Neurodegenerative Conditions

Question 1

_________________ is the impairment of voluntary and spontaneous movement initiation that results in freezing, especially during gait activities.

Answer 1

Akinesia

Question 2

Slowed motor movements are known as…

Answer 2

Bradykinesia

Question 3

Decreased coordination of movements are known as…

Answer 3

Dysmetria

Question 4

Muscle stiffness that impairs movement is called…

Answer 4

Rigidity

Question 5

______________ are marked by involuntary muscle contraction and relaxation and can be observed as a muscle twitch.

Answer 5

Fasciculation

Question 6

Small rapid steps that result from a forward-tilted head and trunk posture are known as…

Answer 6

Festinating gait

Question 7

Numbness and tingling because of sensory nerve changes are called…

Answer 7

Paresthesia

Question 8

Throughout the evaluation process with a client who has neurodegenerative disease, the occupational therapist must pay particular attention to what 2 things?

Answer 8

1. How the client is affected by variable symptoms (e.g., during the day, week, and months/seasons) and the progression of the disease.
2. The client’s understanding of the progressive nature of the disease and their level of acceptance.

Question 9

OT interventions for neurodegenerative diseases help the client ____________________ as function declines.

Answer 9

Compensate and adapt

Interventions include cognitive compensatory strategies, energy conservation, environmental modifications and adaptive equipment, exercise, and caregiver training. See Table 2 at the end of this lesson for more information on intervention strategies.

Question 10

___________ is a progressive, degenerative disease in which the motor neurons in the brain, spinal cord, and peripheral system are destroyed and replaced by scar tissue. The resulting plaques lead to progressive muscle atrophy.

Answer 10

Amyotrophic Lateral Sclerosis (ALS)

Question 11

True or false: Women are affected by ALS more often than men, at a ratio of 1.7 to 1.

Answer 11

FALSE

Men are affected more often than women, at a ratio of 1.7 to 1.

ALS is progressive, moves distal to proximal, and affects multiple parts of the nervous system.

Question 12

What are 2 lower motor neuron symptoms of ALS?

Answer 12

Focal and multifocal weakness
Muscle atrophy (progressing from distal to proximal musculature)
Cramping and fasciculations

Question 13

What are the 2 corticospinal tract symptoms of ALS?

Answer 13

Spasticity
Hyperreactive reflexes

Question 14

What are the 2 corticobulbar symptoms of ALS?

Answer 14

dysphagia (difficulty swallowing)
dysarthria (difficulty speaking)

Question 15

Name at least 2 of the most typical initial symptoms of ALS.

Answer 15

Weakness of the small muscles of the hand

Asymmetrical foot drop with or without night cramps, usually in the calves.

Tripping or stumbling when walking or running, or difficulty fastening clothes.

If the bulbar tract is affected, initial symptoms might include difficulty breathing, slurred speech, decreased volume in speech, or impaired swallowing.

Question 16

What are the 6 stages of ALS?

Answer 16

Stage I: The person can walk, is independent with ADLs, and has some weakness.

Stage II: The person can walk and has moderate weakness.

Stage III: The person can walk but has severe weakness.

Stage IV: The person requires a wheelchair for mobility, needs some assistance with ADLs, and has severe weakness in the legs.

Stage V: The person requires a wheelchair for mobility, is dependent for ADLs, and has severe weakness in the arms and legs.

Stage VI: The person is unable to get out of bed and is dependent for ADLs and most self-care tasks.

Question 17

True or false: The disease course may be longer than 1-5 years for younger people with ALS whose symptoms are milder.

Answer 17

True

Though, mean survival is 3 years.

Question 18

True or false: While there is no cure for ALS, there is a medication to prolong earlier, milder stages for longer.

Answer 18

TRUE

Riluzole may help people remain in the earlier, mild stages longer.

Question 19

What is the first intervention to consider in patient’s with ALS?

Answer 19

Safety, including positioning, transfers, and skin integrity

Question 20

What are 5 types of of intervention for a patient with ALS?

