Neurodegenerative Conditions 😶‍🌫️

ALS, MS, Huntington’s, Guillain Barre, Parkinson’s, Dementia

Akinesia

-freezing gait/ other movements

-teaching rocking is useful in helping clients initiate movement

Ataxia

-uncoordinated movements, tremors may be present

~you need to call “a taxi” because you’re uncoordinated

-tx: stabilize proximally to help distal movements; pt can use unaffected side over hand with tremor to help with fine motor tasks; orthoses like c-collar/wrist splints can help to reduce impact of tremors

Dyskinesias

Involuntary, non-repetitive movements that are associated with basal ganglia damage

Dystonia

-creates sustained abnormal postures/disruptions of ongoing movement

Dysmetria

decreased coordination of movements *dysmetria ~ off meter/ off beat

Festinating gait

Shuffling gait- small, quick steps from forward-tilted head and trunk posture

Special Evaluation Consideration for Neurodegenerative Conditions

-How pt’s disease may progress and/or have variable symptoms (e.g. symptoms that change during the day/week/month/seasons)

-Pt’s understanding/ level of acceptance of progressive nature of disease

General Neurodegenerative Disease OT Interventions

-Help client compensate/adapt as function declines

-Ex: cognitive compensatory strategies, energy conservation, environmental mods, adaptive equipment, exercise, caregiver training

Multiple Sclerosis (onset, diagnosis, symptoms, patterns, medical management)

-slowly progressing CNS disease of demyelination characterized by remissions and exacerbations of symptoms

Onset

• most often in 30s (20-50)

Symptoms

• Acute/beginning

  • weakness/clumsiness in leg or hand

  • paresthesia (in one or more extremities/trunk/face)

  • visual disturbances (diplopia, partial blindness, nystagmus, eye pain)

  • emotional disturbances (lability, depression, euphoria)

• Long term is typically fatigue, general weakness, intention tremors

• Balance issues/vertigo

• Bowel/bladder/sexual dysfunction

• Spasticity/increased reflexes/ataxia/tremors/pain
  

4 types: relapse remitting (best), secondary progressive (has some neurological dysfunction), primary progressive (no remission, neuro function worsens), progressive relapses (worse)

MS OT Intervention

-Gentle ROM is good, so is caregiver education
-Strengthening is usually a bad thing
-cognitive and physical abilities can vary throughout the day- try to time sessions when pt usually feels the most energized

-can benefit mentally/emotionally from group-therapy/CBT

-sitting with at least 90 hip flexion can help reduce spasticity

Amyotrophic Lateral Sclerosis (ALS) (Prognosis, Symptoms, Stages, Medications, Interventions)

-Motor neurons in CNS & PNS are replaced by scar tissue over time (plaques)

-AKA Lou Gehrig’s disease

-onset ~57 y.o.

-death in 2- 5 years after onset

Symptoms

• muscle weakness/atrophy progresses distally to proximally

  • most common first symptoms are weakness in small hand muscles or asymmetrical foot drop

• Muscle cramping at night- usually in calves

• Corticospinal tract involvement symptoms: spasticity & hyperactive deep tendon reflexes

  ⚡️spinal & spasticity

• Corticobulbar tract involvement

  • Dysarthria (slowed/slurred speech)

  • Dysphagia (difficulty swallowing)

  • ⚡️difficult to talk/swallow with a bulb in your mouth

• May eventually have difficulty breathing, requiring respiratory therapist to be on team

• Sensation, vision, hearing, bowel/bladder usually NOT affected

Stages

• Stages I-III

  • can walk, but growing weakness (some → moderate → severe)

• Stage IV

  • requires W/C (d/t extreme weakness in LEs)

  • require some assistance with ADLs

• Stage V

  • Dependent with ADLs (d/t extreme weakness in arms and legs)

• Stage VI

  • unable to get out of bed

Medications- slightly slow disease progression/reduce negative impacts on functioning but have side effects

• Riluzole/Rilutek- dizziness, fatigue, GI issues, liver issues

• Edaravone (Radicava)- swelling, SOB

Interventions:

