract

Dementia with motor neuron disease is a rare subtype of frontotemporal dementia. A 59-year-old woman presented with a twenty month history of abnormal behaviors and progressive cognitive decline. Abnormal behaviors consisted of emotional unconcern, persecutory delusion, distractibility, impulsivity, wondering and excessive laughing. Neuropsychological tests showed profound frontal lobe dysfunction out of proportion to amnesia, aphasia, and visuospatial dysfunction. Brain MRI showed subtle atrophy of both frontal lobes. However, brain FDG-PET showed glucose hypometabolism of bilateral frontal and anterior temporal lobes. An open brain biopsy of right frontal lobe demonstrated nonspecific findings including neuronal loss and microvacuolation with reactive astrogliosis in the absence of Alzheimer changes or Pick body. Four months later she developed amyotrophic lateral sclerosis starting with dysphagia. Her condition had continued to deteriorate, resulting in akinetic mutism and marked limb atrophy by the time she died of pneumonia three years postonset. Key words: frontotemporal dementia, motor neuron disease, PET