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Physiotherapy Assessment and Management of Myasthenia Gravis (MG)
Overview of the Condition
Myasthenia gravis (MG) is an autoimmune neuromuscular junction (NMJ) disorder characterized by fatigable, fluctuating skeletal muscle weakness. Antibodies — most commonly against acetylcholine receptors (AChR) (~85%), and less commonly against muscle-specific kinase (MuSK) or LRP4 — attack postsynaptic NMJ proteins, reducing the number of functional AChRs and impairing neuromuscular transmission (Harrison's Principles of Internal Medicine, 21st ed., p. 12744).
Harrison's (p. 12744): Synaptic transmission at the NMJ showing ACh vesicle release, SNARE proteins, AChE breakdown, and the autoimmune attack targets relevant to MG.
MGFA Classification (Myasthenia Gravis Foundation of America)
Understanding disease severity guides physiotherapy goals and intensity.
| Class | Description |
|---|
| I | Ocular muscle weakness only; all other muscles normal |
| IIa | Mild weakness affecting limb/axial muscles; ocular may also be involved |
| IIb | Mild weakness predominantly affecting oropharyngeal/respiratory muscles |
| IIIa | Moderate limb/axial muscle weakness |
| IIIb | Moderate oropharyngeal/respiratory muscle weakness |
| IVa | Severe limb/axial weakness |
| IVb | Severe oropharyngeal/respiratory weakness |
| V | Intubation with or without mechanical ventilation (myasthenic crisis) |
Physiotherapy Assessment
A thorough, systematic assessment is essential before any treatment is initiated, as inappropriate exercise can precipitate a myasthenic crisis.
1. Subjective Assessment (History)
- Chief complaint — pattern of weakness, fatigability, time of day variation (worse toward end of day or after sustained activity)
- Symptom onset and progression — ocular symptoms (ptosis, diplopia) often precede generalized weakness
- Functional impact — activities of daily living (ADL), work, mobility, stairs, overhead activities
- Medications — pyridostigmine dose and timing, corticosteroids, immunosuppressants (azathioprine, efgartigimod alfa-fcab, mycophenolate); note that medications affect exercise tolerance (Adult Strabismus, p. 66)
- Recent exacerbations — infections, stress, medications that worsen MG (aminoglycosides, beta-blockers, fluoroquinolones)
- Thymectomy history — post-surgical respiratory and functional status
- Sleep quality — hypersomnia, waking with dyspnea (nocturnal hypoventilation)
- Bulbar symptoms — dysphagia, dysarthria, nasal regurgitation (risk of aspiration)
- Red flags — increasing dyspnea, difficulty swallowing saliva, inability to clear secretions (signs of impending crisis)
2. Objective Assessment
A. Muscle Strength and Fatigability Testing
The hallmark of MG is fatigable weakness — strength declines with sustained or repeated contraction and recovers with rest.
| Test | Method | Positive Finding |
|---|
| Sustained upgaze test | Patient looks upward for 2 minutes | Progressive ptosis developing |
| Ice pack test | Ice applied to closed eyelid for 2 min | Improvement in ptosis (cold inhibits AChE, increases ACh) |
| Arm abduction test | Arms held at 90° abduction | Fatigue/dropping within 1–2 min |
| Repetitive counting/speech | Patient counts to 50 or reads aloud | Progressive hypophonia/dysarthria |
| Manual Muscle Testing (MMT) | MRC 0–5 scale, all major muscle groups | Graded weakness; note disproportionate proximal > distal pattern |
| Hand grip dynamometry | Repeated grip at set intervals | Declining force with repetition |
| Timed Up and Go (TUG) | 3-metre walk test | Slowness, instability |
| 6-Minute Walk Test (6MWT) | Functional exercise capacity | Reduced distance; monitor for fatigability |
Muscles to prioritize in MMT:
- Ocular (CN III, IV, VI — via clinical observation)
- Facial (orbicularis oculi, frontalis)
- Neck flexors and extensors (dropped head syndrome in severe MG)
- Deltoids, biceps, triceps (proximal > distal)
- Hip flexors, quadriceps
- Diaphragm and intercostals (via respiratory function tests)
B. Respiratory Assessment
Respiratory muscle weakness is the most life-threatening feature of MG. Physiotherapists must monitor this carefully and serially.
