Objectives To determine whether quality of life (QOL) ratings are reduced in mild cognitive impairment (MCI) and analyze correlations between QOL ratings and cognitive, neuropsychiatric and functional indices in MCI. Design Cross-sectional. Setting Easton Center for Alzheimer’s Disease Research at UCLA. Participants 205 individuals who met criteria for normal cognition (NC; n=97) or MCI (n=108). The MCI group included amnestic (AMN; n=72) and non-amnestic (NON; n=36) MCI. Measurements QOL was assessed using subject and informant ratings on the Quality of Life-Alzheimer’s Disease (QOL-AD) scale. Cognitive performance was assessed with the National Alzheimer’s Disease Coordinating Center Uniform Data Set neuropsychological battery. Neuropsychiatric symptoms were assessed with the Geriatric Depression Scale (GDS) and the Neuropsychiatric Inventory (NPI). Functional abilities were assessed with the Functional Activities Questionnaire (FAQ). Results The NC group had significantly higher QOL-AD scores than the MCI group on both subject and informant assessments. Individual item analyses indicated that the largest group differences were seen on the mood and memory items. Similar QOL-AD scores were seen in the AMN and NON MCI subgroups. Multiple regression analyses within the MCI group indicated that QOL-AD ratings were not correlated with neuropsychological performance. Subject QOL-AD ratings were inversely correlated with GDS scores and informant QOL-AD ratings were inversely correlated with GDS, NPI, and FAQ scores. Conclusions Significant declines in QOL are seen in MCI and are associated with neuropsychiatric symptoms and functional decline. Interventions targeting mood symptoms and/or instrumental activities of daily living may improve QOL in MCI.
Objective: To characterize the presenting symptoms and signs of patients clinically diagnosed with behavioral variant frontotemporal dementia (bvFTD) and who had different neuropathologic findings on autopsy.Methods: This study reviewed all patients entered as clinical bvFTD in the National Alzheimer's Coordinating Center's database and who had both clinical and neuropathologic data from 2005 to 2011. Among the 107 patients identified, 95 had unambiguous pathologic findings, including 74 with frontotemporal lobar degeneration (bvFTD-FTLD) and 21 with Alzheimer disease (bvFTD-AD). The patients with bvFTD-FTLD were further subdivided into t-positive (n 5 23) or t-negative (n 5 51) histopathology subgroups. Presenting clinical signs and symptoms were compared between these neuropathologic groups. Results:The patients with bvFTD-FTLD were significantly more likely than patients with bvFTD-AD to have initially predominant personality changes and poor judgment/decision-making. In contrast, patients with bvFTD-AD were more likely than patients with bvFTD-FTLD to have memory difficulty and delusions/hallucinations and agitation. Within the bvFTD-FTLD group, the t-positive subgroup had more patients with initial behavioral problems and personality change than the t-negative subgroup, who, in turn, had more patients with initial cognitive impairment and speech problems. Conclusion:During life, patients with AD pathology may be misdiagnosed with bvFTD if they have an early age at onset and prominent neuropsychiatric features despite having greater memory difficulties and more intact personality and executive functions than patients with bvFTD-FTLD. Among those with FTLD pathology, patients with t-positive bvFTD were likely to present with behavior/personality changes. These findings offer clues for antemortem recognition of neuropathologic subtypes of bvFTD. Neurology Behavioral variant frontotemporal dementia (bvFTD) is a common dementia among those with an age at onset of 65 years or younger.1 Only patients meeting clinical criteria for Alzheimer disease (AD) are more common than bvFTD among those with early-onset neurodegenerative dementias. bvFTD is associated with disinhibition, apathy, loss of empathy, compulsive or stereotypical behavior, dietary changes, and a dysexecutive neuropsychological profile. 2,3 There is no definitive antemortem test for bvFTD, and diagnosis depends on clinical criteria which have been validated by clinicopathologic correlation.2,4,5 Clinical criteria, however, are not infallible, and some patients diagnosed with bvFTD can still have AD or another disorder on autopsy. 6 This investigation examined whether different underlying neuropathologic findings in clinically diagnosed bvFTD were associated with different presenting signs and symptoms. We analyzed From the
Ganser syndrome or ''syndrome of approximates'' is characterized by the patient giving incorrect answers to questions, despite showing an understanding of the questions, clouding of consciousness, perceptual abnormalities and symptoms of somatic conversion.
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