Motor Function and Treatment of Huntington's

Question 1

What parts of the basal ganglia make up the stratum?

Answer 1

caudate putamen and nucleus accumbens

Question 2

What are the three things the basal ganglia really controls or plays a role in and how does this related to symptoms of HD?

Answer 2

motor, aspects of cognition, affective functions

these are the three arms fo the symptoms in HD

Question 3

In HD, the direct pathway is _____.

Answer 3

hyperactive

Question 4

What neurons are lost first in Huntington’s disease?

Answer 4

the nigrostriatal GABA projection neurons of the indirect pathway, so you have decreased GPi inhibition of the thalamus, thus an increase in motor activity

Question 5

What are the symptoms of HD?

Answer 5

motor: chorea, motor impersistence, dysphagia, dysarthria, vocalizations
psychiatric: depression, anxiety, aggression, irritability, sleep disurbances, suicidality, mania
cognitive: loss of executive function, ultimately dementia

Question 6

What is the general method of treating HD pharmacologicaly?

Answer 6

dopamine blockers or dopamine depleters

Question 7

What are the typical and atypical dopamine blockers used to treat HD?

Answer 7

typical antipsychotic: haloperidol (D2 antagonist)

atpical antipsychotics: olazapine, risperdone, clozaril (D4 antagonist for psych symptoms only)

Question 8

What is the big side effect risk of haloperidol?

Answer 8

tardive dyskinesia, which wont resolve after you stop the drug

Question 9

What is the main dopamine depleter drug and how does it work?

Answer 9

tetrabenazine - its a monoamine depleting agent that inhibits their transport into presynaptic vesicles

Question 10

What are some drugs to treat the psychiatric symptoms of HD?

Answer 10

depakote to enhance GABA effects

Lithium to influence reuptake of 5HT, NE and affect D2 receptors

SSRIs

Benzodiazepines

Question 11

How is the supportive therapy for HD managed?

Answer 11

typically through an HD specialty clinic