• because it is progressive/degenerates a large part of functional skills, CAREGIVER education is really important for issues

• early stage: try to keep some muscle, just dont overexert

• middle stage: muscle loss will happen, better for ROM

• late stage: ulcer prevention

• W/C recs: often start with high backed, reclining, light weight, small turning radius, with head/trunk/extremity support then progress to power W/C with adaptable controls

• Use voice operated/hands-free tech

• Can extend ability to drive a car by using hand controls and adding W/C lift

Presbyopia

Decreased ability to focus clearly on close objects, and switching from near to far

General Parkinson’s Info (Symptoms, Medications, Evaluation, Intervention)

-CNS movement disorder

-Symptoms

• pill-rolling tremor in one hand- usual first sign

• TRAP

  • Tremor

    • -resting tremors of hand (not usually present during sleep/voluntary mvmnts)

  • Rigidity

    • -muscle rigidity that is NOT velocity dependent during passive movement

    • Cogwheel or lead pipe rigidity

  • Akinesia- no/slow voluntary movements

    • Motoric freezing

  • Postural Instability

    • stooped posture, lack of arm swing, festinating gait, retropulsion (falling backward), falling forward (propulsion)

      • inc fall risk

-stress exacerbates symptoms

-life expectancy usually not impacted but QOL is

• depression

• Fatigue

• Urinary/sexual dysfunction

• Swallowing difficulties

• Weight loss

• Sensory symptoms

-Medications

• Used to help with loss of dopamine

  • Levodopa

  • Carbidopa (Sinemet)

  • May see increased dyskinesia when medication is having peak effects; side effects are somewhat unpredictable

• Help with rigidity/tremors

  • Benadryl

  • Arcane

  • Cogentin

• Antiviral drug that helps with symptoms

  • Amantadine

-Evaluation

• COPM to find “empowering” goals, evaluate at multiple times of day to get accurate picture, may need to screen cognition/depression

-Intervention

• freezing (avoid crowds/tight spaces/turns/rushing/clutter/distractions)

• instruct on use of cane/walker

• counting/singing/auditory cueing can help make movements smoother

• Feeding

  • built-up/weighted utensils/spill guards

  • increased time to eat

  • distal wrist weights for tremors

  • put arms on table and keep items close to body to inc stability)

• Movement (LSVT BIG for gross and fine motor coordination- need cert)

• Active ROM against gravity that is occupation based can be good for cogwheel rigidity

  • E.g. reaching overhead for a box of cereal on a shelf when cogwheel rigidity is present in both shoulders

Stages of Parkinson’s Disease

Stage I

• Unilateral tremor

• Minimal-no functional impairment

Stage II

• Bilateral tremor

• Independent with ADL

• No balance impairment

Stage III

• first signs of impaired righting reflexes

• onset of disability in ADL performance (min A)

• can lead independent life

• Start to see high fall risk

Stage IV

• requires help with some or all ADL

• Unable to live alone without some assistance

• Able to walk/stand (with walker)

Stage V

• Unable to stand/walk

• Unable to perform ADLs

• W/C and Max A are required

Extrapyramidal Syndrome

-movement disorders such as akathisia (cant stay still), dystonia (twisting motion), and parkinsonism (tremors) caused by antipsychotic drug therapy
-dangerous if working with tools

Huntington’s Disease AKA Huntington’s Chorea (Prognosis, Symptoms, Stages, Eval, Intervention)

-onset during early middle age (30s/40s)

-usually fatal ~20 years after the onset of symptoms

-hereditary components
Symptoms

• characterized by uncontrollable body movements

  • Chorea (rapid, involuntary movements)

  • Akathisia (motor restlessness)

  • Dystonia (abnormal posturing, usually of arms)

  • Bradykinesia (slow movements)

  • Akinesia (lack of initiation)

  • Impaired walking/balance and decreased motor control (use AE)

• Cognitive Decline

  • difficulty with some cognition (i.e. word retrieval skills)

  • Difficulty with problem solving skills

• Dysphagia- difficulty swallowing

• Psychosocial impacts/Emotional Disturbances

  • Usually significant depression/social isolation b/c it is often diagnosed during middle age

