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NEURODEGENERATIVE & NEUROMUSCULAR DISORDERS – OUTLINE

Multiple Sclerosis (MS)

  • Definition: An autoimmune demyelinating disease – brain and spinal cord
    • Prevents or slows down nerve transmission
    • Functions impacted: vision, cognition, movement, balance, and sensation
  • Cause: Unknown. More frequent in women with onset 20-40 yrs of age
  • Slowly progressive and chronic. Progression occurs in 4 courses
    • Relapse remitting (85%)
    • Fluctuating (50%)
    • Secondary progressive w/relapses
    • Primary progressive – deterioration from onset (10%)
    • Progressive relapsing (5%)
  • Signs & Symptoms
    • FATIGUE is a classic symptom
    • Primary Fatigue – cortical damage, overwhelming feelings of fatigue
    • Secondary Fatigue – deconditioning, respiratory muscle weakness, pain
  • Details of S/S and Effects on Function
  • Comes & Goes
  • Sensory
    • Paresthesias – abnormal pins and needles sensations
    • Sensitive to heat – consider weather
    • Vertigo
    • Pain
    • Impact on ADLs and mobility – ↑ risk for falls
  • Affect/Emotions
    • Lability
    • Euphoria
    • Reactive Depression
  • Visual
    • Diplopia
    • Scotoma
    • Loss of visual acuity (80%)
    • Optic Neuritis – sudden loss of vision w/pain behind the eye; subsides
    • Impact on navigation and activity participation
  • Motor
    • Clumsy and weak in UE and LE common
    • Impaired balance and coordination; ataxia
    • Fatigue, muscle weakness
    • Partial or complete paralysis
    • Spasticity
    • Intention tremors – with movement
    • Cognition – apathy, STM loss, inattention, ↓ exec fxn; slow process
  • OT Intervention
    • Vision – clear paths, contrast, optical devices (lg print, lg buttons)
    • Sensory – sensory re-ed, caution for burns —> visual feedback
    • Urinary – due to increased frequency & urination; monitor fluid intact
    • Cognition – rest breaks with tasks, give time to speak and process
    • Muscle Weakness – ergonomic positioning, joint protection, gentle ther ex
    • Pain from Spasticity – resting splints, 90° hip to ↓ LE extensor tone
    • Plan, pace, prioritize, and position: Energy conservation, work simplification
    • Dysphagia – ↓ distractions, thicken liquids, give time to eat
  • Precautions
    • Exacerbations and remissions: educate about prognosis (NOT feigning illness)
    • Avoid thermal PAMs
    • No extreme physical stress
    • Over several sessions due to fatigue

Amyotrophic Lateral Sclerosis (ALS) aka “Lou Gehrig’s Disease”

  • Definition– characterized by a progressive degeneration of motor neurons. Motor neurons gradually degenerate and die
  • Involves both UMN & LMN
  • 2 major subtypes: spinal progressive muscular atrophy & progressive bulbar palsy
    • Cause: Unknown
    • Onset: On average 57 y.o.
    • Death: within 2-5 yrs
  • ~Stephen Hawking has defied the odds living with ALS for decades!~
  • Comparison with Multiple Sclerosis
    • Does not affect sensory functions such as vision, touch, hearing
    • Does not affect bowel & bladder function nor does it affect sexual functions
    • Cognition is intact
  • Progression – Occurs distal-to-proximal, asymmetrically
  • Signs & Symptoms
    • LMN: Hands, Feet & progresses proximally towards trunk
    • Corticospinal tract: breathing, low-tone speech, swallow impaired
    • Loss of FMC – AD + splints to maximize function
  • 6 Stages
    • Walk, I with ADLs
    • Walk with moderate weakness
    • Walk with severe weakness
    • W/C with some A with ADLs with severe leg weakness
    • W/C with Total A with ADLs with severe arm and leg weakness
    • Bedbound – hospice; palliative
  • OT Intervention*
    • Compensatory & supports: Built-up handles, plate guards, U-cuff, neck collar
    • Mobility aids: foot drop splint, cane, high-back & recline-type walker that moves in small spaces, power w/c (late stage)
    • Adapt food consistency, give time to eat, ↓ distractions due to dysphagia
    • E Conservation and Work Simplification
    • Family collaboration – manage dysphagia, environmental mod, skin integrity
    • Ther ex: AAROM, PROM to prevent contractures (late stages), positioning to ↓ spasticity, diaphragmatic – decrease work of breathing, avoid cold that could influence spasticity
    • Communicaton: AAC – single-switch or eye gaze control
  • Precautions
    • Muscle strengthening thru Progressive Resistive Exercise (PRE) may cause cramping and fatigue: contraindicated
    • Watch for aspiration/choking and ↓respiratory function; suction required
    • Watch for pressure sores due to lack of movement
    • Avoid overwhelming person w/equipment & devices – may not receive well

