6 research outputs found
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Expanding the Phenotype of Frontotemporal Lobar Degeneration With FUS-Positive Pathology (FTLD-FUS).
Atypical frontotemporal lobar degeneration with ubiquitin-positive inclusions (aFTLD-U) is an uncommon cause of frontotemporal dementia characterized by fused in sarcoma-positive inclusions. It is classified as a subtype of frontotemporal lobar degeneration with FUS pathology. Cases with aFTLD-U pathology typically display an early onset of symptoms and severe psychobehavioral changes in the absence of significant aphasia, cognitive-intellectual dysfunction or motor features. This phenotype is regarded as being sufficiently unusual and consistent as to allow antemortem diagnosis with a high degree of accuracy. In this report, we describe 2 cases with aFTLD-U pathology that broaden the associated phenotype to include later age of onset, milder behavioral abnormalities and early memory and language impairment
Recommended from our members
Expanding the Phenotype of Frontotemporal Lobar Degeneration With FUS-Positive Pathology (FTLD-FUS).
Atypical frontotemporal lobar degeneration with ubiquitin-positive inclusions (aFTLD-U) is an uncommon cause of frontotemporal dementia characterized by fused in sarcoma-positive inclusions. It is classified as a subtype of frontotemporal lobar degeneration with FUS pathology. Cases with aFTLD-U pathology typically display an early onset of symptoms and severe psychobehavioral changes in the absence of significant aphasia, cognitive-intellectual dysfunction or motor features. This phenotype is regarded as being sufficiently unusual and consistent as to allow antemortem diagnosis with a high degree of accuracy. In this report, we describe 2 cases with aFTLD-U pathology that broaden the associated phenotype to include later age of onset, milder behavioral abnormalities and early memory and language impairment
Cognitive Impairment and the Dementias
The term mild cognitive impairment (MCI) was introduced as a stage in the Global Deterioration Scale to describe the progression from normal aging to dementia. Alzheimer's disease (AD) is the most common form of dementia in the United States. The potential clinical and demographic risk factors for developing AD at an earlier age of onset identified over the last three decades include depression, female gender, low levels of education, smaller head circumference, and family history of Down's syndrome. Dementia with Lewy bodies (DLB) is a synucleinopathy characterized by a progressive dementia syndrome that is usually associated with parkinsonism and typically dominated by attentional, visuospatial, and executive dysfunction and relatively preserved memory. Vascular cognitive impairment, frontotemporal dementia, primary progressive aphasia, prion diseases, and normal pressure hydrocephalus are also discussed in the chapter
Retinal microvascular abnormalities and cognitive function in latino adults in Los Angeles
10.3109/09286586.2011.615452Ophthalmic Epidemiology193127-136OPEP