  • Some individuals die by suicide

  • Fatigue (use activity mod/breaks)

Stages

• Early stages-

  • Stage 1 (Early Stage) & Stage 2 (Early Intermediate Stage)

  • some impacts at work and home from impacts on balance/motor/coordination, but mostly independent

• Middle stages-

  • Stage 3 (Late Intermediate Stage)

  • difficulty with memory and decision making

  • work becomes difficult as well as IADLs

• Later stages-

  • Stage 4 (Early Advanced Stage) & Stage 5 (Advanced Stage)

  • dysarthria makes speaking difficult

  • High risk for choking & falling

  • unable to work

  • needs major and eventually full assistance with care

Evaluation

• Unified Huntington’s Disease Rating Scale- assesses motor, cognitive, functional, and behavioral changes

Intervention

• Should be compensatory

• Early on modifications should focus on activities that require fine motor skills (fastening zippers, buttons, hooks, etc.) since challenges are usually limited to fine motor coordination at the beginning

• Community/support groups

• Eating- may need modified diets, adaptive utensils, minimal distractions when eating

• Maintain community mobility + safety in home

• Maintain valued roles

  • May need to find a simpler job

• Finance management

  • Can encourage auto payments

• Environmental Mods

Guillain-Barré Syndrome (GBS) Overview (Onset, Prognosis, Diagnosis, Symptoms)

-Acute and rapidly progressing demyelination of the peripheral nerves and spinal roots

Onset- equal for all ages

Prognosis

• Recovery generally happens after 2-4 weeks after onset

• Long-term Impacts

  • Fatigue is most commonly reported residual symptom

  • 80% are ambulatory within 6 months

  • 50% have mild neurological deficits

  • 15% have residual functional deficits

  • 5% die from complications

Symptoms

• acute, rapidly progressing symmetric muscular weakness

  • Weakness starts distally and progresses proximally

• mild distal sensory loss/parasthesias

  • Often start in stocking-glove distribution

• Loss of deep tendon reflexes

• Respiratory failure and dysphagia in some cases (need to monitor vitals and have a respiratory therapist)

Acute Guillain-Barre Syndrome

-onset is sudden and rapidly progressing- usually starts with paralysis of feet that moves up

-most prominent factors is decreased strength of muscles which can lead to swallowing issues, fatigue (biggest pt complaint), and pain

-loss of deep tendon reflexes

Plateau Guillain-Barre Syndrome

-where symptoms are worse, may need ventilator
-paralysis of the muscles of the head and neck can affect speaking, swallowing

-OT eval often covers positioning to prevent skin breakdown, communication, control of phys env, comfort, and anxiety management

Intervention:

-train communication tools (signs/picture boards),

-adjust laying/sitting positions to optimize function/comfort and limit risk of skin breakdown (teach caregivers as well)

-Position client for trunk/head/ UE stability

-Educate pt/family about condition and anxiety-reduction techniques

Recovery Guillain-Barre Syndrome

-GBS has no cure but ~80% of pts can walk after 6 months, 60% get full strength back after a year

-OT eval focuses mobility, ADLs, communication, and community/work reintegration

Intervention:

-energy conservation/fatigue management good diet
-gradually increase tolerance of complexity of task

-dynamic splints can help maintain ROM for wrist/fingers/ankle i.e. hinged drop-foot orthosis

-Adapt modes of communication based on client (e.g. speech to text on phone)

Myasthenia Gravis

Rare condition caused by autoimmune attack on acetylcholine receptor

Onset

• Younger women

• Older men

Prognosis

• varied, but progressive and disabling

• Death may occur from respiratory complications

Symptoms

• Episodic muscle weakness

  • Muscle fatigue after exercising

  • Proximal limb weakness

  • Weakness in muscles for swallowing/talking (dysphagia/dysarthria)

  • Difficulty breathing (may require ventilator)

• Impacts on eyes

  • Ptosis (eyelid drooping)

  • Diplopia (double vision)

