Huntington's

Question 1

What is Huntington’s

Answer 1

Dopamine increase with >35 CAG repeats in Huntington protein due to lack of GABA and excess nigrostatial pathway activity

Question 2

What are the clinical features of Huntington’s

Answer 2

Dominant inheritance
Chorea/hyperkinesia
Dementia/ Depression
Dystonia

Question 3

What is Hyperkinesia

Answer 3

Chorea, Dystonia and unco-ordination

Question 4

What is choreoathetosis

Answer 4

Involuntary movements (jerking)

Question 5

What are the investigations for Huntington’s

Answer 5

Family history
MRI/CT
-Loss of trial volume
-Increased size of frontal horns of lateral ventricles
Genetic tests

Question 6

How can you manage Huntington

Answer 6

Chorea? Benzodiazepine
Depression? SSRI
Psychosis? Haloperidol

Question 7

What is the prognosis of Huntington

Answer 7

Poor

2nd most cause of suicide

Question 8

What is anticipation in terms of Huntington’s

Answer 8

More CAG repeats at younger age = more severe

Question 9

Why is benzodiazepine given for huntngtons

Answer 9

Chorea

Question 10

What can be given for depression in Huntingtons

Answer 10

SSRI

Question 11

What is an anti psychotic given in Huntington

Answer 11

Haloperidol

Question 12

What pathway has increased potential in Huntington’s

Answer 12

Nigrostriatal pathway

Question 13

How does the nigrostriatal pathways differ between Huntington’s and Parkinson’s

Answer 13

Parkinson = Low NSP with low Dopamine
Huntington = High NSP with Low GABA and High CAG