Answer 20

Safety, including positioning, transfers, and skin integrity

Augmentative communication equipment

Assessment and management of dysphagia (manual swallowing techniques, minimize distractions during mealtime, allowing time for meals)

Social participation

Selection of adaptive equipment and technology to support occupational performance

Question 21

This disease occurs from cortical atrophy of the frontal, parietal, and temporal lobes as well as the hippocampal region caused by an accumulation of amyloid-beta senile plaques and tau protein neurofibrillary tangles. Clinically, it presents as a progressive impairment of memory, executive function, attention, language, visual processing, and praxis. Commonly, it also can present with behavioral disturbances.

Answer 21

Alzheimer’s Disease

Question 22

During the _________ stage of Alzheimer’s Disease, Individuals experience frequent forgetfulness; mood changes, especially when socially or mentally challenged; poor decision-making when selecting clothing appropriate for the weather or situation; personality changes; disorientation; and risk of wandering or becoming lost.

Answer 22

Middle stage (moderate)

Question 23

During the _________ stage of Alzheimer’s Disease, Individuals may function largely independently and engage in many occupations of daily life without assistance. Individuals may be aware of having memory lapses or forgetting words.

Answer 23

Early stage (mild)

Question 24

During the _________ stage of Alzheimer’s Disease, Individuals are unable to engage with the environment, cannot carry on conversations, and require significant levels of assistance for daily activities

Answer 24

Late stage (severe)

Question 25

This disease occurs from cerebrovascular disease (often a series of small strokes) that leads to focal lesions on the brain and neurotransmitter disruption. Clinically, it presents as cognitive decline similar to AD but often less severe memory involvement. Commonly, it involves gait disturbance and progresses as either abrupt or stepwise (rather than continuous) decline.

Answer 25

Vascular dementia

Question 26

This disease occurs from neuronal, intranuclear inclusions and presents clinically as progressive aphasia, corticobasal syndrome, or symptoms similar to AD or Parkinson’s disease.

Answer 26

Frontotemporal dementia

Question 27

This disease occurs from Lewy body proteins present in the brain, usually predominant in the limbic or neocortical areas; overall decline in acetylcholine and dopamine levels. Clinically, this presents as progressive deficits in attention and executive function, memory impairment, fluctuating cognition, visual hallucinations, parkinsonism, autonomic dysfunction, and falls; rapid eye movement sleep behavior disorder as a possible prodromal symptom.

Answer 27

Dementia ith Lewy Bodies

Question 28

_____________ is the loss of skilled, purposeful movements; can affect ideation and concept formation as well as programming and planning of movement and is common in dementia.

Answer 28

Apraxia

Question 29

Expressive and/or receptive aphasia is common in what neurodegenerative disease?

Answer 29

Dementia

Question 30

_______ can be experienced as auditory, visual, and tactile, and is common in dementia.

Answer 30

Agnosia

Question 31

Name at least 5 symptoms of dementia.

Answer 31

Memory impairment: deficits in short term, procedural, episodic, and semantic memory.

Cognitive deficits
Expressive and/or receptive aphasia
Apraxia
Agnosia
Disturbance in executive functioning: disruption of the broad band of skills that enable a client to engage in independent, self-directed behavior, including volition, planning, purposeful action, and self-awareness.

Other cognitive or personality symptoms
Difficulty with topographic orientation (i.e., orientation of the self within the environment; can cause the person to get lost easily
Difficulty with spatial tasks
Poor judgment
Anxiety and defensiveness
Disinhibited behavior
Psychotic symptoms

Motor deficits: gait disturbances, hyperreflexia, paratonia, and dysphagia.

Question 32

How does dementia affect occupational performance in the Early Stage?

Answer 32

** first signs of memory loss manifest in IADLs**, including financial management, shopping, meal preparation, driving, reading, health management, and sexual activity; leisure activities; and social participation.

Dementia affects orientation to place, which impairs community mobility.

Work performance is often impaired, and relationships with coworkers and supervisors can be negatively affected.

Communication is impaired because of language problems.

The person clings to old habits and repetitive routines.

Cultural and spiritual context remain intact, but participation declines.

Question 33

How does dementia affect occupational performance in the Middle Stage?

Answer 33

The person can no longer live alone and may no longer attend to ADLs.

IADLs are totally neglected or performed out of sequence. The person may be completely dependent on others for IADLs.

