Primary Progressive Amnesia 3 Cases: a Case Study

Seo, Sang Won; Kwon, Jay C; Chin, Ju Hee; Kang, Sue J; Tae, Woo Suk; Hong, Seung Bong; Na, Duk L

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 1580.46/1586.38, 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.