| Parameter | Tool | Threshold for Concern |
|---|
| Forced Vital Capacity (FVC) | Spirometry | <20 mL/kg or <1.0 L → consider ICU monitoring; <15 mL/kg → intubation risk |
| Negative Inspiratory Force (NIF/MIP) | Manometer | Less negative than −25 cmH₂O → significant weakness |
| Sniff Nasal Inspiratory Pressure (SNIP) | Occlude one nostril; sharp sniff | <−70 cmH₂O (M) / <−60 cmH₂O (F) suggests clinically significant diaphragm weakness (Botulism Guidelines, p. 18) |
| Single Breath Count Test | Deep breath → count at 2/second | <25 correlates with abnormal FVC (~116 mL per counted number) (Botulism Guidelines, p. 18) |
| Peak Cough Flow (PCF) | Peak flow meter during cough | <160 L/min = ineffective cough; <270 L/min = at risk |
| SpO₂ | Pulse oximetry | Late indicator — normal SpO₂ does not exclude impending failure |
| EtCO₂ | Capnography | Rising CO₂ is an early predictor of ventilatory failure (Botulism Guidelines, p. 18) |
| Respiratory rate, pattern | Observation | Paradoxical abdominal movement, use of accessory muscles |
Key clinical pearl: In neuromuscular respiratory failure (as in MG and GBS), hypoxia and hypercapnia are late signs — SpO₂ may remain normal until respiratory failure is advanced. Serial FVC and NIF ("20/30 rule": FVC <20 mL/kg or NIF less negative than −30 cmH₂O) are the primary monitoring tools.
C. Functional and Activity Assessment
| Domain | Tools |
|---|
| ADL independence | Barthel Index, FIM (Functional Independence Measure) |
| Fatigue severity | Fatigue Severity Scale (FSS), MG-specific Quality of Life (MG-QOL15) |
| Balance and falls risk | Berg Balance Scale, single-leg stance, functional reach |
| Gait analysis | Observational gait analysis, cadence, step length, use of aids |
| Dysphagia screen | 3-oz water swallow test (refer to SLT if positive) |
| Posture | Cervical/thoracic alignment, dropped head, forward head posture |
| Exercise tolerance | 6MWT, CPET (if stable), Borg RPE and dyspnea scales during activity |
D. Pain and Musculoskeletal Assessment
- Chronic MG and corticosteroid use leads to osteoporosis, proximal myopathy, and joint pain — assess these as secondary impairments
- Screen for contractures or disuse atrophy in patients with severe or prolonged disease
- Postural pain secondary to weakness compensation patterns
Physiotherapy Management
MG management is collaborative — physiotherapy works alongside neurology (pyridostigmine, immunosuppression, thymectomy) and aims to optimize function, prevent complications, and safely maintain or improve physical capacity.
Core Principles
- Fatigue is the enemy — all exercise must respect the fatigable nature of MG; "no pain, no gain" does NOT apply
- Timing around medication — exercise should be scheduled 30–60 minutes after pyridostigmine when cholinergic effect is at its peak
- Activity pacing — distribute activities across the day; avoid prolonged sustained contractions
- Monitor for crisis signs — any session showing increasing dyspnea, dysphagia, or rapidly worsening weakness must stop immediately
- Disease phase dictates intensity — stable/remission MG tolerates more aggressive rehabilitation; active/fluctuating MG requires conservative management
1. Respiratory Physiotherapy
This is the highest priority in MG management, particularly in classes IIb–V.