**Eating problems and weight loss **occur.

Simple home management tasks are difficult but can be completed with assistance.

Safety is a major concern; risks include wandering, letting a stranger into the house, or causing a fire.

Leisure and social participation is very limited, and work is likely not possible. General roles continue to be lost.

Orientation is diminished

Question 34

How does dementia affect occupational performance in the Late Stage?

Answer 34

All areas of occupation are lost.

The person is dependent in all ADLs.

The person can no longer ambulate safely.

Communication is lost.

The person has no understanding of cultural, social, or spiritual contexts.

Question 35

___________ are an increase in activity and often agitation that begins in the late afternoon and extends into the evening or night.

Answer 35

Sundowning behaviors

Question 36

What are some approaches to interventions with individuals with dementia?

Answer 36

Behavior management: Communication, sundowning behaviors, anger, wandering, hoarding

Affective functioning: teach coping and relaxation

**Sensory perceptual functioning*

Social functioning: reminiscence therapy

Sleep functioning

Environmental interventions

Question 37

______________ is an inflammatory disease that causes demyelination of axons in peripheral nerves.

Answer 37

Guillain-Barré syndrome (GBS)

Onset is most often in adults ages 20 to 24 and 70 to 74 years, with men at a slightly higher risk than women.

Question 38

At which phase of Guillain-Barré syndrome are symptoms are at their most disabling, with little or no change over a few days or weeks?

Answer 38

Plateau phase

Question 39

Remyelination and axonal regeneration occur over a period as long as 2 years. Recovery tends to start at the head and neck and travel distally.

What disease is this?

Answer 39

Guillain-Barré syndrome

About 80% of clients can walk independently 6 months after diagnosis.

About 60% of clients fully recover motor strength 1 year after diagnosis.

About 5% to 10% of clients have very delayed and incomplete recovery.

Question 40

____________________ is a fatal, 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, emotions, and behavior.

Answer 40

Huntington’s Disease

Question 41

What types of voluntary and involuntary movement patterns are common in Huntington’s Disease?

Answer 41

Involuntary movement patterns: chorea, akathisia, and dystonia

Voluntary movement patterns: bradykinesia, akinesia, incoordination of movement

In the middle stages, gait and balance problems, such as wide-based gait pattern and difficulties walking on uneven terrain

During later stages, hypertonicity replaces chorea, and large bursts of movement when small movements are intended.

Question 42

How does Huntington’s Disease affect communication and feeding?

Answer 42

Dysarthria
Dysphagia

Question 42

Symptoms of this disease usually appear in the third or fourth decade of life and progress over a 15- to 20-year period.

Answer 42

Huntington’s Disease

Question 43

How does Huntington’s Disease affect occupational performance?

Answer 43

Deterioration of muscle strength and motor control make occupational performance more difficult.

Deterioration of cognitive abilities may lead to dismissal from employment.

The client may have difficulty sequencing tasks.

Loss of function may contribute to depression, depression may decrease desire to do activities.

Performance of voluntary motor tasks is slowed.

Initiation of voluntary movements for a task is compromised.

At the later stage of HD, the client is usually unable to talk, walk, or perform basic ADLs without significant assistance.

Loss of fine motor control and hand–eye coordination affect ability to use tools and utensils, to write, and to use a keyboard.

Loss of oral–motor control causes indistinct speech, drooling, choking, and difficulty eating.

Weight loss can be a consequence of the movement disorder.

Question 44

True or false: Treatment approaches should be focused on remediation for clients with Huntington’s Disease.

Answer 44

FALSE

Focus is on compensatory strategies

Question 45

When working with someone with Huntington’s Disease (late/final stages), what are some interventions OT can do?

Answer 45

Pay attention to positioning.

Use splinting to prevent contractures.

Facilitate a smooth transition to tube feedings.

Put environmental controls into place as needed and desired.

Use consistent daily schedules and routines.

Question 46

________________ is a chronic, often progressive, demyelinating disease of the central nervous system; a person’s body attacks the myelin sheath that covers the neurons of the brain and spinal cord.

Answer 46

Multiple sclerosis (MS)

This demyelination produces scar tissue or plaque (sclerosis) on the myelin sheath of nerve fibers that interferes with the axons’ ability to conduct impulses.

