Nonfluent/agrammatic PPA with in-vivo cortical amyloidosis and Pick's disease pathology.
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.
Memory and Aging Center, University of California, San Francisco, CA 94143-1207, USA. FCaso@memory.ucsf.edu
Francesca
F
Caso
Benno
B
Gesierich
Maya
M
Henry
Manu
M
Sidhu
Amanda
A
LaMarre
Miranda
M
Babiak
Bruce L
BL
Miller
Gil D
GD
Rabinovici
Eric J
EJ
Huang
Giuseppe
G
Magnani
Massimo
M
Filippi
Giancarlo
G
Comi
William W
WW
Seeley
Maria Luisa
ML
Gorno-Tempini
D000368:Aged; D000544:Alzheimer Disease / Q000150:complications / Q000473:pathology; D000686:Amyloidosis / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D000814:Aniline Compounds; D002250:Carbon Radioisotopes; D018450:Disease Progression; D005260:Female; D005462:Fluorine Radioisotopes; D019788:Fluorodeoxyglucose F18; D005625:Frontal Lobe / Q000000981:diagnostic imaging / Q000473:pathology*; D059907:Functional Neuroimaging / Q000379:methods / Q000523:psychology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods / Q000523:psychology; D009483:Neuropsychological Tests / Q000706:statistics & numerical data; D020774:Pick Disease of the Brain / Q000150:complications / Q000473:pathology*; D049268:Positron-Emission Tomography / Q000379:methods / Q000523:psychology; D057178:Primary Progressive Nonfluent Aphasia / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology; D013844:Thiazoles
C475519:2-(4'-(methylamino)phenyl)-6-hydroxybenzothiazole; D000814:Aniline Compounds; D002250:Carbon Radioisotopes; D005462:Fluorine Radioisotopes; D013844:Thiazoles; D019788:Fluorodeoxyglucose F18