Parkinson’s Disease (PD)

  • Definition: CNS movement disorder – slow, decreased movement and muscular rigidity, lack of the neurotransmitter dopamine
    • Cause: Unknown/Idiopathic
    • Onset: After age 40,
    • average: 55-60 yrs of age
  • Progression: Slowly progressive
  • Etiology: Degeneration – basal ganglia. The function of the basal ganglia
    • To initiate and maintain desired movements
    • To filter out undesired movements
  • Unable to control shakiness when their hands are resting on the table. They have trouble picking up their feet when walking
  • Parkinsonism – 2° to drugs such as anti-psychotic medications because they affect dopamine receptors. May demonstrate similar clinical features
  • Signs & Symptoms
    • Cardinal signs (MD’s use to diagnose): tremors, rigidity, + to Sinemet, akinesia, retropulsion, propulsion (falls fwd), mask-like face, micrographia
  • Types*
    • Primary: resting tremors – when does it ↑ or ↓?___, muscle rigidity/stiffness, cogwheel, bradykinesia, postural instability
    • Secondary: gait dysfunction – festinating gait, shuffling gait, ↓ FMC, ↓ executive function, impaired memory, visual-spatial, Dementia
  • Other – Communication (hypophonia, expression), dysphagia/drooling/slow eating, depressive mood, disrupted sleep
  • Clinical Stages – 5 Stages
    • Unilateral – no or limited loss of function
    • Bilateral – problems with trunk mobility & postural reflexes
    • Impaired balance – mild-to-moderate impairments
    • Decreased postural stability – A with ADLs
    • Total dependence for mobility and ADLs
  • OT Intervention
    • Impaired functional mobility – avoid tight spaces, clear pathways, wear flat shoes (not rubber soles, which ↑ risk for falls)
    • Caregiver training with single auditory cues (verbal cues, rhythmic) to ↑ quicker, smooth movements
    • Self-care & feeding – supports for coordination/managing tremorsdistal wrist wt, weighted utensils, thicken liquids, small portions, soft consistency
    • Communication – felt-tip pen, vocal responses, mirror for facial awareness
    • Rigidity and pain – moist heat, gentle ROM, diligently monitor pain
    • Maintain employment – sedentary job with minimal comm & low GM move
    • Encourage group exercise – manages bradykinesia, elevates mood, yoga
    • *Timing of medication doses – home health/caregiver training on med mgt
  • Precautions
    • High fall risk– postural instability; remove loose rugs & keep pathways clear
    • Dysphagia – prone to aspiration
    • Mask-like face – may make it difficult to determine if they have pain or depression. So, you want to diligently monitor.
    • *Side effects from meds can cause orthostatic hypotension, stomach problems, or dystonias

Guillain-Barre Syndrome (GBS)

