Specific Pop: Chapter 25: Degenerative Diseases of the CNS

General Considerations

• Disease course is progressive and unpredictable although decline is a probable eventuality

  • When prescribing AE and DME consider the duration of time it may be functional

• ADL performance may fluctuate throughout the day and day to day

• Psychological impact on patient and family

Conditions

. Multiple Sclerosis (MS)

• What it is: An autoimmune disease where the body attacks the myelin sheath around nerves in the brain and spinal cord.

• Symptoms: Fatigue, muscle weakness, vision problems, balance issues, cognitive changes.

• Type: Unpredictable; has relapsing-remitting or progressive forms.

2. Parkinson’s Disease (PD)

• What it is: A movement disorder caused by low dopamine levels due to death of brain cells in the substantia nigra.

• Symptoms: Tremors, slow movement (bradykinesia), stiffness, postural instability, freezing, facial masking.

• Type: Chronic and progressive.

3. Amyotrophic Lateral Sclerosis (ALS)

• What it is: A motor neuron disease causing the death of nerve cells controlling voluntary muscles.

• Symptoms: Muscle weakness, atrophy, difficulty speaking/swallowing/breathing. Cognition is usually intact.

• Type: Rapidly progressive and fatal.

4. Alzheimer’s Disease (AD)

• What it is: A form of dementia that causes progressive memory loss and cognitive decline.

• Symptoms: Forgetfulness, confusion, trouble with daily tasks, personality changes.

• Type: Chronic and progressive.

MS Eval Perfromance Skills

Motor Skills – Often affected by fatigue, weakness, and coordination issues

• Aligns / Stabilizes – May struggle with posture or maintaining balance due to core weakness or spasticity

• Positions – Difficulty adjusting body safely due to limited range of motion or dizziness

• Reaches – Fatigue or tremors may limit overhead or repeated reaching

• Bends – Risk of falls or loss of balance when bending

• Grips / Manipulates – Fine motor coordination may be impaired (e.g., buttoning, writing)

• Coordinates – Ataxia or tremors can affect bilateral hand use

• Moves / Endures – Muscle fatigue limits sustained movement; gait may be unsteady

• Paces – May rush or slow down inconsistently due to fatigue or cognitive fog

🔹 Process Skills – Can be impacted by cognitive changes like slowed thinking or memory

• Initiates – May have trouble starting tasks due to fatigue, depression, or poor attention

• Attends – Difficulty staying focused due to "brain fog" or mental fatigue

• Chooses / Uses – May use wrong tools or forget proper use due to cognitive symptoms

• Sequences / Organizes – Steps may be out of order or disorganized (e.g., in cooking or dressing)

• Navigates – May bump into things or misjudge distances due to sensory or visual changes

• Adjusts – Harder to adapt when something goes wrong during a task

🔹 Social Interaction Skills – Can be impacted by fatigue, speech issues, or mood changes

• Initiates / Transitions – May avoid or struggle to start conversations due to social withdrawal or low energy

• Speaks Clearly – Speech may be slurred or slowed (dysarthria)

• Looks / Turns Toward – May be distracted or fatigued, reducing responsiveness

• Regulates – Emotional lability (mood swings) can impact interactions

MS Interventions

🔹 1. Strengthening & Endurance

• Use low-resistance, high-rep exercises

• Avoid overexertion — energy conservation is key

• Focus on core stability, balance, and UE/LE function

• Use rest breaks and monitor fatigue levels

🔹 2. Contracture Prevention & Treatment

• Regular ROM (range of motion) exercises

• Stretching to reduce spasticity

• Splinting or positioning devices for hands, feet, or limbs

• Encourage movement throughout the day

🔹 3. ADL, IADL, Work, Leisure Training

• Adaptive strategies for dressing, grooming, cooking, etc.

