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Lower Limb Exercise Protocol for Pelvic/Limb-Girdle, Facioscapulohumeral (FSHD), and Becker Muscular Dystrophies

Core Principles Across All Three Conditions

1. Submaximal effort only - high-intensity or maximal resistance exercises risk "overwork weakness," a well-documented phenomenon where a muscle permanently loses function after excessive loading
2. Avoid eccentric contractions (the lengthening phase under load, e.g., slowly lowering a heavy weight) - these produce the greatest muscle fiber damage in dystrophic muscle
3. Avoid prolonged bed rest and inactivity - deconditioning accelerates functional decline faster than the disease itself
4. No high-resistance exercise - keep loads below 30% of 1-repetition maximum (1RM) for isolated movements
5. Warning signs for stopping: dark urine (myoglobinuria), muscle cramping lasting >24 hours, or feeling weaker the day after exercise - if any occur, reduce intensity
6. Obesity prevention - excess body weight overloads already weakened lower limb muscles

• Adams and Victor's Principles of Neurology, 12th Ed.
• Miller's Review of Orthopaedics, 9th Ed.

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1. Pelvic/Limb-Girdle Muscular Dystrophy (LGMD)

Profile

• Primarily affects pelvic girdle (hip flexors, extensors, abductors) and proximal lower limb muscles
• Falls risk is high; functional decline targets sit-to-stand and stair climbing
• Eccentric contractions must be especially avoided; dysferlinopathy subtype (LGMD R2) can mimic polymyositis and steroids should be avoided

Phase 1 - Foundation (Weeks 1-4)

Phase 2 - Progressive Resistance (Weeks 5-12)

Aerobic Component (all phases)

• Stationary cycling (no/low resistance): 10-20 min, 3x/week - ideal for cardiovascular health without eccentric loading
• Aquatic exercise/walking: preferred if osteoporosis risk exists; buoyancy reduces muscle load significantly
• Target perceived exertion: 11-13/20 on Borg scale (light to somewhat hard)

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2. Facioscapulohumeral Muscular Dystrophy (FSHD)

Profile

• Primarily descends craniocaudally; significant lower limb involvement includes hip girdle, tibialis anterior (footdrop risk), and peroneal weakness
• 50% of adults retain ambulatory function
• Footdrop is a major fall risk; ankle stretching and AFO use are important adjuncts

Stretching Protocol (minimum 4-6x/week, hold 30 seconds each)

Lower Limb Strengthening (2-3x/week)

Aerobic Component

• Swimming/pool exercise: optimal choice - non-weight-bearing, no eccentric loading, cardiovascular benefit
• Stationary bike: 15-20 min at light resistance, 3-5x/week
• Avoid high-resistance strength training (>30% 1RM)
• FSHD Society guidelines

Coordination Activities (lower limb focus)

• Shuffling and stepping over obstacles
• Step-tap sequences
• Balance training on stable surfaces (avoid unstable surfaces if footdrop present)

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3. Becker Muscular Dystrophy (BMD)

Profile

• Dystrophinopathy (same gene as Duchenne, but partial protein function retained)
• Later onset, milder course; many adults remain ambulatory
• Exercise intolerance, myalgias, and cramps after exertion are common even in mild cases
• Cardiomyopathy is a major comorbidity - cardiac clearance required before aerobic training
• Isometric contractions are the least damaging type

Lower Limb Strengthening (2x/week, supervised)

Aerobic Component

• Stationary cycling (no resistance): excellent option; avoids eccentric loading and is easily adjustable
• Walking (short distances): preferred when ambulatory; have a spotter available
• Swimming/water exercise: kickboard, water walking, swimming
• Breathing exercises: relevant given potential respiratory involvement in advanced BMD
• Yoga/stretching: improves flexibility and relaxation
• iNMD Exercise Guidelines; PM&R KnowledgeNow

Key Precautions for BMD

• Cardiac evaluation before initiating any aerobic program (cardiomyopathy risk)
• Myalgias lasting >24 hours = reduce load by 20% at next session
• Dark urine after exercise = stop all exercise and seek medical review

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Evidence-Based Resistance Training Protocol (Applicable to All Three)

• Frequency: 2 supervised sessions/week for 12 weeks
• Warm-up: 5 min (step-up on a box)
• Lower limb exercises included: seated knee extension, seated knee flexion, standing hip extension, standing hip abduction, squat, step-up
• Sets/reps: 2 sets x 10RM (weeks 1-3), increasing to 3 sets x 10RM after 3 weeks
• Tempo: 2 seconds concentric : 4 seconds eccentric (note: some eccentric allowed at submaximal loads in supervised settings)
• Rest: 1 minute between sets
• Load adjustment: every 3 weeks if participant can complete 2 reps above target
• Modification: if full range of motion not possible, use manual assistance, resistance bands, or gravity-neutral/side-lying position
• Outcomes: 13% increase in knee flexor MVC torque; significant improvements in sit-to-stand, stair ascent, and stair descent times (p<0.05)

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Summary: Protocol Comparison at a Glance

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Important Clinical Notes

• Overwork weakness is the primary risk - if the patient feels weaker over days/weeks despite exercising, reduce volume immediately
• Passive stretching (20-30 repetitions/day to affected muscle groups) delays contracture formation in all three conditions - Adams and Victor's Principles of Neurology
• Night splinting can supplement stretching for ankle/plantar flexor contractures (especially in FSHD with footdrop)
• Orthotics (AFO): consider for footdrop in FSHD and later-stage BMD to optimize gait and reduce fall risk
• Maximal resistance exercises begun early in the disease course can strengthen muscles in LGMD and FSHD (Vignos data), but timing matters - once significant weakness has set in, the threshold drops

Recent evidence note: A 2022 RCT (PMID 35443813) on aerobic training in Duchenne MD showed motor function improvements, and a 2021 RCT (PMID 34233722) demonstrated ambulatory gains with wearable exoskeleton (HAL) in slowly progressive neuromuscular diseases including Becker-type dystrophies. These support active rehabilitation even in progressive disease.