  • Definition: Immune system attacks part of the PNS – myelin sheath and axons of peripheral nerves. Results are extreme muscle weakness, mild distal sensory loss, and tetraplegic-like paralysis. Differs from Multiple Sclerosis which affects the Central Nervous System.
  • Progression: 1st S/S are tingling sensations in the legs with different degrees of weakness – weakness and sensations spread to the arms and upper body and can increase in intensity. If diaphragmatic breathing affected, put on a ventilator. Good recovery even in the most severe cases.
  • Prognosis
    • Rate of recovery is very slow and variable
    • May take up to 2 years for full recovery for most, some residual weakness
    • **Note: This syndrome is extremely emotionally distressful ** Provide positive encouragement and instruct on disease course to ↓ fear and anxiety
  • Etiology: youngest age of onset 20-24 y.o., commonly between 30-50 y.o.
  • Signs & Symptoms: Acute, rapidly non-progressive polyneuropathy with symmetrical muscle weakness (vs ALS asymmetrical) and mild, distal sensory loss and paresthesias
  • Jack’s experience
  • Hallmark sign: muscle belly tenderness
  • Other: Weakness more apparent than sensory – begins in feet and ascends symmetrically, pain in LE, fatigue, edema (elevate feet), absent DTR, stocking-glove pattern (mild sensory loss pattern), possible facial palsy, some autonomic – check vitals for hypotension, diaphoresis, urinary retention
  • Three phases
  • Onset & Acute Inflammatory Phase – Symptoms are worse 2-3 weeks: no OT until patient is stable.
  • Plateau – Symptoms are stabilized, 2-4 weeks **Diabling**
  • Anxiety mgt focus on comfort and positioning, comm w/call button
  • Positioning trunk, head, and UE: stability
  • Adjust in supine and sitting positions to optimize fxn and comfort
  • PROM when at or below FAIR
  • Recovery Phase – Starts 6-12 mos
  • Head and neck, then recovers distally – i.e. use MAS for FM tasks
  • Resume occupations and roles
  • Gradually increase the number and complexity of tasks
  • *OT Implications: Functional levels may change; determine their current functional level and work from there, i.e. have them sit for grooming, then move to dressing and bathing as strength and endurance↑
  • OT Interventions in line with the 3 phases above including…E Conservation, Work Simplification, fine motor Q: Should we provide AD/AE during early stages?__
  • Precautions
    • Watch for signs of fatigue and discontinue activity to prevent overexertion
    • Watch for signs of substitution – use suspension slings or MAS to allow hand activities to continue without fatigue to shoulder and upper arms

Myasthenia Gravis

  • Definition: A disorder of neuromuscular transmission. It’s characterized by abnormal fatigue of voluntary muscle, mostly targeting the areas innervated by cranial nerves, such as that for the eyelids, eyes, and oropharyngeal muscles.
    • Etiology: Unknown
    • Onset: occurs at any age, more in women than men
  • Progression: Prognosis varies, but it’s usually a progressive, disabling process
  • Proximal > distal, sometimes asymmetric distribution
  • Intensity of the disease fluctuates, and its course is unpredictable
  • Death usually occurs due to respiratory failure
  • Signs & Symptoms
    • Muscle progressively weak during activity, improves with rest
    • Impaired facial muscles – dysphagia, dysarthria
    • Proximal limb weakness
    • Hallmark signs: Fatigue, ptosis
    • DTR intact (vs Parkinson’s which is not)
    • Quadraparesis
  • OT Intervention – FOCUS…**
    • Primary goal: regain muscle power and endurance
    • Lifestyle Mod: light weight dishes, place objects within easy reach, graded activities gentle/non-resist, E Conserv/Work Simplication/Pacing, Sit vs stand
    • Self-assess & monitor fatigue and drooling – mirror to check
    • Best after a period of rest and worst after activity; compensate
    • Self-feeding: small bites, food/liquid consistency
    • Educate on disease process
    • If UE are too weak: overhead slings, electronic aids, MAS, stress management
  • Precautions
    • Respect the pt’s activity tolerance levels
    • Educate on Borg Level – have them use a scale to rate their perceived exertion (6-20 scale: 6=no exertion, 20=max””), i.e. How do you feel sitting in a chair relaxed? 8 = requires little effort, 13 = somewhat hard
    • Watch changes in respiration and watch for emotional distress

**TIPS ON WORK SIMPLIFICATION AND ENERGY CONSERVATION: Rest breaks throughout the day, plan, delegate or omit part of an activity, change body position when performing certain activities and identify ergonomically poor work heights.

References :

  • The Benefits of Group Occupational Therapy for Patients with Parkinson’s Disease. American Journal of Occupational Therapy, June 1987, Vol. 41, 360-365. doi:10.5014/ajot.41.6.360
  • Cooper, C. (2007) Fundamentals of Hand Therapy: Clinical Reasoning and Treatment Guidelines for Common Diagnoses of the Upper Extremity. St. Louis, MO: Mosby/Elsevier.
  • Physical Dysfunction Practice Skills for the Occupational Therapy Assistant, 3rd Edition – Mary Beth Early
  • Quick Reference to Occupational Therapy, 2nd Edition – Kathlyn L. Reed
  • Myasthenia Gravis
  • Parkinsonism
  • Guillain-Barre Syndrome
  • Dysphagia and multiple sclerosis 
  • Ventilatory control in ALS
  • Exercise is effective for delaying respiratory insufficiency in ALS patients (Aboussouan 2009, de Almeida et al. 2012)