Primary Progressive Amnesia 3 Cases - a Case Study

J Korean Neurol Assoc > Volume 19(6); 2001 > Article

Journal of the Korean Neurological Association 2001;19(6): 592-597.

원발성 진행성 기억상실증 3예-증례 연구-

서상원, 권재철 진주희 강수진 태우석 홍승봉 나덕렬

성균관대학교 의과대학 삼성서울병원 신경과,연세대학교 의과대학 신경과학교실*

Primary Progressive Amnesia 3 Cases - a Case Study

"Sang Won Seo, M.D.*, Jay C. Kwon, M.D., Juhee Chin, M.A., Sue J. Kang, M.S., Woo Suk Tae, Seung Bong Hong, M.D., Duk L. Na, M.D."

"Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine Department of Neurology, College of Medicine, Yonsei University*"

Abstract

Background : Isolated amnesia without dementia results from various etiologies. When caused by degenerative etiol-ogy, it is recognized as a subtype of Alzheimer’s disease (AD) and was termed progressive isolated amnesia or primary progressive amnesia (PPAm). Patients with PPAm have rarely been reported. We describe neuropsychogical and neu-roimaging findings in 3 patients with PPAm. Methods : Patient 1 (M/74) showed progressive amnesia for 8 years and then developed probable AD. Patient 2 (M/76) and 3 (F/69) showed severe progressive amnesia without dementia for 9 years. Neuropsycholgical evaluations were conducted 3 times in each patient at 1 to 4 year interval. Hippocampal vol-ume was measured by a manual tracing in 1.6 mm thick coronal MRI slices which was obtained perpendicular to the long axis of the hippocampus. Results : Neuropsychological tests revealed verbal and nonverbal memory loss with preservation of attention, language, praxis, visuospatial and frontal-executive functions. Right and left hippocampal vol-umes for patient 1, 2 and 3 were 580.46/1586.38, 682.96/609 and 1152.84/1272.50 mm3 respectively, a result indica-tive of severe hippocampal atrophy. Conclusions : Neuropsychological profiles and clinical course of our patients fur-ther support the view that PPAm results from degenerative etiology. Severe atrophy of the hippocampus with relative preservation of other association cortices suggest that PPAm may be another focal cortical atrophy syndrome involving medial temporal region. Key Words : Primary progressive amnesia, Hippocampus, Alzheimer’s disease, Focal cortical atrophy, Isolated amnesia