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Terms in this set (14)
What are Frontotemporal dementias or Frontotemporal Lobar Dementias characterized by?
class of diseases characterized by neurodegeneration in the frontal and temporal lobes
What are the three major symptomatic areas?
o Progressive behavior/personality decline characterized by changes in personality, behavior, emotions and judgment
o Progressive language decline characterized by changes in speaking, understanding, reading, and writing or difficulties with pairing words with their meanings
o Progressive motor decline characterized by shaking, difficulty walking, frequent falls, poor coordination and other difficulties with physical movement
T/F: Symptoms fall in the same order with every person, and there is no overlap with other disorders.
• Symptoms and the order in which they appear can vary wildly from one person to the next
• Symptoms overlap in different disorders
What determines the order of symptoms?
• Which lobe, and which part of the lobe, is affected first determines which symptoms appear first
60% of all inherited cases are linked to three genes. What are these genes?
o Microtubule-associated protein tau (MAPT)
o Chromosome 9 open reading frame 72 (C9ORF72)
o Progranulin (GRN)
There are three major proteins that abnormally aggregate into pathology in FTD including:
Tau pathology is most commonly associated with which three FTDs?
Pick's disease is a disease characterized by early changes in personality and has symptoms such as behavior changes, speech difficulty and problems thinking.
Progressive supranuclear palsy is a brain disorder that affects movement, control of walking (gait) and balance, speech, swallowing, vision, mood and behavior, and thinking. One of the classic signs of the disease is an inability to aim and move the eyes properly, which individuals may experience as blurring of vision.
Corticobasal degeneration is characterized by the initial symptoms including stiffness; shaky, slow or clumsy movements; and difficulty with speech and comprehension.
T/F: Tau pathology in FTD is different from that found in Alzheimer's disease and varies between different types of FTD
o Although the tau is abnormally aggregated and hyperphosphorylated as is seen in AD, the pathology can take many shapes and non-neuronal cells such as astrocytes and microglia may also have pathology
What is an example of toxic GOF in FTD?
Inhibiting the proteasome or the autophagy/lysosome system, abnormal gene expression
What is an example of toxic LOF in FTD?
Tau aggregation: decrease in microtubule stability
TDP43 (Tar DNA-binding protein) aggregation: alterations in RNA splicing, stability or trafficking
Is gene fused in sarcoma (FUS) more common in ALS or FTLD?
How is FUS similar to TDP-43?
They are both DNA binding proteins
What is the most vital characteristic needed to diagnose FTD?
o The symptoms present and more importantly, the order in which symptoms appeared
T/F: The symptoms in FTD are determined more by the region of the brain affected rather than by the type of pathology
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