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Executive functioning is widely targeted when human cognition is assessed, but there is little consensus on how it should be operationalized and measured. Recognizing the difficulties associated with establishing standard operational definitions of executive functioning, the National Institute of Neurological Disorders and Stroke entered into a contract with the University of California-San Francisco to develop psychometrically robust executive measurement tools that would be accepted by the neurology clinical trials and clinical research communities. This effort, entitled Executive Abilities: Measures and Instruments for Neurobehavioral Evaluation and Research (EXAMINER), resulted in a series of tasks targeting working memory, inhibition, set shifting, fluency, insight, planning, social cognition and behavior. We describe battery conceptualization and development, data collection, scale construction based on item response theory, and lay the foundation for studying the battery's utility and validity for specific assessment and research goals. (JINS, 2013, 19, 1–9)
On tests of design fluency, an examinee draws as many different designs as possible in a specified time limit while avoiding repetition. The neuroanatomical substrates and diagnostic group differences of design fluency repetition errors and total correct scores were examined in 110 individuals diagnosed with dementia, 53 with mild cognitive impairment (MCI), and 37 neurologically healthy controls. The errors correlated significantly with volumes in the right and left orbitofrontal cortex (OFC), the right and left superior frontal gyrus, the right inferior frontal gyrus, and the right striatum, but did not correlate with volumes in any parietal or temporal lobe regions. Regression analyses indicated that the lateral OFC may be particularly crucial for preventing these errors, even after excluding patients with behavioral variant frontotemporal dementia (bvFTD) from the analysis. Total correct correlated more diffusely with volumes in the right and left frontal and parietal cortex, the right temporal cortex, and the right striatum and thalamus. Patients diagnosed with bvFTD made significantly more repetition errors than patients diagnosed with MCI, Alzheimer's disease, semantic dementia, progressive supranuclear palsy, or corticobasal syndrome. In contrast, total correct design scores did not differentiate the dementia patients. These results highlight the frontal-anatomic specificity of design fluency repetitions. In addition, the results indicate that the propensity to make these errors supports the diagnosis of bvFTD. (JINS, 2012, 18, 1–11)
Good cognitive performance requires adherence to rules specific to the task at hand. Patients with neurological disease often make rule violation (RV) errors, but the anatomical basis for RV during cognitive testing remains debated. The present study examined the neuroanatomical correlates of RV errors made on tests of executive functioning in 166 subjects diagnosed with neurodegenerative disease or as neurologically healthy. Specifically, RV errors were voxel-wisely correlated with gray matter volume derived from high-definition magnetic resonance images using voxel-based morphometry implemented in SPM2. Latent variable analysis showed that RV errors tapped a unitary construct separate from repetition errors. This analysis was used to generate factor scores to represent what is common among RV errors across tests. The extracted RV factor scores correlated with tissue loss in the lateral middle and inferior frontal gyri and the caudate nucleus bilaterally. When a more stringent control for global cognitive functioning was applied using Mini Mental State Exam scores, only the correlations with the right lateral prefrontal cortex (PFC) remained significant. These data underscore the importance of right lateral PFC in behavioral monitoring and highlight the potential of RV error assessment for identifying patients with damage to this region. (JINS, 2009, 15, 354–364.)
The purpose of this study was to examine the relationships between
lobar volumes and set shifting. We studied 101 subjects, including 36
normal controls, 16 patients with probable Alzheimer's disease, 30
patients with frontotemporal dementia (FTD), and 19 patients with semantic
dementia (SD), using a shifting paradigm that carefully controlled for
component abilities. Subjects were administered two conditions of the
Delis–Kaplan Executive Function System (D-KEFS) Design Fluency Test.
In the control condition (DF:Control), examinees generated as many unique
designs as possible in 60 s by drawing lines connecting only unfilled
dots. In the switching condition (DF:Switch), examinees generated designs
by drawing lines alternating between filled and unfilled dots. We used
BRAINS2 software to generate volumes of the right and left frontal,
temporal, and parietal lobes. Partial correlations and multiple
regressions showed that, after controlling for Mini-Mental State
Examination and DF:Control, only the right and left frontal lobe volumes
significantly correlated with the DF:Switch, most clearly in the FTD and
SD groups. Follow-up analyses indicated that frontal contributions to
shifting were not related to working memory. Results highlight the
importance of carefully controlling for component cognitive processes when
studying executive functioning. (JINS, 2007, 13,
This study tested the hypothesis that the hippocampus has a
relatively specific role in retaining information over delays.
Thirty-seven subjects with probable Alzheimer's disease were
evaluated with a verbal memory task and structural MRI. Cortical gray
matter but not hippocampal volume predicted immediate free recall. In
contrast, hippocampal volume was the best predictor of how well
information was retained over a delay, even after controlling for
levels of immediate recall. Results suggest that the role of the
hippocampus is relatively specific to the consolidation of new
memories. (JINS, 2004, 10, 639–643.)
Bruce L. Miller, Department of Neurology, UCSF School of Medicine, San Francisco, CA, USA,
Howard J. Rosen, Department of Neurology, UCSF School of Medicine, San Francisco, CA, USA,
Michael Greicius, Department of Psychiatry and Behavioral Sciences, Stanford, CA, USA
Historically, classification schemas for degenerative dementias were framed around the clinical and pathological phenomenology of the illness. With improved understanding of the molecular basis for many degenerative conditions, traditional taxonomies are being replaced by molecule-based schemas. Nowhere has this transition been more evident than with frontotemporal dementia (FTD) where, until recently, FTD (or Pick's disease) was used to define a group of patients with selective degeneration of frontotemporal cortex in whom Alzheimer's disease (AD) pathology was absent. With the discovery of tau exon mutations (Poorkaj et al., 1998; Spillantini & Goedert, 1998; Clark et al., 1998) and intron mutations (Hutton et al., 1998) in familial cases with selective frontotemporal degeneration, a clinical/pathological syndrome suddenly had a well-defined molecular and genetic basis. This work clarified the importance of tau protein in the pathogenesis of both sporadic and familial FTD and many hoped that this would lead to a molecule-based diagnostic schema for FTD. However, not all cases with tau pathology have selective frontotemporal anatomic involvement and there are many patients with selective frontotemporal degeneration in whom tau pathology is absent (Kertesz et al., 2000). This has left the field somewhat in limbo with many patients falling in between clinical, pathological or molecule-based diagnostic criteria. Thus, although new insights about abnormalities in tau metabolism are an important piece of the FTD story, it is clear that other factors contribute to producing the clinical syndrome that is recognizable as FTD.
Despite still unresolved issues related to nomenclature, FTD represents an important disorder with distinctive epidemiology, genetics, neuropathology, clinical features and treatment. Importantly, it is possible to diagnose most FTD patients during life (Lopez et al., 1999) and to differentiate them from patients with AD. Because FTD has a distinctivepattern or brain degeneration, it offers many clues to the function of the frontal and anterior cortical regions.
Terminology and epidemiology
In recent years FTD has been recognized as a common cause for degenerative dementia. However, in many clinical settings FTD is rarely, if ever, diagnosed. One problem limiting its recognition is that many patients present with psychiatric symptoms (Gustafson, 1993; Lesser et al., 1989) and do not develop a dementia until much later in the course of their illness. Persistent variability in the diagnostic accuracy and diagnostic suspicion from site to site has compounded the confusion related to the epidemiological features of FTD.
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