A. Breathing Exercises
- Diaphragmatic breathing — hands on abdomen, focus on diaphragmatic excursion; improves respiratory efficiency
- Segmental breathing — lateral costal and posterior basal breathing exercises to maintain lung volumes
- Incentive spirometry — sustain alveolar recruitment; set targets based on current FVC
- Glossopharyngeal breathing (GPB) — technique where patients use pharyngeal muscles to augment tidal volume; useful in severe diaphragm weakness
B. Secretion Clearance
- Assisted cough / manually assisted cough — therapist applies abdominal thrust timed to the patient's cough effort when PCF <270 L/min
- Mechanical insufflation-exsufflation (MI-E / CoughAssist) — delivers positive pressure breath then rapidly reverses to negative pressure, simulating a cough; indicated when PCF <160 L/min (ineffective cough)
- Active cycle of breathing technique (ACBT) — breathing control → thoracic expansion exercises → forced expiration technique (FET/huffing)
- Postural drainage — positional drainage to facilitate secretion movement in patients with concurrent chest infection or weak cough
C. Respiratory Muscle Training
- Inspiratory Muscle Training (IMT) with a threshold IMT device (e.g., Threshold IMT, POWERbreathe) — sets at 30–50% of MIP; sessions of 15–20 min, 5× weekly
- Evidence in neuromuscular diseases supports improvements in MIP, FVC, and quality of life with IMT; start conservatively and progress based on tolerance
D. Non-Invasive Ventilation (NIV) Support
- Physiotherapists assist with BiPAP interface fitting, education, and tolerance training
- NIV is indicated for nocturnal hypoventilation (rising CO₂, morning headaches, poor sleep)
- During myasthenic crisis, physiotherapy supports ICU team with secretion management, positioning, and early mobilization post-extubation
2. Exercise Prescription
Principles
- Begin with low intensity, short duration, frequent rest periods
- Submaximal exercise targeting 50–70% of heart rate reserve or RPE 11–13 (Borg 6–20 scale)
- Prioritize endurance over strength training initially
- Avoid isometric holds and Valsalva maneuver (raises intrathoracic pressure, worsens venous return, can precipitate crisis)
- Aquatic therapy is particularly well-tolerated — buoyancy reduces effort, cool water may reduce fatigability
Progressive Resistance Training
- Low load (40–60% 1RM), high repetition (15–20 reps) with adequate rest between sets (2–3 min)
- Focus on functional muscle groups: hip flexors, quadriceps, shoulder girdle, cervical extensors (for dropped head)
- Avoid exercising to failure
Aerobic Exercise
- Walking, cycling, swimming at moderate intensity
- Interval training may be better tolerated than continuous activity (e.g., 2 min walk / 2 min rest)
- Progress duration before intensity
Sample Weekly Exercise Framework (Stable MG)
| Day | Activity | Duration |
|---|
| Monday | Aerobic (walking/cycling) + breathing exercises | 20–30 min |
| Tuesday | IMT + gentle stretching | 15 min |
| Wednesday | Resistance training (lower limb focus) | 20 min |
| Thursday | Rest or gentle ADL focus | — |
| Friday | Aerobic + upper limb resistance | 20–25 min |
| Saturday | Functional activities / balance training | 15–20 min |
| Sunday | Rest | — |
3. Fatigue Management and Energy Conservation
- Activity pacing — prioritize high-demand tasks early in the day when energy is highest
- Rest scheduling — structured rest periods (10–20 min) between activities
- Environmental modifications — ergonomic aids, perching stools, grab rails, raised toilet seats
- Assistive devices — rollators, grab rails, orthotics for foot drop if present
- Thermal precautions — heat worsens NMJ transmission; advise cool environments, avoid hot baths/showers, use cooling vests during exercise in warm weather
- Cognitive fatigue — MG fatigue is neuromuscular, not purely central, but mental exertion can worsen perceived fatigue; address with scheduling and prioritization strategies
4. Posture and Orthotic Management
- Cervical collar / head support — for dropped head syndrome (bilateral neck extensor weakness); helps maintain neutral head position during activities
- Postural taping / kinesiotaping — scapular retraction taping to offload fatigued trapezius
- AFO (ankle-foot orthosis) — if distal lower limb weakness causes foot drop (less common in MG, more relevant in MuSK-antibody positive patients)
- Eyelid crutch — lid crutch attached to glasses frame for ptosis (in consultation with ophthalmology)
- Postural exercises — thoracic extension, scapular stabilization, chin tucks; must be paced appropriately
