Huntington's disease

Question 1

what causes huntingtons disease

Answer 1

caused by the loss of inhibitory neurons in the striatum
progressive, incurable and terminal neurodegenerative disorder that affects mvmt, intellect, and emotions

Question 2

what is the progression of huntingtons

Answer 2

when inhibitory neurons in striatum die off, they no longer inhibit the GPe
GPe activity increases
leads to inhibition of STN and GPi

Question 3

what are the results of huntingtons disease

Answer 3

decrease in GPi activity which usually inhibits thalamic nuclei
increased activity in cortical motor areas, leading to undesired movements

Question 4

are there hyper or hypokinetic symptoms with huntingtons

Answer 4

hyperkinetic

Question 5

what is the prevalance of huntingtons

Answer 5

1 in 10 000 people in canada
autosomal dominant inherited condition (children have a 50% chance if a parent has it)
onset usually around 30-50 yo

Question 6

what are the motor symptoms from huntingtons

Answer 6

chorea
balance deficits
restlessness
abnormal postures
hemiballism

Question 7

what causes the motor symptoms in huntingtons

Answer 7

caused by reduced inhibition of the indirect pathway leading to involuntary mvmts

Question 8

what is chorea

Answer 8

most common symptom of huntingtons
- development of jerky, uncontrolled mvmts of limbs, trunk, and face

Question 9

what is hemiballism

Answer 9

lesions in the STN cause violent and uncontrolled movements