• Anxiety

  • Tachypnea

  • Tachycardia

  • Restlessness

Medical Treatments

• Cholinesterase inhibitors/anticholinergics

• Immunosuppressive agents/corticosteroids

Dementia vs. Normal Aging

-If a change is noticed look deeper

-Treatable causes of change- mental health issue, physical health issue, medication complication, sensory change like hearing loss, new chronic condition like diabetes

Dementia vs. Alzheimer’s

Dementia- not a diagnosis, but a general umbrella term for deterioration of brain capacity (at least 2 parts of brain are dying)/cognitive decline that is constantly changing; consists of 85-90 different conditions; can’t be fixed/stopped at least right now

Alzheimer’s- most common form of dementia (~75%), associated with specific patterns of brain changes

4 A’s of Alzheimer’s (Amnesia, Apraxia, Aphasia, Agnosia)

Amnesia

• loss of memories (facts, info, experiences)

Apraxia

• loss of ability to map out motor actions in order to repeat them in functional activities

Aphasia

• inability to comprehend/formulate language because of damage to specific brain regions

Agnosia

• loss of ability to recognize objects, faces, voices, or places

Vascular Dementia

-from series of mini strokes

-2nd most common type of dementia after Alzheimer’s

-Cognitive deficits similar to Alzheimer’s but with better memory in tact

-Gait impairment

-Typically see sudden appearance/ abrupt worsening of symptoms

-Requires immediate emergency medical attention

Frontotemporal Dementia

-marked by behavioral changes such as impulsive, listless, inappropriate social, lack of empathy, loss of insight to behaviors, increased sex drive, progressive aphasia, symptoms similar to Parkinson’s

-Sudden onset

-Medical tx still in development

Dementia with Lewy Bodies

-Marked by visual hallucinations and parkinson’s-like motor symptoms

⚡️hallucinations~ ha”lewy”cinations

-gradual progression

Early Stage Dementia

-Difficulty with IADLs (money management, way-finding)

-Increased irritability, mood swings, sadness, anxiety

-More forgetful of recent events

-Signs can look similar to age-related memory loss

Middle Stage Dementia

-Confusion with time and place

• May become lost in familiar places

-Moderate IADL issues
-Difficulty making choices (like with clothes)

• May behave inappropriately e.g. wearing winter clothes on a hot day

-General confusion/difficulty recalling recent events
-Issues with judgement of direction, distance may increase falls

• Difficulty executing simple movements
  

-May have visual/perceptual issues, difficulty with reading
-They may think they’re in an earlier stage of life

-May see/hear things that aren’t there

Late Stage Dementia

-Unable to remember events that happened a few minutes before

-Difficulties with posture and balance

• Bed-bound

-Swallowing and chewing difficulties
-Speaking issues, may only say a few words

-Need help with feeding, washing, bathing, etc.

-May have uncontrolled movements

Random Dementia Pointers for the Exam

-Observation and interviewing family is good for assessment
-Most assessments likely too cognitively challenging
-Family can use locks, door/bed alarms, visual barriers for safety
-Visual cues are good for memory issues, as well as bathroom schedules for independence

Screening Tools for Cognition

-Mini Mental and BIMS are quick and good to start out with

-Use MOCA/SLUMS for early stages of Alzheimer’s as they are for executive functioning

Types of Skill Learning (Generalization vs. Transfer)

-Generalization: skill from one context to another
-Tranfer: strategy of one task to another

Motor Learning Key Words for the Exam

-doing the activity is a good answer
-any type of cueing is a good answer

General Interventions for Dementia (Environmental changes, Monitoring personal comfort, Providing security objects, Providing opportunities for exercise)

Environmental Changes

• Create a safe environment

• Prevent falls, avoid scatter rugs/clutter

• Create a calm environment by reducing background distractions, stressors or clutter

Monitor Personal Comfort

• Make sure room temp is comfortable

• Monitor skin irritation

Provide Security Object

• Give favorite blanket/book/anything comfortable that client is familiar with

Provide Opportunities for Exercise

• Wandering is sometimes caused by need to move

Allen’s Cognitive Levels (Mnemonic & Overview)