• AE: Reachers, dressing sticks, built-up handles, shower chairs

• Task simplification and activity pacing

• Return-to-work planning or job modification

• Promote engaging leisure activities (can reduce depression/fatigue)

🔹 4. Communication Support

• Collaborate with SLP if needed (for dysarthria or cognitive-communication deficits)

• Use communication aids: voice-to-text, memory notebooks, timers, planners

🔹 5. Seating & Wheeled Mobility

• Evaluate for proper wheelchair fit

• Support postural alignment

• Recommend cushions, backrests, or tilt features as needed

• Encourage self-propulsion if appropriate

🔹 6. Psychosocial Concerns

• Emotional Stages: Shock → Denial → Anger → Grief

• OT role:

  • Help develop coping strategies

  • Build a support system (family, groups, therapy)

  • Use positive reframing (focus on strengths)

  • Encourage future planning

  • Promote humor, hope, and acceptance

Parkinson’s Disease

🔹 4 Cardinal Signs

1. Rigidity

   • Stiffness in skeletal muscles (not velocity dependent like spasticity)

   • Can affect facial expressions ("masked face") and limbs

2. Resting Tremor

   • Usually seen in hands as a "pill-rolling" motion

   • Decreases with voluntary movement, increases at rest or under stress

3. Bradykinesia

   • Slowness of movement

   • Delayed initiation of movement

   • Difficulty with repetitive actions (e.g., buttoning, writing)

4. Postural Instability

   • Stooped posture, forward head

   • Poor balance and increased fall risk

   • Narrow base of support, shuffling gait

PD

Epidemiology

• ~500,000 people living with PD in the U.S.

• ~50,000 new cases diagnosed yearly

• More common in men – ratio 3:2

• Average age of diagnosis: ~60 years old

🔹 Cause

• Multifactorial – combo of genetics and environment

• Genetic links in ~10–15% of cases

• Environmental risks:

  • Repeated traumatic brain injury (TBI)

  • Long-term pesticide/herbicide exposure

🔹 Impact on Client Factors

• Motor: Rigidity, tremors, bradykinesia, postural instability

• Cognition: Slowed processing, executive dysfunction, memory decline

• Speech: Monotone, low volume (hypophonia), poor articulation

• Facial expression: Reduced (masked face)

• Sensory: Possible pain or decreased proprioception

• Fatigue: Common and persistent

• Autonomic issues: Constipation, orthostatic hypotension, sweating

🔹 Medical Management

• Medications:

  • Levodopa/Carbidopa (main treatment) – replaces dopamine

  • Dopamine agonists and other symptom-managing drugs

• Surgical options:

  • Deep brain stimulation (DBS) – helps with tremors and rigidity

• Multidisciplinary care:

  • Neurology, OT, PT, speech therapy, mental health support

PD; OT Management

Research Insight

• Studies show that focused, repetitive practice of meaningful tasks improves functional performance in individuals with PD.

• Neuroplasticity is supported through task-specific training, which enhances motor learning.

🔹 Evidence-Based Interventions

1. LSVT BIG® (for Movement)

• Focuses on large, exaggerated movements

• Improves gait, balance, and motor control

• Often used by OT and PT to treat bradykinesia and rigidity

2. LSVT LOUD® (for Speech)

• Led by Speech-Language Pathologists

• Trains clients to use louder, clearer speech

• Improves vocal strength and communication

3. Rock Steady Boxing

• Non-contact boxing program for individuals with PD

• Targets coordination, strength, endurance, and reaction time

• Also supports confidence and emotional well-being

4. Music Therapy

• Uses rhythm and beat to improve gait initiation, movement timing, and emotional health

• Can include dance, drumming, or rhythmic cueing to assist with freezing episodes

PD; OT Management

1. Decreased Postural Control

• Fall risk is high due to stooped posture, poor balance, and delayed reflexes

• Use gait belts when needed

• Encourage environmental safety (grab bars, remove tripping hazards)

🔹 2. Orthostatic Hypotension

• Sudden drop in blood pressure when standing

• Can cause dizziness or fainting

• Instruct clients to stand slowly, stay hydrated, and monitor symptoms

🔹 3. Dysphagia (Swallowing Difficulty)

