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    ABSTRACT: We aimed to determine the extent to which patients with progressive language impairment conform to 2011 primary progressive aphasia (PPA) classification and to examine clinicopathologic correlations within PPA variants. Sixty-two consecutive patients with pathologically confirmed dementia who presented clinically with aphasia were identified. Patients with insufficient clinical information were excluded. PPA classifications were applied to anonymized clinical data taken from patients' initial assessment by raters who were blinded to clinical and pathologic diagnosis. The final cohort comprised 52 patients, 30 of whom met basic PPA criteria. Twenty-five patients met one of the 3 PPA classifications (13 logopenic, 8 nonfluent/agrammatic, and 4 semantic). Five patients did not meet the criteria for any of the PPA variants. All patients who met semantic variant PPA and 75% of patients who met nonfluent/agrammatic variant PPA classifications had frontotemporal lobar degeneration spectrum pathology. Pathologies were heterogeneous in patients who met logopenic variant PPA criteria (46% Alzheimer disease [AD], 8% AD mixed with dementia with Lewy bodies, 23% frontotemporal lobar degeneration, and 23% other). The 2011 PPA recommendations classify a large proportion of patients who meet basic PPA criteria. However, some patients had aphasic syndromes that could not be classified, suggesting that the 2011 recommendations do not cover the full range of PPA variants. Classification of semantic variant PPA provides a good prediction of underlying pathology. Classification of logopenic variant does not successfully differentiate PPA due to AD from PPA due to other pathologies.
    No preview · Article · Oct 2013 · Neurology
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    ABSTRACT: Objective: We aimed to assess sensitivity and specificity of the updated criteria for behavioral variant frontotemporal dementia (bvFTD) based on a large autopsy-confirmed cohort of patients with dementia. Methods: Two hundred thirty-nine consecutive pathologically confirmed dementia patients, clinically assessed in a specialist cognitive unit were identified. Patients with predominant aphasia, motor disorders, or insufficient clinical information were excluded. Frontotemporal Dementia Consensus criteria were applied to anonymized clinical data taken from patients' initial assessment by raters who were blinded to clinical and pathologic diagnosis. Results: The final study cohort comprised 156 patients with predominantly early-onset dementia. The updated criteria for possible bvFTD had a sensitivity of 95% and specificity of 82%. Probable bvFTD criteria had a sensitivity of 85% and specificity of 95%. False positives were predominantly patients with presenile Alzheimer disease. Conclusion: Revised diagnostic criteria show encouragingly high sensitivity and specificity when applied to patients with early-onset dementia. They therefore provide a useful tool both for specialist researchers and general clinicians. There is a need for further prospective studies of sensitivity and specificity involving a broader spectrum of patients with dementia.
    No preview · Article · Apr 2013 · Neurology
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    ABSTRACT: Vascular diseases contribute to the causation and progression of clinical dementia. To evaluate the quality of medical care for vascular diseases provided to people with dementia, the patient and practice characteristics that influence quality, and to compare care with that provided to those without dementia. Observational, cross-sectional review of primary care records of people with dementia from 52 general practices from five primary care trusts in the UK, and comparison with publicly available summary data on patients without dementia. A total of 700 patients with ≥1 diagnosed vascular disease or risk factor were identified from dementia registers. Quality of care was measured on 30 indicators from the UK Quality and Outcomes Framework (QOF) for hypertension, coronary heart disease, stroke, diabetes mellitus, atrial fibrillation, heart failure, and smoking. Overall quality of vascular care was calculated for each patient with dementia. Level of care received by people with dementia was significantly lower compared with those without dementia for 22 of 30 (73%) indicators; most notably for measurement processes such as peripheral pulses check and neuropathy testing for diabetes, and cholesterol measures for stroke. Among people with dementia, women, those in care homes, and those with fewer comorbid physical conditions and medications were associated with lower scores for overall quality of vascular care. The quality of medical care provided to people with dementia with regard to vascular diseases is not concordant with quality, as defined by the QOF. Research is needed to improve access to high-quality care.
    No preview · Article · Feb 2013 · British Journal of General Practice
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