The result is impaired nerve conduction and inflammation, which causes neurological dysfunction.

The specific location of demyelination differs with each person and dictates symptoms.

Question 47

True or false: MS can cause dysphagia and partial or complete paralysis of a part of the body.

Answer 47

TRUE

Question 48

People with MS may experience partial loss of vision, called…

Answer 48

Scotoma

Question 49

True or false: MS does not affect the bladder or bowels.

Answer 49

FALSE

They can experience:
1. Incontinence or urinary retention (failure to empty the bladder)

2. Increased urgency or frequency, as well as constipation

Question 50

Which type of MS is described below?

the first episode of MS symptoms. Not everyone at this stage develops MS, but those with brain lesions at this stage have a high likelihood of developing relapsing-remitting MS.

A. Clinically isolated syndrome
B. Relapsing-remitting MS
C. Secondary progressive MS
D. Primary progressive MS

Answer 50

A. Clinically isolated syndrome

Question 51

Which type of MS is described below?

follows the course of relapsing-remitting MS, but in some people neurologic function worsens progressively.

A. Clinically isolated syndrome
B. Relapsing-remitting MS
C. Secondary progressive MS
D. Primary progressive MS

Answer 51

C. Secondary progressive MS

Question 52

Which type of MS is described below?

neurologic function worsens progressively as soon as symptoms appear, with no remissions.

A. Clinically isolated syndrome
B. Relapsing-remitting MS
C. Secondary progressive MS
D. Primary progressive MS

Answer 52

D. Primary progressive MS

Question 53

Which type of MS is described below?

the most common type of MS, characterized by active (often worsening) and nonactive (stable) stages.

A. Clinically isolated syndrome
B. Relapsing-remitting MS
C. Secondary progressive MS
D. Primary progressive MS

Answer 53

B. Relapsing-remitting MS

Question 54

What is one of the top things to consider when scheduling appointments for a client with MS?

Answer 54

Endurance and strength vary throughout the day, depending on participation in occupations and timing of medications; therapy should be scheduled for when the client reports feeling most energized.

Goals should address both the exacerbation and remission stages and should be compensatory because of the progressive nature of MS.

Question 55

Name at least one treatment consideration related to spasticity for clients with MS.

Answer 55

Maintain hips at 90° or more of flexion to reduce extensor tone in the lower extremities.

Apply focal heat modalities on muscle trigger points.

Monitor body mechanics to avoid stressing joints and increasing musculoskeletal pain.

Reduce extended periods of standing or mobility by adapting tasks and using power mobility when appropriate.

Question 56

How might you address ataxia in a client with MS?

Answer 56

Encourage proximal stabilization for improved distal movements.

Modify tasks by promoting hand-over-hand techniques for fine motor tasks (e.g., using the unaffected extremity over the hand with tremor when dialing a phone).

Use orthoses:
- Cervical collar to reduce the travel of the head and neck
- Wrist orthoses to minimize travel and number of joints in motion in the presence of a tremor

Question 57

How might you work with a client with MS who experiences cognitive and behavioral disturbances?

Answer 57

Consider cognitive retraining, memory enhancement programs, cognitive–behavioral therapy, visual compensation strategies, or group therapy.

Advise the client to allow for increased time, delegation, and repetition when learning new ideas and to perform difficult mental tasks earlier in the day.

Teach stress management and relaxation techniques; emotional and physical stress can temporarily worsen symptoms.

Explain coping strategies that can help in handling exacerbations.

Question 58

_____________ is a progressive, variable condition that occurs most frequently in later adulthood, with a mean onset age of 55 to 60 years. Major degenerative changes occur in the basal ganglia, the gray matter that contributes to complex movements. The substantia nigra becomes depigmented, affecting the production of dopamine, a neurotransmitter that influences the speed and accuracy of motor skills, postural stability, cognition, and affect and expression. The loss or reduction of dopamine leads to disabling symptoms.

Answer 58

Parkinson’s Disease

Question 58

What are the 4 primary symptoms of Parkinson’s Disease?

Answer 58

Resting tremor: Often absent with voluntary movement.

