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Department of Clinical Neurological Sciences, St. Joseph's Hospital (Dr. Kertesz), and the Department of Pathology (Drs. Hudson, Mackenzie, and Munoz), University of Western Ontario, London, ON, Canada.
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.
Address correspondence and reprint requests to Dr. Andrew Kertesz, Department of Clinical Neurological Sciences, St. Joseph's Hospital, 268 Grosvenor Street, London, ON, N6A 4V2 Canada.
Dr. Kertesz was supported by grant OMH512 from the Alzheimer Society and Ontario Mental Health Foundation. Dr. Munoz was supported by Medical Research Council grant MT-10850.
Received March 10, 1994. Accepted in final form May 20, 1994.
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