Neill R Graff-Radford8
John A Lucas8
8Neill R Graff-Radford
8John A Lucas
7Glenn E Smith
7Robert J Ivnik
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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)
In humans, the neural circuitry underlying facial expressions differs, depending on whether facial expressions are spontaneously (i.e., limbic, subcortical) or voluntarily initiated (i.e., frontal cortex). Previous investigators have suggested that the "masked face" of Parkinson's disease involves spontaneous, but not intentional, facial expressions. In(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)
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)
Serial assessments are commonplace in neuropsychological practice and used to document cognitive trajectory for many clinical conditions. However, true change scores may be distorted by measurement error, repeated exposure to the assessment instrument, or person variables. The present study provides reliable change indices (RCI) for the Boston Naming Test,(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)
OBJECTIVE Confrontation naming is commonly assessed in neuropsychological practice, but few standardized measures of naming exist and those that do are susceptible to the effects of education and culture. The Neuropsychological Assessment Battery (NAB) Naming Test is a 31-item measure used to assess confrontation naming. Despite adequate psychometric(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)