What is the optimal first‑line medication for anxiety in a patient with Duchenne/Becker muscular dystrophy who has cardiomyopathy and chronic respiratory muscle weakness?

Managing Anxiety in Muscular Dystrophy Patients

For patients with Duchenne or Becker muscular dystrophy experiencing anxiety, benzodiazepines are the recommended first-line agents, with careful attention to avoiding contraindicated anesthetics and ensuring appropriate cardiac and respiratory monitoring. 1

Primary Pharmacological Approach

Benzodiazepines are the established anxiolytic agents for DMD patients, particularly those with advanced disease and cardiopulmonary complications. 1 A retrospective study of 11 DMD patients with severe congestive heart failure demonstrated that benzodiazepines successfully treated unrest and anxiety in all cases. 1 When benzodiazepines alone are insufficient, haloperidol can be added as an adjunctive agent for more severe agitation. 1

Key Considerations for Anxiolytic Selection

• Avoid all inhaled anesthetic agents (halothane, isoflurane, sevoflurane) as they pose extreme risk of hyperthermic events, rhabdomyolysis, hyperkalemia, and sudden cardiac death in DMD patients. 2
• Succinylcholine is absolutely contraindicated due to risk of acute rhabdomyolysis, hyperkalemia, and cardiac arrest from disruption of unstable cell membranes. 2
• Procedural sedation requires anesthesiologist supervision with full monitoring capabilities and ICU availability for post-procedure management. 2

Critical Safety Framework

Cardiac Monitoring Requirements

Given the universal presence of cardiomyopathy in DMD/BMD patients, anxiety management must account for cardiac vulnerability:

• Continuous SpO2 and blood pressure monitoring should be standard during any sedation or anxiolytic administration. 2
• Four-chamber dilation and reduced left ventricular function develop independently of skeletal muscle severity, requiring cardiac assessment before initiating any sedating medications. 3
• Arrhythmias affecting atrial and ventricular rhythms may be life-threatening and can be exacerbated by anxiety or its treatment. 3

Respiratory Considerations

The respiratory muscle weakness inherent to DMD creates additional complexity:

• Measure FVC, MIP, MEP, PCF, and SpO2 before any procedural sedation or when initiating anxiolytics that may cause respiratory depression. 2
• If SpO2 is ≤95% in room air, measure blood or end-tidal CO2 levels to assess for chronic hypoventilation. 2
• Patients with FVC <50% predicted are at increased risk for respiratory complications from sedating medications. 2

Multidisciplinary Context

Baseline Anxiety Prevalence

Anxiety is highly prevalent in DMD populations, affecting 29.3% of patients in surveillance data, with higher frequencies among non-ambulatory males compared to ambulatory patients. 4 This underscores the importance of:

• Screening emotional status at every clinic visit using standardized rating scales. 5
• Comprehensive neuropsychological assessment at diagnosis and prior to formal schooling. 5
• Proactive behavioral health referral when anxiety symptoms emerge or worsen. 2

Glucocorticoid-Related Behavioral Effects

Since most DMD patients receive chronic glucocorticoid therapy, anxiety management must account for steroid-induced behavioral changes:

• Establish baseline mood and temperament before initiating glucocorticoids to distinguish disease-related from medication-related anxiety. 5
• Consider timing of glucocorticoid dosing (later in day) if behavioral issues emerge. 5
• Deflazacort may be preferred over prednisone when pre-existing behavioral issues are present. 2, 5

Common Pitfalls to Avoid

• Never use inhaled anesthetics or succinylcholine even for brief procedures, as sudden death has been reported in previously undiagnosed DMD cases. 2
• Do not assume cardiac function parallels skeletal muscle severity—cardiomyopathy assessment is mandatory before sedation. 3
• Avoid underestimating respiratory compromise—relative physical inactivity masks the degree of cardiopulmonary dysfunction. 3, 6
• Do not initiate anxiolytics without establishing monitoring protocols, particularly for non-ambulatory patients with advanced disease. 2

Practical Implementation Algorithm

1. Screen for anxiety using standardized scales at routine clinic visits (every 6 months). 5
2. Assess cardiac function with ECG and echocardiography before initiating anxiolytic therapy. 3, 6
3. Measure baseline pulmonary function (FVC, SpO2) to stratify respiratory risk. 2
4. Initiate benzodiazepines at conservative doses with close monitoring for respiratory depression. 1
5. Add haloperidol if benzodiazepines alone are insufficient for severe agitation. 1
6. Ensure families have steroid emergency card listing considerations for acute illness or procedures requiring sedation. 5
7. Coordinate with anesthesiology for any procedural sedation, emphasizing contraindications to inhaled agents and succinylcholine. 2