Normal and Abnormal function of Basal Ganglia: Huntington's

Question 1

What is Huntington’s Disease

Answer 1

genetic disorder affecting basal ganglia

Question 2

Symptoms of Huntington’s Disease

Answer 2

Dyskinesia: odd involuntary movements

Later motor impairment, depression, dementia, choeric movements

Question 3

What is deficit of Huntington’s

Answer 3

Basal Ganglia dysfunction

Loss of GABA (not dopamine)

Question 4

What does brain look like in Huntington’s

Answer 4

Enlarged lateral ventricles

Massive reduction in caudate nucleus and putamen

Question 5

What is the striatum

Answer 5

caudate nucleus and putamen

Question 6

What neurones degenerate first in Huntington’s and which pathway is this

Answer 6

Enkephalin containing GAbAergic medium spiny neurons

The indirect pathway

Question 7

What neurones degerante next in Huntington’s

Answer 7

Direct pathway-medium spiny GABAergic neurons

Question 8

What does the initial loss of the indirect pathway do

Answer 8

More thalamocortical drive to cortex and uncontrolled excess movement (choreiform movement)

Question 9

What does later degeneration of direct pathway do

Answer 9

Gives rigidity loss of voluntary movement

Question 10

Treatment of Huntington’s?

Answer 10

Some improvement by decreasing dopamine function- Tetrabenazine

Question 11

How to treat Tourette’s

Answer 11

Dopamine receptor antagonists (reduce dopamine function)