• Risk of choking or aspiration

• Collaborate with SLP

• Monitor for coughing during meals, drooling, or wet-sounding voice

🔹 4. Skin Integrity

• Reduced movement and posture changes can cause pressure sores

• Monitor for skin breakdown in bony areas

• Encourage movement and weight shifting

PD OT Management 

1. ADLs, IADLs, Work, & Leisure

• ADLs: Use adaptive equipment (e.g., weighted utensils, long-handled tools) and task simplification

• IADLs: Break tasks into steps, use visual cues and written reminders (e.g., for cooking, meds)

• Work: Recommend job modifications or ergonomic supports

• Leisure: Support engagement in meaningful hobbies to boost mood and quality of life; modify activities for energy conservation

🔹 2. Motor Skills & Prevention of Deformities

• Maintain ROM and flexibility through daily stretches and movement

• Promote upright posture with seated alignment and standing balance exercises

• Use splints or positioning tools to prevent contractures (especially in hands and spine)

• Encourage regular movement to prevent joint stiffness and loss of function

🔹 3. Communication

• Work with SLP to support speech issues (hypophonia, facial masking)

• Use external cueing: visual schedules, written communication, communication boards or apps

• Encourage amplification devices if voice volume is very low

🔹 4. Psychosocial Issues

• Address depression, anxiety, social withdrawal

• Encourage routines, support groups, and coping strategies like positive reframing

• Help client maintain social roles and identity (e.g., as a parent, spouse, worker)

• Promote autonomy and dignity through independence in tasks

🔹 5. Advanced Parkinsonism

• May involve severe bradykinesia, rigidity, dementia, freezing of gait, or bed dependence

• OT focuses on:

  • Positioning and skin integrity

  • Caregiver education

  • Transfers and mobility aids

  • Environmental modifications (hospital bed, commode, etc.)

  • Simplified ADLs with max assist or total assist

  • End-of-life care and support for client/family

Amyotrophic Lateral Sclerosis

What It Is

• A progressive, fatal neurodegenerative disease that affects motor neurons in the brain and spinal cord.

• Leads to muscle weakness, paralysis, and eventually respiratory failure.

• Cognition is usually intact, especially in early to mid stages.

🔹 Epidemiology

• Men are about 20% more likely to develop ALS than women.

• Average age of diagnosis: ~55 years old.

• ~30,000 people are living with ALS in the U.S.

🔹 Etiology (Cause)

• Unknown in ~90% of cases (called sporadic ALS)

• ~10% of cases are familial/genetic

• Potential factors: military service, environmental toxins, head trauma, and viral exposure – but none confirmed

🔹 Prognosis

• Most people with ALS die from respiratory failure within 2–5 years of symptom onset

• Some may live longer, especially with ventilator support (e.g., Stephen Hawking)

ALS OT Management

Assessment Tool

• ALS Functional Rating Scale (ALSFRS-R)

  • Assesses physical function in areas like walking, dressing, speaking, swallowing, and breathing

  • Helps track disease progression and guide treatment priorities

🔹 Additional Areas to Evaluate

• ADL/IADL performance

• Muscle strength and endurance

• Fine motor control

• Respiratory function impact on activity tolerance

• Assistive device needs

• Cognitive status (if affected)

OT Interventions in ALS 🔸 1. ADLs

• Use adaptive equipment (button hooks, reachers, shower chairs)

• Focus on energy conservation

• Train caregivers for increasing assistance needs

• Modify tasks to support independence as long as possible

🔸 2. Motor Skills & Deformity Prevention

• Maintain ROM through stretching and gentle exercises

• Use splints or positioning supports to prevent contractures

• Avoid overfatiguing weak muscles – conserve strength

🔸 3. Communication

• Collaborate with SLP

• Use low-tech aids (communication boards) or high-tech options (speech-generating devices, eye-tracking systems)

• Educate caregivers on communication strategies

🔸 4. Assistive Technology

• Recommend voice-to-text tools, mobility aids, and environmental controls (e.g., Alexa, switches)

• Progressive adaptation: Update tools as motor function declines

• Support independence in phone use, computer access, lighting, etc.

