Huntington's Disease Flashcards
What is Huntington’s Disease?
hereditary disease where degeneration in the cerebral cortex and BG causes chronic progression CHOREA & mental deterioration ending in dimentia
What is Chorea?
abnormal involuntary movement of the hands and feet that look like dancing
How genetically is Huntington’s caused?
autosomal dominant on the 4th chromosome mutation
What are the main characteristics of HD?
movement disorder
behavior change
dementia
Where is HD most common and where is it rare?
rare in Japan
high in places with large families
What is HD age onset?
25-55
When is death in HD? and what is present?
death within 10-15 years with immobility, weight loss, aspiration, pneumonia, CHF or suicide
What 2 NT disturbance is present?
GABA and Dopamine
IN advanced stages of HD what is present?
20-25% brain atrophy
What part of the brain is the most brain atrophy?
BG (caudate nucleus)
and
frontal lobe
How is HD diagnosed?
S&S
DNA test
a common sign of HD is dystonia, what is it?
involuntary muscle contractions that result in slow repetitive movements, cramps, or abnormal posture.
What are some common clinical motor signs in HD?
dystonia
rigidity
bradykinesia
PD-like
What are two major oromotor signs of HD?
hyperkinetic speech
dysphasia
What is a cardinal sign of HD?
intellectual decline “executive funciton”
