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Primary progressive aphasia: A review of the neourobiology of a common presentation of Pick complex

Department of Clinical Neurological Sciences, St. Joseph's Health Care, London, Ontario; University of Western Ontario, London, Ontario, Canada

David G. Munoz, MD, FRCPC

Department of Pathology and Clinical Neurological Sciences, University of Western Ontario, London, Ontario, Canada; Servicio de Neurologia Hospital Universitario "Doce de Octubre," Madrid, Spain

Primary progressive aphasia (PPA) is an identifiable and distinct form of clinical presentation of focal degenerative disease. The underlying pathology is often considered heterogeneous, but we propose that, in fact, this syndrome has a characteristic underlying neurobiology related to Pick's disease and frontotemporal dementia. The authors reviewed the literature of PPA and found 58 cases with autopsy. The majority of the cases had related underlying pathology, termed "Pick complex," a form of non-Alzheimer's dementia. This includes Pick's disease, corticobasal degeneration, dementia lacking distinctive histology, and motor neuron disease inclusions. Several of the cases described with focal Alzheimer's pathology do not fulfill the criteria of PPA, but have Alzheimer's disease with a major aphasic component.

Key Words: Alzheimer's disease(AD) • aphasia • frontotemporal dementia(FTD) • Pick complex • Pick's disease(PiD) • primary progressive aphasia(PPA)

American Journal of Alzheimer's Disease and Other Dementias®, Vol. 17, No. 1, 30-36 (2002)
DOI: 10.1177/153331750201700105


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