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17 Cards in this Set

  • Front
  • Back
______________________ is a hereditary progressive neurodegenerative disorder of the basal ganglia.
-HUNTINGTONS DISEASE DUH
When does HD typically occur?
midlife, 30-50
HD occurs as a result of mutation to chromosome ____?
-4
The mutation to chromosome 4 results in what?
Excessive multiplication of a nucleotide triplet (36-125x)
an individual with > ____ CAG repeats WILL have huntingtons?
-40
T or F:
There is a direct correlation between a higher number of CAG repeats and early onset of symptoms?
-TRUE
T or F:
each child of a parent with HD will develop the disease?
-FALSE

-50-50 chance
-FALSE

-50-50 chance
In early HD, is there an increase or decrease in unwanted movements?
INCREASE:

the D2 (indirect) pathway is stopped.  This causes decreased inhibition, thus allowing more unwanted movements to occur.
INCREASE:

the D2 (indirect) pathway is stopped. This causes decreased inhibition, thus allowing more unwanted movements to occur.
As the disease progresses, what happens to the striatum?
-In later stages of HD, all striatal projection neurons degenerate, as well as cortical neurons, resulting in a relatively hypokinetic state.
-In later stages of HD, all striatal projection neurons degenerate, as well as cortical neurons, resulting in a relatively hypokinetic state.
___________: activation of a programmed cell death pathway.
-Apoptosis
Why do some individuals choose to not receive the Gene test?
This allows them to know whats to come, and as of now there is no way to change the outcome
T or F:
A stage 1 TFC is worse off then a stage 5 TFC??
-FALSE
-FALSE
Describe 2 signs of premanifested HD.
-mild gait changes
-Irritability
What are two signs a patient with HD has moved into late stage?
-Global dementia
-Delirium
T or F:
Voluntary movement continues to worsen throughout the lifespan of a patient with HD?
-TRUE
What is the typical survival rate of HD?
-15-25 years

Pneumonia and cardiovascular disease are the most common primary causes of death.
What is the Mechanism of action for HD medications?
-OPPOSITE OF PARKINSONS

-BLOCK DOPAMINE