Medical ConditionsBehavior & neurodevelopment: Muscular dystrophies
Medical ConditionsBehavior & neurodevelopment: Muscular dystrophies
Muscular dystrophies
Overview
Board takeaway: Identify the dystrophy subtype and assess respiratory reserve, cardiomyopathy, swallowing, and anesthesia-specific risks.
How do the major muscular dystrophies differ?
Muscular dystrophies result from pathogenic variants affecting muscle structure or function.
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| Pattern | Distinguishing features |
|---|---|
| Duchenne and Becker | X-linked dystrophin disorders; Duchenne generally begins earlier and progresses faster |
| Myotonic | Myotonia with difficulty relaxing muscles, plus systemic disease |
| Congenital | Early-onset forms with variable severity and molecular cause |
| Limb-girdle | Predominant shoulder and pelvic-girdle weakness; multiple genetic causes |
| Other forms | Distribution, conduction disease, and bulbar involvement vary by subtype |
Which systemic findings change dental risk in muscular dystrophy?
Respiratory muscle weakness, hypoventilation, ineffective cough, cardiomyopathy or arrhythmia, scoliosis, dysphagia, and fatigue are important. Steroid or antiresorptive treatment may add adrenal or bone-health considerations. Normal appearance at rest does not establish adequate respiratory reserve for sedation.
What oral findings occur in muscular dystrophies?
Weak oral muscles can cause poor clearance, drooling, chewing fatigue, limited opening, malocclusion, open bite or crossbite, and difficulty maintaining hygiene. Tongue enlargement and developmental dental anomalies are reported in some forms. Myotonic disease can involve substantial masticatory and facial weakness.
How should routine dental care be adapted for muscular dystrophy?
Use comfortable supported positioning, allow breaks, control water and debris, and provide caregiver-assisted hygiene where needed. Assess dysphagia and aspiration history. Coordinate significant cardiac or respiratory disease and avoid independent sedative or opioid prescribing without considering ventilatory risk. Detailed Duchenne management is covered in the linked condition note.
What changes the anesthesia plan for muscular dystrophy?
Obtain subtype-specific neuromuscular, cardiac, pulmonary, and anesthesia assessment. In Duchenne or Becker disease, succinylcholine can cause life-threatening hyperkalemia; volatile anesthetics may cause rhabdomyolysis and require specialist avoidance or risk assessment. This is distinct from simply labeling all muscular dystrophies malignant-hyperthermia susceptible. Plan postoperative ventilation and any established steroid coverage.
What treatment and prognosis apply to muscular dystrophies?
Medical therapies, respiratory support, cardiac care, rehabilitation, and mobility aids are subtype-specific. Disease-modifying treatment eligibility is a specialist decision. Progression and lifespan differ substantially, so one Duchenne survival estimate should not describe every muscular dystrophy.