Journal of the International Neuropsychological Society



Primary progressive aphasia: Diagnosis, varieties, evolution


ANDREW  KERTESZ  a1 c1, WILDA  DAVIDSON  a1, PATRICIA  MCCABE  a1, KENJI  TAKAGI  a2 and DAVID  MUNOZ  a3
a1 Department of Clinical Neurological Sciences, St. Joseph's Health Care London, University of Western Ontario, London, Ontario, Canada
a2 Kashima Rosai Hospital, 1-9108-2 Doai-honchou Hasaki-chou, Kashima-gun, Ibaragi, 314-0343 Japan
a3 Department of Pathology and Clinical Neurological Sciences, University of Western Ontario, London, Ontario, Canada and Banco de Tejidos para Investigacion Neurológica Universidad Autonoma, Madrid, Spain

Abstract

A referred cohort of 67 clinically defined PPA patients were compared to 99 AD patients with formal language and nonverbal cognitive tests in a case control design. Language fluency was determined at the first and last follow up visits. Quantitation of sulcal and ventricular atrophy on MRI was carried out in 46 PPA and 53 AD patients. Most PPA patients (57%) are relatively fluent when first examined. Visuospatial and memory functions are initially preserved. Aphemic, stuttering, “pure motor” presentation, or agrammatic aphasia are seen less frequently. Later most PPAs become logopenic and nonfluent, even those with semantic aphasia (dementia). In contrast, AD patients were more fluent and had relatively lower comprehension, but better overall language performance. MRI showed significant left sided atrophy in most PPA patients. Subsequent to PPA, 25 patients developed behavioral manifestations of frontotemporal dementia and 15 the corticobasal degeneration syndrome, indicating the substantial clinical overlap of these conditions. Language testing, particularly fluency scores supported by neuroimaging are helpful differentiating PPA from AD. The fluent–nonfluent dichotomy in PPA is mostly stage related. The aphemic-logopenic-agrammatic and semantic distinction is useful, but the outcomes converge. (JINS, 2003, 9, 710–719.)

(Received November 6 2001)
(Revised July 12 2002)
(Accepted July 16 2002)


Key Words: Primary progressive aphasia; Frontotemporal dementia; Pick complex; Alzheimer's disease.

Correspondence:
c1 Reprint requests to: Dr. Andrew Kertesz, Department of Clinical Neurological Sciences, St. Joseph's Hospital, University of Western Ontario, London, ON N6A 4V2, Canada. E-mail: andrew.kertesz@sjhc.london.on.ca