Week 8 Huntington's Disease

Question 1

who is affected by HD

Answer 1

generally aged 30-50 mean age is 40. men and women. Caucasians

Question 2

what is the lifespan with HD

Answer 2

15-25 years.

Question 3

what is the HD etiology

Answer 3

autosomal dominant feature, 50% change to get it from their parents. mostly inherited, if you have the mutation you get the disease

Question 4

What is the genetic mutation with HD

Answer 4

CAG repeat gene, many repeats means an increased repeats

Question 5

what is broken down and lost with ND

Answer 5

basal ganglia

Question 6

what are the 3 things that are part of the presentation of HD

Answer 6

psychiatric, cognitive, sensorimotor deficits.

Question 7

what are the psychiatric issues with HD

Answer 7

depression, irritability, anxiety, apathy, aggressive outbursts, impulsive social withdrawal, and suicidal tendencies. Depressive, rapid decline.

Question 8

TF: depression symptoms are associated with a more rapid decline in function

Answer 8

true

Question 9

what are the cognitive characteristics of HD

Answer 9

slowed thinking impaired ability to manipulate information, poor attention and impaired memory, hard time switching between task, and difficulties IADLs.

Question 10

what are the sensorimotor deficits of HD

Answer 10

chorea (most obvious)
dystonia (disabling: schouder IR, fist clenching, and knee flexion and foot inversion)
involuntary movements 
voluntary movement disorders.
-slow and hypsometric saccadic eyes
-dysphagia and dysarthria
-slow and uncoordinated arms and hands
-abnormal control of posture, balance and gait

Question 11

what is the biggest impairment with HD

Answer 11

motor impairments,

Question 12

who must we coordinate care with for people with HD

Answer 12

OT, speech, home health and psychologist or psychiatrist.

Question 13

what are some drugs we can use to treat HD

Answer 13

• selective serotonin re-uptake inhibitors (SSRIs) (depression and anxiety)
• tetrabenazine: chorea in HD, promotes early degradation of dopamine

Question 14

what are some outcome measures we use for HD (6)

Answer 14

• BERG balance (fall with less than 40-56),
• TUG (more 14 seconds, fall risk),
• activities specific balance confidence scale (less than 50% recurrent falls),
• Tinetti mobility test (less then 21/28, increased risk fall by 1.8).
• unified HD rating scale (motor cognitive and behavioral)
• HD ADL scale (functional things)