Two Cases of Primary Progressive Aphasia

Reu, Jong Hyun; Hwang, Seung Ryong; Jung, Won Young

Abstract

Primary progressive aphasia (PPA) is an uncommon neurodegenerative syndrome characterized by a progressive deterioration of language, while nonverbal cognitive and other neurological functions of PPA are relatively preserved for a longer period. However, it still remains unclear whether PPA represents a distinct diagnostic entity or a precursor of global dementia syndrome. We report PPA cases that presented with a slowly progressive language dysfunction without disturbing other daily living activities for several years. Repeated neuropsychological tests revealed progres-sive deterioration of executive aspects of language and mild memory dysfunction, although their receptive language and nonverbal cognitive functions were relatively preserved. The imaging of the brain showed prominent atrophic changes in the left perisylvian and the adjacent temporal region. In considering the mild cognitive decline accompanied by language deterioration, we conclude that in these cases it is clinically heterogenous and may be parts of a spectrum of focal forms of non-Alzeimer type dementia.