NDD-Huntington's Disease

Question 1

What is huntington’s disease?

Answer 1

It is a hereditary neurological disorder that leads to severe physical and mental disabilities. Over time, HD causes progressive loss of nerve cells in the brain, affecting movement, cognition and behavior.

Question 2

What is chorea?

Answer 2

Rapid, involuntary, irregular movements, increasing during stressful situations and lessening or absent during voluntary motor activities and sleep

Question 3

What is motor restlessness?

Answer 3

akathisia

Question 4

What is dystonia?

Answer 4

abnormal, sustained posturing of a body part, typically the arms, head or trunk

Question 5

What involuntary movement patterns occur in individuals with HD?

Answer 5

choreiform movements of hands, chorea, akathisia, and dystonia

Question 6

What is bradykinesia?

Answer 6

slowed movement

Question 7

What is delayed initiation of movement, caused by changes to the functioning of the basal ganglia?

Answer 7

akinesia

Question 8

What is incoordination of movement?

Answer 8

alteration of rhythmical, repetitive movements

Question 9

What voluntary movement pattens occur in individuals with HD?

Answer 9

bradykinesia, akinesia, incoordination of movement

Question 10

During the later stages of HD, what replaces chorea?

Answer 10

hypertonicity

Question 11

What problems arise during the middle stages of HD?

Answer 11

gait and balance problems, such as wide based gait pattern and difficulties walking on uneven terrain

Question 12

During later stages, what happens with movements?

Answer 12

large bursts of movement when small movements intended

Question 13

What motor difficulties arise with clients with HD?

Answer 13

smaller movements and hand eye coordination

Question 14

What cognitive and behavioral abilities deteriorate in clients with HD?

Answer 14

forgetfulness, difficulty concentrating, difficulty with mental calculations, sequencing of tasks and memory, irritability and depression, depression possibly leading to suicide

Question 15

What cognitive and behavioral abilities deteriorate in the initial stages of HD?

Answer 15

difficulty maintaining work performance

Question 16

What cognitive and behavioral abilities deteriorate in the middle stages of HD?

Answer 16

disturbances in memory and decision making

Question 17

What cognitive and behavioral abilities deteriorate in the later stages of HD?

Answer 17

compromised pronunciation because of dysarthria

Question 18

T or F: Some primary signs and symptoms of HD include slowing of saccadic eye movements and ocular pursuits and dysphagia.

Answer 18

True

Question 19

When do symptoms typically appear in clients with HD?

Answer 19

In the third or fourth decade of life

Question 20

How long does it take for symptoms of HD to typically progress?

Answer 20

15 to 20 years, HD is a progressive disorder

Question 21

T or F: With progression, the client eventually requires long-term care or hospitalization.

Answer 21

True

Question 22

T or F: Death results from secondary causes related to the disease

Answer 22

True

Question 23

T or F: There is tx for HD

Answer 23

False, no effective course of treatment has been identified to arrest the progression of HD

Question 24

T or F: Medical management addresses the disease of HD.

Answer 24

False, it addresses the symptoms

Question 25

T or F: Intervention to replace deficient neurotransmitters changes the course or rate of progression

Answer 25

True

Question 26

T or F: A client with HD requires continuous evaluation of symptoms and progression.

Answer 26

True

Question 27

T or F: A client with HD is in control of their movements.

Answer 27

False, initiation of voluntary movements for a task is compromised and performance of voluntary motor tasks is slowed

Question 28

T or F: Sequencing is not a problem for clients with HD.

Answer 28

False, they may have difficulty with sequencing tasks

Question 29

T or F: clients with HD may experience depression

Answer 29

true, due to loss of function

Question 30

T or F: At the later stage of HD, the client can still talk, walk and perform basic ADLs without significant assistance

Answer 30

False, they are unable to do these tasks

Question 31

What are some things that are affected in clients with HD due to the loss of small motor control and hand-eye coordination?

Answer 31

the ability to use tools and utensils, write and use a keyboard

Question 32

What are some things that are affected in clients with HD due to the loss of oral-motor control?

Answer 32

indistinct speech, drooling, choking and difficulty eating

Question 33

T or F: Clients with HD usually gain weight.

Answer 33

False, weight loss can be a consequence of the movement disorder.

Question 34

T or F: During the early stages of HD, it is important to ask open-ended questions.

Answer 34

False, avoid open ended questions during therapy sessions

Question 35

During which stage of HD should you refer a client for a driving evaluation?

Answer 35

early stage, it is important to discuss other forms of community mobility

Question 36

What should an OT do for a client in the early stage of HD to address motor disability?

Answer 36

provide modifications to diminish the effect of chorea and fine motor incoordination on performance of functional activities and introduce a home exercise program to address flexibility and endurance of the entire body.

Question 37

What are some techniques you could use to encourage safety in the kitchen in clients with HD in the early stage?

Answer 37

use unbreakable dinnerware, store commonly used items in accessible cabinets, use a kitchen timer as a reminder to turn off appliances, lower the hot water temperature, use covered mugs for hot liquids, and use oven mitts instead of pot holders.

Question 38

What are some techniques you could use to encourage safety in the bathroom in clients with HD in the early stage?

Answer 38

use nonskid mat in the shower, soap on a rope, shower chair and safety bars

Question 39

What are some techniques you could use to encourage safety in the living room and bedroom in clients with HD in the early stage?

Answer 39

stabilize furniture so it does not move, use chair with high backs and armrests, clear rooms of unnecessary furniture, remove scatter rugs or thick carpet, keep tables and lamps away from walkways, padding doorways and furniture when contact with them is frequent

Question 40

What stage of HD should you encourage the client to arrange for others to handle finances?

Answer 40

middle stages

Question 41

What stage of HD should you encourage the client to use ambulatory devices?

Answer 41

middle stages

Question 42

What are some positioning techniques and adaptive equipment you should educate the client and family on to assist with feeding?

Answer 42

sitting in a sturdy chair as close to the table as possible, wrapping the legs around the chair legs to stabilize the pelvis and placing the elbows on the table to stabilize the upper trunk, using utensils with built-up handles, putting utensils down after each bite to rest muscles and prevent fatigue, using nonskip placemat and using covered cups or mugs.

Question 43

During what stage should you put a ring on zippers for easier fastening?

Answer 43

middle stages

Question 44

During which stage should you use splinting to prevent contractures?

Answer 44

final stages

Question 45

Which stage typically required a smooth transition to tube feedings?

Answer 45

final stages

Question 46

Which stage uses consistent daily schedules and routines?

Answer 46

final stages

Question 47

Which stage should you reduce tasks to simple steps?

Answer 47

middle stages