Huntington Disease

Question 1

What is the cause of Huntington Disease?

Answer 1

CAG trinucleotide repeats on Chr 4

Question 2

What can be seen on brain scan in Huntington Disease?

Answer 2

Caudate and Putamen atrophy

Question 3

How does Huntington Disease commonly present?

Answer 3

Choreiform movement disorder: Dyskinesia

Dementia and Depression

Behavioral change: Irritability, moodiness, anstisocial behavior

Onset age 30-50 with family history of HD

Question 4

How does the movement disorder of Huntington Disease start?

Answer 4

Fidgetiness or restlessness: progressing to dystonic posturing, rigidity and akathisis

Question 5

What are commonly seen features of Huntington Disease?

Answer 5

Abnormal Eye movements

Loss of voluntary eye saccades and smooth pursuit

Inability to maintain handgrip

Question 6

What is the best test for Huntington Disease?

Answer 6

Genetic Test: 99% sensitive

Question 7

How do you manage Huntington Disease?

Answer 7

Haloperidol, quetiapine for psychosis

VMAT 2 Inhibitors for movement disorder