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FTLD-tau is characterised by tau positive inclusion bodies often referred to as Pick-bodies. [4] Examples of FTLD-tau include; Pick's disease, corticobasal degeneration, progressive supranuclear palsy. FTLD-TDP (or FTLD-U ) is characterised by ubiquitin and TDP-43 positive, tau negative, FUS negative inclusion bodies. The pathological histology ...
There are three main histological subtypes found at post-mortem: FTLD-tau, FTLD-TDP, and FTLD-FUS. In rare cases, patients with clinical FTD were found to have changes consistent with Alzheimer's disease on autopsy. [41] The most severe brain atrophy appears to be associated with behavioral variant FTD, and corticobasal degeneration. [42]
Alternatively, diseases exhibiting tau pathologies attributed to different and varied underlying causes are termed 'secondary tauopathies'. Some neuropathologic phenotypes involving tau protein are Alzheimer's disease , frontotemporal dementia , progressive supranuclear palsy , and corticobasal degeneration .
Frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) is an autosomal dominant neurodegenerative tauopathy and Parkinson plus syndrome. [3] FTDP-17 is caused by mutations in the MAPT (microtubule associated protein tau) gene located on the q arm of chromosome 17, and has three cardinal features: behavioral and personality changes, cognitive impairment, and motor symptoms.
However, when tau is hyperphosphorylated, it is unable to bind and the microtubules become unstable and begin disintegrating. The unbound tau clumps together in formations called neurofibrillary tangles. [4] More explicitly, intracellular lesions known as pretangles develop when tau is phosphorylated excessively and on improper amino acid residues.
The majority of SD patients have ubiquitin-positive, TDP-43 positive, tau-negative inclusions, although other pathologies have been described more infrequently, namely tau-positive Pick's disease and Alzheimer's pathology. [14] Of all the FTLD syndromes, SD is least likely to run in families and is usually sporadic. [15]
In some cases, dry macular degeneration can convert to “wet” degeneration, caused by “bleeding or a leaky blood vessel,” that leads to a much more rapid loss of visual acuity, explains ...
The symptoms of this dementia depend on where in the brain the strokes occurred and whether the blood vessels affected were large or small. [13] Repeated injury can cause progressive dementia over time, while a single injury located in an area critical for cognition such as the hippocampus, or thalamus, can lead to sudden cognitive decline. [75]
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