🔸 5. Seating, Positioning & Mobility

• Ensure postural alignment and pressure relief

• Evaluate for power wheelchair early in disease progression

• Recommend tilt/recline features, cushions, and custom positioning

• May need hospital bed or Hoyer lift in later stages

🔸 6. Psychosocial Support

• Address depression, anxiety, and loss of independence

• Encourage support group participation

• Help client maintain roles and routines (e.g., as a parent, spouse)

• Promote legacy projects, journaling, or goal setting

• Offer grief support and end-of-life planning when appropriate

Alzheimer’s Disease

What It Is

• The most common known cause of dementia in adults.

• A progressive, degenerative brain disorder that affects memory, thinking, behavior, and ability to perform daily tasks.

🔹 Epidemiology

• Affects over 4.5 million Americans

• Incidence increases significantly after age 65

• Women are more often diagnosed, partly due to longer lifespan

🔹 Etiology (Cause)

• Multifactorial and still not fully understood

  • Age: #1 risk factor

  • Genetics: Family history increases risk

  • Lifestyle: Heart health, diet, exercise, and mental stimulation may play a role

  • APOE-e4 gene linked to increased risk

🔹 Signs & Symptoms (General Progression)

• Early stage:

  • Mild memory loss, confusion, difficulty with word-finding or planning

• Middle stage:

  • More noticeable memory loss, wandering, personality changes, difficulty with ADLs

• Late stage:

  • Severe cognitive decline, inability to communicate or walk, total dependence

🔹 Medical Management

• No cure — focus is on slowing progression and managing symptoms

• Medications:

  • Cholinesterase inhibitors (e.g., Donepezil) for memory and cognitive symptoms

  • NMDA antagonists (e.g., Memantine) for moderate to severe stages

• Behavioral meds: May be prescribed for agitation, depression, or sleep issues

• Supportive care: Multidisciplinary team including OT, PT, SLP, nursing, and social work

OT Evaluation in Alzheimer’s Disease

• Cognitive status: memory, attention, orientation, judgment

• ADLs & IADLs: identify retained skills vs. areas of decline

• Safety awareness: wandering, falls, medication errors

• Motor skills: especially in later stages (balance, coordination)

• Communication abilities

• Environment: assess for hazards, structure, and support

• Caregiver capacity and needs

Assessment tools may include the Allen Cognitive Level Screen (ACLS), Mini-Mental State Exam (MMSE), or MOCA.

OT Interventions in Alzheimer’s Disease 🔹 1. ADL Support

• Use simple cues and step-by-step instructions

• Maintain routines to reduce confusion

• Use visual cues, labels, and contrasting colors for task guidance

• Grade tasks for success and independence

🔹 2. Environmental Design

• Keep layout simple and consistent

• Reduce clutter and distractions

• Use signage (with pictures/words) to support navigation

• Install grab bars, lighting, and safety locks

• Create a safe space for wandering

🔹 3. Day Care & Group Activities

• Structured adult day programs reduce caregiver stress and support engagement

• Activities may include:

  • Music, art, gardening

  • Reminiscence groups

  • Cooking or craft activities with supervision

🔹 4. Reality Orientation

• Use clocks, calendars, labeled photos, and current events

• Encourage verbal and visual reminders of date, location, and identity

• Be gentle—orientation should not cause distress

🔹 5. Exercise Programs

• Focus on balance, strength, flexibility, and mobility

• Helps with falls prevention and mood regulation

• Use repetitive, rhythmic activities (e.g., walking groups, chair yoga)

🔹 6. Psychosocial Issues

• Common: depression, anxiety, paranoia, agitation

• OT can:

  • Provide calming activities and sensory regulation

  • Support caregiver coping and education

  • Encourage meaningful routines and purposeful roles

  • Foster dignity, reassurance, and validation