⚡️All Players Must Get Extra Points

1- Automatic

2- Postural

3- Manual

4- Goal-directed

5- Exploratory

6- Purposeful

Allen’s Cognitive Level 1

Automatic/Awareness

• Essentially in a coma

• Automatic motor response

• Total assistance with all activities and 24/7 care

• Attention is fleeting and non-selective/not conscious

Focus on single sensory activities, bed mobility, skin integrity, passive mobility

1.4 location stimuli
1.6 rolling in bed
1.8 raising body part

Allen Cognitive Level 2

Postural/ Large Body Movements

• Some awareness about posture, gross body movements, large objects in the room

• Can overcome effects of gravity

  (work on sitting and righting reactions)

• Can imitate gross motor actions

  • ADLs can be accomplished by imitation

• Max assistance 24/7 care

  • Need close on site-supervision

  • Risk of wandering (try to give client activity and implement safety measures)

• Create a sensory rich environment that helps to give client sensory stimulation

2.0 overcoming gravity/sit
2.4 aimless walking
2.6 directed walking

Allen Cognitive Level 3

Manual actions/ Moderate assistance

• Uses hands to manipulate objects

• Can complete manual repetitive actions e.g. coloring

• Need set up/supervision for ADLs; can perform familiar ADL tasks but may be distractible/unpredictable

  • May need tactile cues/ demonstration/ long-term repetitive training

• Cause and effect emerges

• Able to imitate running stitch (three stitches)
  

3.0 grasping objects
3.2 distinguishing objects
3.4 sustaining actions on objects

Allen Cognitive Level 4

Familiar Activity/ Min assistance

• Able to perform purposeful short tasks (making sandwich, buying drink from a store)

• May need visual cues (but no longer need tactile cues)

• Needs supervision at lower levels, but can live alone at 4.6 with some check-ins

• Needs assistance with new tasks

• Able to imitate whip stitch (three stitches)

• attention last up to an hour, can do goal directed actions e.g. putting a bird feeder together
  

4.0 sequencing familiar actions
4.2 differentiating features of objects
4.4 completing a goal
4.6 cut off for independent living

Allen Cognitive Level 5

Exploratory/ Learning new activity

• New learning can occur

• Learns through trial and error

• Standby assist for ADLs

• Poor organization and socialization

• Able to imitate the single cordovan stitch using overt trial and error method for three stitches
  
  

  5.2 live independently with weekly checks

  5.4 self directed learning
  5.6 can live independently with no checks; considering social standards
  5.8 consulting with others

Allen Cognitive Level 6

Planned

• No global cognitive impairment

• Independent with all activities (No supervision needed)

• New motor learning can be done safely

• Plans new activities

• Reflect on mistakes

• May seek guidance

Reisberg Scale (Global Deterioration Scale)

Stage 1

• no noticeable impairment

Stage 2

• Memory impairment begins to be noticeable

• might get memory confused with aging

Stage 3

• Family members/health care providers notice mild decline with problem solving/remembering names/details

Stage 4 Alzheimer’s Stage I

• Obvious difficulty with problem solving/remembering names/details

• May withdraw from new social situations

• Interventions: Visual aids, familiarity

Stage 5 [Alzheimer’s Stage II (Bashful)]

• Patient will begin to need help with functional ADLs

• May benefit from adult day care

• Interventions: simplify tasks in familiar locations, make objects accesible, eliminate environment distractions, goal-directed with visuals and initiation cues (e.g. help select outfit), use personal photos to promote memories and social connection

Stage 6 [Alzheimer’s Stage III (Grumpy)]

• Requires assistance with most ADLs

• Personality begins to change

• May become delusional/aggressive

• Not oriented to time

• Interventions: Caregiver training on routine/behavior management, structured tasks, home mods for safety (Audiovisual systems ~baby monitors, camouflage exits, hide knives), keep them active during the day to reduce night-time wandering

Stage 7 [Alzheimer’s Stage IV (Sleepy)]

• Lose ability to walk/communicate

• Dependent with all activities

• Nursing home likely necessary

• Interventions: quality of life training for caregivers, contracture/pressure sore prevention (splinting and positioning), sensory stimulation for comfort and contact with reality (soothing sounds, touch)