NDD-Huntington's Disease Flashcards

1
Q

What is huntington’s disease?

A

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.

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2
Q

What is chorea?

A

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

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3
Q

What is motor restlessness?

A

akathisia

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4
Q

What is dystonia?

A

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

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5
Q

What involuntary movement patterns occur in individuals with HD?

A

choreiform movements of hands, chorea, akathisia, and dystonia

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6
Q

What is bradykinesia?

A

slowed movement

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7
Q

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

A

akinesia

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8
Q

What is incoordination of movement?

A

alteration of rhythmical, repetitive movements

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9
Q

What voluntary movement pattens occur in individuals with HD?

A

bradykinesia, akinesia, incoordination of movement

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10
Q

During the later stages of HD, what replaces chorea?

A

hypertonicity

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11
Q

What problems arise during the middle stages of HD?

A

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

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12
Q

During later stages, what happens with movements?

A

large bursts of movement when small movements intended

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13
Q

What motor difficulties arise with clients with HD?

A

smaller movements and hand eye coordination

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14
Q

What cognitive and behavioral abilities deteriorate in clients with HD?

A

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

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15
Q

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

A

difficulty maintaining work performance

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16
Q

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

A

disturbances in memory and decision making

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17
Q

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

A

compromised pronunciation because of dysarthria

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18
Q

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

A

True

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19
Q

When do symptoms typically appear in clients with HD?

A

In the third or fourth decade of life

20
Q

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

A

15 to 20 years, HD is a progressive disorder

21
Q

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

A

True

22
Q

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

A

True

23
Q

T or F: There is tx for HD

A

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

24
Q

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

A

False, it addresses the symptoms

25
Q

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

A

True

26
Q

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

A

True

27
Q

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

A

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

28
Q

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

A

False, they may have difficulty with sequencing tasks

29
Q

T or F: clients with HD may experience depression

A

true, due to loss of function

30
Q

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

A

False, they are unable to do these tasks

31
Q

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

A

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

32
Q

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

A

indistinct speech, drooling, choking and difficulty eating

33
Q

T or F: Clients with HD usually gain weight.

A

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

34
Q

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

A

False, avoid open ended questions during therapy sessions

35
Q

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

A

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

36
Q

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

A

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.

37
Q

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

A

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.

38
Q

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

A

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

39
Q

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

A

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

40
Q

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

A

middle stages

41
Q

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

A

middle stages

42
Q

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

A

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.

43
Q

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

A

middle stages

44
Q

During which stage should you use splinting to prevent contractures?

A

final stages

45
Q

Which stage typically required a smooth transition to tube feedings?

A

final stages

46
Q

Which stage uses consistent daily schedules and routines?

A

final stages

47
Q

Which stage should you reduce tasks to simple steps?

A

middle stages