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Lower Limb Exercise Protocol

Pelvic/Limb-Girdle MD (LGMD) | Facioscapulohumeral MD (FSHD) | Becker MD (BMD)

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Understanding the Weakness Patterns

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Universal Principles for All Three Conditions

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SECTION 1: Pelvic / Limb-Girdle Muscular Dystrophy (LGMD)

Clinical Profile for Exercise Planning

• Weakness is symmetric and proximal - primarily hip flexors, extensors, abductors, and knee extensors
• Falls and difficulty rising from chairs/floor are the primary functional deficits
• Sarcoglycanopathies (LGMD R3-R6) resemble Duchenne in severity; dysferlinopathy (LGMD R2) - eccentric exercise must be strictly avoided
• Stationary cycling 3x/week for 30 min has been shown to improve functional capacity (sit-to-stand, rise from floor) without elevating CK or worsening muscle morphology (Sveen et al.; Vissing et al., 10-week home ergometer program)

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LGMD Stretching Protocol

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LGMD Strengthening Protocol

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LGMD Aerobic Protocol

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SECTION 2: Facioscapulohumeral Muscular Dystrophy (FSHD)

Clinical Profile for Exercise Planning

• Descends craniocaudally: face → shoulder → abdominal/trunk → hip girdle → tibialis anterior (footdrop)
• Increased lumbar lordosis is common (see clinical photo in Campbell's 15th Ed, Fig. 37.18)
• Footdrop from tibialis anterior weakness is a major fall risk - ankle dorsiflexion exercises and AFO assessment are essential
• Approximately 50% of adults remain ambulatory; exercise goals differ significantly between ambulatory and non-ambulatory patients
• HHS guidelines endorsed by FSHD Society: ~150 min/week moderate aerobic exercise; strengthening 2-3 days/week

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FSHD Stretching Protocol

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FSHD Lower Limb Strengthening Protocol

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FSHD Aerobic and Balance Protocol

• Swimming/aquatic exercise: the single best aerobic mode for FSHD - non-weight-bearing, no eccentric loading, whole-body cardiovascular benefit. Kickboard walking, water walking, gentle swimming
• Stationary cycling (light resistance): 15-20 min at Borg RPE 11-13, 3-5x/week
• Balance training: on stable flat surface (avoid unstable surfaces if footdrop present); single-leg stance with wall support; shuffle patterns on floor; stepping over low obstacles
• Yoga/Pilates: gentle, low-load flexibility work is endorsed by FSHD Society; improves lumbar lordosis and hip mobility
• FSHD Society PT Guidelines 2025 (ENMC Standards endorsed)

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FSHD Orthotic Consideration

• Ankle-foot orthosis (AFO): should be assessed for all patients with footdrop (tibialis anterior weakness); reduces fall risk and improves gait efficiency
• Night splinting for plantar flexor contracture prevention

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SECTION 3: Becker Muscular Dystrophy (BMD)

Clinical Profile for Exercise Planning

• Partial dystrophin function retained; milder and later-onset than Duchenne
• Proximal lower limb weakness (hip flexors, quadriceps, gluteals) is primary
• Exercise intolerance, myalgias, and post-exertional cramps are common even in mild disease - Adams and Victor's, 12th Ed.
• Cardiomyopathy is a major comorbidity and must be evaluated before starting any aerobic program
• Many adults remain ambulatory; isometric contractions are the least damaging and are preferred in early strengthening

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BMD Stretching Protocol

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BMD Strengthening Protocol

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BMD Aerobic Protocol

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Consolidated Comparison Table

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Evidence Base

• Bostock et al. (2019, Frontiers in Neurology) - RCT in exactly these three conditions (FSHD n=6, LGMD n=6, BMD n=5): 12-week twice-weekly supervised resistance training produced 13% increase in knee flexor torque and significant improvements in sit-to-stand, stair ascent, and stair descent times (p<0.05). Protocol: 2 sets x 10RM → 3 sets x 10RM at week 3; exercises included seated knee extension/flexion, hip extension, hip abduction, squat, and step-up; 2 sec concentric : 4 sec eccentric tempo; 1 min rest between sets.
• Vissing et al. / Sveen et al. - Stationary cycling ergometer (3x/week, 30 min) in LGMD improved functional capacity and aerobic fitness without CK elevation or muscle damage on biopsy.
• FSHD Society / ENMC Standards (2025 edition) - aerobic 150 min/week moderate intensity; strengthening 2-3x/week; stretching daily; high-resistance and eccentric exercise avoided.
• Adams and Victor's Principles of Neurology, 12th Ed. - "Maximal resistance exercises, if begun early, can strengthen muscles in Duchenne, limb-girdle, and facioscapulohumeral dystrophies... none of the muscles was weaker at the end of a year than at the beginning."
• PMC scoping review 2025 (PMID: PMC11708444) - confirms aerobic exercise at moderate intensity (<70% maximal aerobic capacity) is the most feasible and safe option across LGMD subtypes; eccentric and high-resistance training should be avoided.

Clinical reminder: All exercise programs for these conditions should begin with a baseline assessment of strength (manual muscle testing or dynamometry), functional tasks (timed sit-to-stand, 6-minute walk test), and a daily symptom log. Programs must be individually tailored and monitored by a trained physiotherapist familiar with neuromuscular disease. Persistent post-exercise soreness beyond 24 hours signals overtraining and requires load reduction.