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Numerous kindreds with familial frontotemporal dementia and/or amyotrophic lateral sclerosis have been linked to chromosome 9, and an expansion of the GGGGCC hexanucleotide repeat in the non-coding region of chromosome 9 open reading frame 72 has recently been identified as the pathogenic mechanism. We describe the key characteristics in the probands and(More)
Multiple studies have explored the relationship between MRI-based volumetric measurements of the hippocampus and amygdala, the degree of volumetric asymmetry of these structures, and symptom manifestation. However, considerable variability exists with regard to the reported volumetric values of these structures. The present study employed meta-analytic(More)
KIBRA single nucleotide polymorphism (SNP) rs17070145 was identified in a genome-wide association study (GWAS) of memory performance, with some but not all follow-up studies confirming association of its T allele with enhanced memory. This allele was associated with reduced Alzheimer's disease (AD) risk in 1 study, which also found overexpression of KIBRA(More)
Covariance structure analyses of a core neuropsychological test battery consisting of the Wechsler Adult Intelligence Scale-Revised, Wechsler Memory Scale-Revised, and Auditory Verbal Learning Test have previously identified a 5-factor model in a sample of cognitively normal White volunteers from Mayo's Older Americans Normative Studies (MOANS). The present(More)
The Dementia Rating Scale (DRS) is a widely used measure of global cognition, with age- and education-corrected norms derived from a cross-sectional sample of adults participating in Mayo's Older Americans Normative Studies (MOANS). In recent years, however, studies have indicated that cross-sectional normative samples of older adults represent an admixture(More)
Acetylcholinesterase inhibitors are commonly used to treat patients with dementia with Lewy bodies. Hippocampal atrophy on magnetic resonance imaging and amyloid-β load on positron emission tomography are associated with the Alzheimer's disease-related pathology in patients with dementia with Lewy bodies. To date, few studies have investigated imaging(More)
A central role for neuropsychological evaluations is the measurement of change in cognitive functioning over time. However, change scores obtained from repeated neuropsychological assessments may be affected by normal variability because of measurement error and practice effects caused by repeated measurements. The current study uses reliable change(More)
Clinical and neuropathological characteristics associated with G4C2 repeat expansions in chromosome 9 open reading frame 72 (C9ORF72), the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia, are highly variable. To gain insight on the molecular basis for the heterogeneity among C9ORF72 mutation carriers, we(More)
Accurate neuropsychological assessment of older individuals from heterogeneous backgrounds is a major challenge. Education, ethnicity, language, and age are associated with scale level differences in test scores, but item level bias might contribute to these differences. We evaluated several strategies for dealing with item and scale level demographic(More)
BACKGROUND Genetic variants at the CLU, CR1, and PICALM loci associate with risk for late-onset Alzheimer's disease (LOAD) in genomewide association studies. In this study, our aim was to determine whether the LOAD risk variants at these three loci influence memory endophenotypes in black and white subjects. METHODS We pursued an association study between(More)