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Behavioural Neurology
Volume 26, Issue 1-2, Pages 95-106
http://dx.doi.org/10.3233/BEN-2012-120255
Clinical Note

Nonfluent/Agrammatic PPA with In-Vivo Cortical Amyloidosis and Pick’s Disease Pathology

Francesca Caso,1,2 Benno Gesierich,1 Maya Henry,1 Manu Sidhu,1 Amanda LaMarre,1 Miranda Babiak,1 Bruce L. Miller,1 Gil D. Rabinovici,1 Eric J. Huang,1 Giuseppe Magnani,1 Massimo Filippi,2 Giancarlo Comi,2 William W. Seeley,1 and Maria Luisa Gorno-Tempini1

1Memory and Aging Center, University of California, San Francisco, CA, USA
2Department of Neurology, Scientific Institute and University Hospital San Raffaele, Milan, Italy

Received 23 April 2012; Accepted 23 April 2012

Copyright © 2013 Hindawi Publishing Corporation and the authors. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

The role of biomarkers in predicting pathological findings in the frontotemporal dementia (FTD) clinical spectrum disorders is still being explored. We present comprehensive, prospective longitudinal data for a 66 year old, right-handed female who met current criteria for the nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA). She first presented with a 3-year history of progressive speech and language impairment mainly characterized by severe apraxia of speech. Neuropsychological and general motor functions remained relatively spared throughout the clinical course. Voxel-based morphometry (VBM) showed selective cortical atrophy of the left posterior inferior frontal gyrus (IFG) and underlying insula that worsened over time, extending along the left premotor strip. Five years after her first evaluation, she developed mild memory impairment and underwent PET-FDG and PiB scans that showed left frontal hypometabolism and cortical amyloidosis. Three years later (11 years from first symptom), post-mortem histopathological evaluation revealed Pick's disease, with severe degeneration of left IFG, mid-insula, and precentral gyrus. Alzheimer’s disease (AD) (CERAD frequent/Braak Stage V) was also detected. This patient demonstrates that biomarkers indicating brain amyloidosis should not be considered conclusive evidence that AD pathology accounts for a typical FTD clinical/anatomical syndrome.