A case of Pick's disease presenting with progressive nonfluent speech

J Korean Neurol Assoc > Volume 15(5); 1997 > Article

Journal of the Korean Neurological Association 1997;15(5): 1162-1172.

진행하는 언어장애로 나타난 Pick 병 1례

안성신, 나덕렬, 정필욱, 이정일, 서연림, 김상은

성균관의대 삼성서울병원 신경과. 신경외과. 진단병리과. 핵의학과, 서울의대 신경과

A case of Pick's disease presenting with progressive nonfluent speech

Sung Shin Ahn, M.D.* , Duk L Na, M.D. , Pil Wook Jung, M.D. , Jung Il Lee, M.D. **, Yeon Lim Suh, M.D. ***, Sang Eun Kim, M.D. ****

Dept of Neurology and Neurosurgery**, Pathology*** and Nuclear Medicine****,Sangsung Medical Center, College of Medicine, Sung Kyun Kwan University, Dept of Neurology, College of Medicine, Seoul National University

Abstract

Background and Significance : Frontotemporal dementia is a behavioral disorder arising from nonAlzheimer's disease atrophy of frontal and anterior temporal lobe. Clinical manifestations include frontal lobe dysfunction. Kluver-Bucy syndrome or progressive language impairments. Two types of histological change underline, the atrophy. The commoner pathology is nerve cell loss and spongiform change with astrocytic gliosis. The second one is typical Pick-type histology characterized by intraneuronal inclusion body and astrocytic gliosis. We report a case with biopsy proved Pick's disease presenting with progressive nonfluent speech. Case : A 41-years, old right-handed woman developed progressive language impairment over a period of 6 months. Brain MRI revealed asymmetric frontotemporal cortical atrophy more severe on the left side and 18F-FDG-brain, PET showed hypometabolism in the same area. Neuropsychological test including Korean version-Western Aphasia Battery revealed non-fluent speech as well as frontal lobe, dysfunction. A biopsy from left frontal lobe, demonstrate neuronal loss and diffuse astrogliosis. In the cytoplasm of remaining neurons are eosinophilic inclusion bodies which are neurofilament-positive with immunostaining. Senile plaque, neurofibrillary tangle and cortical Lewy body were absent. Comment : We report a case presenting with progressive nonfluent speech whose imaging and pathological findings are compatible with Pick's disease, which may be the first biopsy proven case in Korea