Exam 3 Week 12 pp 4 Other disorders

Question 1

What is Huntington’s disease?

Answer 1

-an Inherited neurodegenerative disease. It is an Autosomal dominant mutation in either of an individual’s two copies of a gene called Huntington

Question 2

Huntington’s triad

Answer 2

triad of manifestations

1. Motor changes – choreoathetosis
2. Cognitive decline leading to dementia
3. Psychiatric disorders- including paranoid and psychosis behaviors

Question 3

Age of onset and expected progression

Answer 3

Typical age of onset 40–50

Most people progress to a vegetative state within 10–15 years and die

Question 4

What happens in the brain with Huntington’s

Answer 4

• Gross atrophy of the stiatum (caudate and putamen)
• Overall loss of GABAergic neurons and reduction of inhibition in the basal ganglia circuitry
• Loss of excitation of the subthalamic nucleus of the indirect pathway to the basal ganglia output nuclei

Question 5

Existing medical management for Huntington’s

Answer 5

Dopamine antagonists
Fetal transplantation (unsuccessful)
Deep brain stimulation
Surgical ablation of the GPi
**but relatively little value
Genetic testing, counseling & prevention

Question 6

What causes hemiballismus?

Answer 6

Caused by a discrete lesion of the subthalamic nucleus contralateral to symptoms which reduces activity of the indirect pathway and thus reduces the inhibition of movement.

Question 7

Movement patterns seen with hemiballismus

Answer 7

Wild, unpatterned, flinging movements of an entire extremity

Question 8

What commonly results from vascular disorder of penetrating branch of the posterior cerebral artery?

Answer 8

hemiballismus