| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
From the Memory and Aging Center (R.M., F.A., K.R., A.K., S.B., G.D.R., B.L.M., M.L.G.-T.), Department of Neurology, University of California, San Francisco; Department of Neurological Sciences (R.M.), II University of Naples, Naples, Italy; Neuroimaging Research Unit (F.A.), Institute of Experimental Neurology, Division of Neuroscience, Scientific Institute and University Ospedale San Raffaele, Milan, Italy; and Center for Mind/Brain Sciences (CIMeC) (M.L.G.-T.), University of Trento, Trento, Italy.
Address correspondence and reprint requests to Dr. Maria Luisa Gorno-Tempini, Memory and Aging Center, Department of Neurology, University of California, San Francisco, 350 Parnassus Avenue, Suite 905, San Francisco, CA 94143-1207 marilu{at}memory.ucsf.edu
Objective: Posterior cortical atrophy (PCA) and logopenic progressive aphasia (LPA) are clinical syndromes associated with posterior brain atrophy. We compared PCA and LPA to each other and to an age-matched group of patients with early age at onset of Alzheimer disease (EO-AD). We hypothesized that these 3 syndromes are part of a single clinical and biologic continuum.
Methods: Voxel-based morphometry (VBM) was used to assess atrophy in 14 PCA, 10 LPA, and 16 EO-AD patients compared to 65 healthy controls. Genetic analysis for APOE was conducted in 30 patients and 44 controls. Four patients came to autopsy. An additional 14 were studied with the beta-amyloid specific PET with tracer 11C-labeled Pittsburgh Compound-B (PIB).
Results: VBM results demonstrated that, compared to controls, each patient group showed a large area of overlapping atrophy in bilateral parietal, occipital, precuneus, posterior cingulate, posterior temporal, and hippocampal regions. Surrounding this common area, group-specific atrophy was found in small, symptom-specific regions for each group: the right ventral-occipital and superior parietal regions in PCA, the left middle and superior temporal gyri in LPA, and the prefrontal cortex in EO-AD. APOE 
4 frequency was higher in all patient groups compared to controls. Four PCA, 5 LPA, and 8 EO-AD patients showed evidence of cortical amyloid at pathology (n = 3) or on PIB-PET (n = 14).
Conclusions: Logopenic progressive aphasia and posterior cortical atrophy showed largely overlapping anatomic and biologic features with early age at onset of Alzheimer disease, suggesting that these clinical syndromes represent the spectrum of clinical manifestation of the nontypical form of Alzheimer disease that presents at an early age.
AD = Alzheimer disease; CBD = corticobasal degeneration; EO-AD = early age at onset of Alzheimer disease; LPA = logopenic progressive aphasia; MAC = Memory and Aging Center; PCA = posterior cortical atrophy; PIB = Pittsburgh Compound-B; PPA = primary progressive aphasia; UCSF = University of California San Francisco; VBM = voxel-based morphometry.
Supplemental data at www.neurology.org
Supported by the NIH (NINDS R01 NS050915, NIA P50 AG03006, NIA P01 AG019724); State of California (DHS 04-35516); Alzheimer’s Disease Research Center of California (03-75271 DHS/ADP/ARCC); John Douglas French Alzheimer’s Foundation; Alzheimer’s Association (NIRG-07-59422); Larry L. Hillblom Foundation; Koret Family Foundation; and McBean Family Foundation. PIB-PET scans were supported by NIA AG027859.
Disclosure: Author disclosures are provided at the end of the article.
Received March 3, 2009. Accepted in final form August 7, 2009.
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
