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Mutations in the Tau gene (known as MAPT or Microtubule Associated Protein Tau) can cause a FTLD presenting with tau pathology (FTLD-tau). [11] There are over 40 known mutations at present. Mutations in the progranulin gene (PGRN) can cause a FTLD presenting with TDP-43 pathology (FTLD-TDP43). Patients with progranulin mutations have type 3 ...
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. [1]
In other words, the symptoms of LATE are similar to those of Alzheimer's disease. The acronym LATE stands for L imbic-predominant A ge-related T DP-43 E ncephalopathy. “ Limbic ” is related to the brain areas first involved, “age-related” and the name “LATE” itself refer to the onset of disease usually in persons aged 80 or older.
The normal life expectancy for 60 to 70 years old is 23 to 15 years; for 90 years old it is 4.5 years. [227] Following AD diagnosis it ranges from 7 to 10 years for those in their 60s and early 70s (a loss of 13 to 8 years), to only about 3 years or less (a loss of 1.5 years) for those in their 90s.
[12] [17] [18] Detachment of tau from microtubules causes the neuron to lose its ability to sustain itself and thus ultimately it loses function. Hyper-phosphorylation of tau protein was initially thought to be caused by Aβ 42 but since PART cases generally lack senile plaques, other causes were investigated.
In 2012, the Oscar winner shared that she’d been diagnosed with the eye condition macular degeneration, which is the most common cause of vision loss in people 50 and older, according to Johns ...
Other degenerative pathologies that can cause corticobasal syndrome include: Alzheimer's disease; Pick's disease with Pick bodies; Lewy body dementias; Neurofilament inclusion body disease; Creutzfeldt–Jakob disease; Frontotemporal degeneration due to progranulin gene mutation; Motor neuron disease‐inclusion dementia. [9]
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