Muscle rigidity: Tone is increased, adversely affecting movement. Cogwheel motions: jerky, sometimes painful movements with joint mobility, most commonly in the upper extremities
Fatigue: caused by the increased effort needed to produce voluntary movement; becomes a barrier to occupational performance.

Bradykinesia: Extreme slowness when the person initiates or performs volitional movements. Examples: shuffling gait, difficulty moving from sitting to standing, freezing, and increased time required for fine motor tasks such as shaving or fastening clothes.

Postural instability: Stooped posture, with a lack of arm swing during mobility and loss of postural reflexes. Increased fall risk, especially when balance is challenged.

Question 59

At what stage of Parkinson’s Disease is there Significantly decreased function, impaired mobility, need for assistance with ADLs, poor fine motor skills and dexterity?

Answer 59

Stage 4

Stage 1: Unilateral symptoms; function is maintained
Stage 2: Bilateral symptoms; balance is not affected
Stage 3: Bilateral symptoms, balance is impaired, mild to moderate impairments in function
*Stage 4: Significantly decreased function, impaired mobility, need for assistance with ADLs, poor fine motor skills and dexterity
Stage 5: Total dependence for mobility and ADLs.

Question 60

What are some interventions for safety and functional mobility for a client with Parkinson’s Disease?

Answer 60

Provide instruction to manage freezing, such as avoiding crowds, tight spaces, and turns or corners; reducing distractions and avoiding multitasking; eliminating clutter in pathways; and avoiding rushing to answer the phone or door.

Instruct the client in the use of assistive devices such as a walker or cane. If a wheelchair is required, evaluate for proper positioning and train the client and family in its use, transport, and maintenance.

Use of a single auditory cue can help the person produce quicker and smoother movements. The rhythm of counting out loud and singing also helps clients fine-tune movements.

Question 61

What are some interventions for feedings for a client with Parkinson’s Disease?

Answer 61

Encourage the client to use adaptive equipment such as built-up or weighted utensils, cups with lids, plate guards, and nonslip surfaces.

Educate the client and caregiver to allow increased time for feeding and ADLs; stress from rushing increases symptoms.

Use distal wrist weights if effective in decreasing tremors.

Instruct the client to work on feeding and other activities as close to the body as possible and, when appropriate, with upper-extremity support on a table. Using proximal muscles can help stabilize distal joints and muscles, which may reduce tremors.

Question 62

What are some interventions for communication for a client with Parkinson’s Disease?

Answer 62

Use larger paper and large felt-tip pens; rest before writing

Pay bills and perform other IADL tasks online, including through cell phone apps. Clients with sufficient cash flow can set up autopay options.

Use speed dial or voice-controlled telephones, speech-to-text programs, and voice- and other remote-control systems for electronics.

Use a mirror to increase the client’s awareness of facial expression and how it might affect communication.

Instruct the client in articulation and speech volume and in breaking up sentences; advise the family to phrase questions in a way that elicits shorter responses.

Question 63

What are some interventions for sexual routine for a client with Parkinson’s Disease?

Answer 63

Educate the client to engage in sex after resting and voiding bowel and bladder, and when symptoms are best controlled with medications.

Screen for depression if passivity, lack of interest, or general apathy is a factor.

Question 64

What are some interventions for cognition for a client with Parkinson’s Disease?

Answer 64

Train the client to use external or visual cues during daily tasks. Use rhythmic cues and music, and practice with repetition.

Instruct the client in ways to reduce environmental distractions during cognitively demanding activities.

Educate caregivers to speak slowly and clearly with simple, one-step instructions and to introduce new concepts one at a time.

Question 65

The masklike facial expression of people with PD makes it imperative to diligently monitor pain during all exercise and stretching.

What are some interventions for rigidity and associated pain for a client with Parkinson’s Disease?

Answer 65

Use moist heat, stretching, and gentle ROM.

For clients with contractures, an antispasticity orthotic or a dynamic or static progressive splint gives a low-intensity stretch against the contraction of the antagonist muscles. Advise the client on a wearing schedule and skin integrity checks to prevent breakdown.

Help the client advocate for better control over the environment; family or coworkers may prefer a cool home or work environment, but colder temperatures increase muscle stiffness.