PT  - JOURNAL ARTICLE
AU  - Andrew Kertesz
AU  - Lawrence Hudson
AU  - Ian R.A. Mackenzie
AU  - David G. Munoz
TI  - The pathology and nosology of primary progressive aphasia
AID  - 10.1212/WNL.44.11.2065
DP  - 1994 Nov 01
TA  - Neurology
PG  - 2065--2065
VI  - 44
IP  - 11
4099  - http://n.neurology.org/content/44/11/2065.short
4100  - http://n.neurology.org/content/44/11/2065.full
SO  - Neurology1994 Nov 01; 44
AB  - We present three cases of primary progressive aphasia (PPA) with Pick-variant pathology to support a hypothesis of an underlying nosologic relatedness. Neuropathologic examination demonstrated focal brain atrophy with corresponding neuronal loss and gliosis, accompanied by superficial spongiosis. Specific histologic findings were ballooned neurons (Pick cells) in the atrophic areas, and in two of the cases, Pick bodies. They were immunoreactive for tau. In contrast to classic Pick's disease, there were no Pick bodies in the hippocampus. The intense neurofilament immunoreactivity of the perikarya of the ballooned neurons greatly facilitated their recognition. Based on our cases and a critical review of the literature, we hypothesize that the common underlying pathology of PPA is a variant of Pick's disease. Furthermore, we propose the concept of “Pick complex” to include other neurodegenerative diseases characterized by focal cortical degeneration, such as PPA, frontal lobe dementia, ALS with PPA, and corticonigral and corticobasal ganglionic degenerations.