5. Balance and Falls Prevention
- MG patients have elevated falls risk due to proximal limb and neck weakness, visual disturbance (diplopia, ptosis), and medication side effects (corticosteroid myopathy, sedation)
- Static balance training: single-leg stance (with support), tandem stance
- Dynamic balance training: stepping tasks, obstacle courses, Tai Chi (modified)
- Proprioceptive training: balance board (supervised, low intensity)
- Gait retraining: address wide-base gait, steppage, cadence irregularities
- Environmental hazard assessment: home modification referral
6. Management During and After Myasthenic Crisis
During Crisis (ICU phase)
- Positioning — 30–45° head elevation to optimize diaphragm mechanics and reduce aspiration risk
- Passive/active-assisted range of motion (ROM) — prevent contractures and DVT
- Chest physiotherapy — secretion management via MI-E, suctioning support, assisted cough
- Monitoring — serial FVC, NIF, EtCO₂; communicate with ICU team
- DVT prevention — early passive mobilization, compression, positioning
Post-Crisis Rehabilitation
- Begin with bed mobility → sitting balance → transfers → standing
- Progressive mobilization as FVC improves (target >20 mL/kg before increasing activity demands)
- ICU-acquired weakness (ICUAW) may superimpose on MG weakness — distinguish via serial assessment
- Rebuild respiratory endurance before cardiovascular endurance
7. Patient and Carer Education
- Understanding the disease — fluctuating nature, factors that worsen MG (infections, overexertion, heat, certain medications)
- Warning signs of crisis — increasing breathlessness, difficulty swallowing secretions, rapid fatigue of speech → seek emergency care
- Exercise diary — document symptoms, fatigue levels, and exercise tolerance to guide progression
- Medication timing — reinforce exercising 30–60 min post-pyridostigmine
- Avoid triggers — emotional stress, extreme temperatures, systemic illness (vaccinate against influenza/pneumococcus), contraindicated medications
- Community resources — Myasthenia Gravis Foundation support groups, vocational rehabilitation
Outcome Measures Summary
| Domain | Measure |
|---|
| Disease severity | MGFA Classification, MG Composite Score |
| Patient-reported outcomes | MG-QOL15, Fatigue Severity Scale (FSS) |
| Muscle strength | MMT (MRC Scale), Hand Grip Dynamometry |
| Respiratory | FVC, MIP/NIF, SNIP, Single Breath Count, PCF |
| Functional mobility | TUG, 6MWT, Barthel Index, FIM |
| Balance | Berg Balance Scale, Functional Reach Test |
| Exercise tolerance | Borg RPE, 6MWT distance, HR response |
Key Precautions and Contraindications
| Precaution | Rationale |
|---|
| Exercise in heat / hot environment | Heat impairs NMJ transmission; can precipitate crisis |
| High-intensity/exhaustive exercise | May trigger exacerbation or crisis |
| Isometric exercise to failure | Rapid fatigue, Valsalva effect |
| Exercise during systemic illness | Infection is a major crisis trigger |
| Bulbar symptoms present | High aspiration risk; avoid supine exercise, ensure swallow assessment |
| Unoptimized medication | Do not commence exercise rehab until pyridostigmine dose is stabilized |
| FVC <20 mL/kg | Defer all non-respiratory physiotherapy; focus on respiratory monitoring and positioning |
Summary Framework
MG Physiotherapy Approach
├── ASSESS
│ ├── Subjective: fatigue pattern, medications, bulbar sx, crisis Hx
│ ├── Respiratory: FVC, NIF, SNIP, single breath count, cough
│ ├── Strength: MMT, dynamometry, fatigability tests
│ └── Function: TUG, 6MWT, balance, ADLs
│
├── PLAN (based on MGFA class + stability)
│ ├── Stable MG (I–IIa): full rehab, exercise + pacing
│ ├── Moderate MG (IIb–III): respiratory focus, conservative exercise
│ └── Severe/Crisis (IV–V): ICU chest PT, passive ROM, positioning
│
└── TREAT
├── Respiratory: breathing Rx, IMT, secretion clearance, NIV support
├── Exercise: submaximal aerobic + resistance, aquatic therapy
├── Fatigue: pacing, energy conservation, thermal precautions
├── Posture: orthoses, taping, postural exercise
├── Balance: falls prevention, gait retraining
└── Education: crisis recognition, exercise timing, triggers
Physiotherapy in MG is not about aggressive strengthening but about intelligent, paced, timed, and monitored rehabilitation that respects the fatigable nature of the disease. The respiratory system must always be the primary concern, and every physiotherapist managing MG patients should be competent in recognizing the early signs of myasthenic crisis.