Medication Selection for Muscle Spasms in Hypermobile Ehlers-Danlos Syndrome
Neither baclofen nor Robaxin (methocarbamol) should be used as primary treatment for muscle spasms in hypermobile Ehlers-Danlos syndrome (hEDS), as muscle relaxants are not recommended and physical therapy with low-resistance exercise is the cornerstone of musculoskeletal symptom management. 1, 2
Why Muscle Relaxants Are Not Appropriate
FDA-Approved Indications Don't Match hEDS Pathophysiology
- Baclofen is FDA-approved only for spasticity from multiple sclerosis and spinal cord diseases, not for musculoskeletal pain or the type of muscle dysfunction seen in hEDS 3
- Methocarbamol is indicated only for acute, painful musculoskeletal conditions as an adjunct to rest and physical therapy, and it does not directly relax tense skeletal muscles 4
- The muscle "spasms" in hEDS are fundamentally different from true spasticity—they represent compensatory muscle overactivity attempting to stabilize hypermobile joints, not primary muscle pathology 1, 5
Guideline-Based Contraindications
- The American Academy of Pain Medicine explicitly recommends avoiding opioids in hEDS patients with chronic pain, and this caution extends to centrally-acting muscle relaxants that can worsen outcomes 2
- Up to 98% of hEDS patients have gastrointestinal manifestations, and both baclofen and methocarbamol can cause sedation and worsen GI symptoms including nausea and constipation 6, 2
The Evidence-Based Alternative: Physical Therapy First
Primary Treatment Approach
- The American College of Rheumatology recommends initiating low-resistance exercise and physical therapy as the cornerstone of treatment for musculoskeletal pain in hEDS 1
- Physical therapy should focus on increasing muscle tone through therapeutic exercise and motor function training to improve joint stability, not on relaxing muscles 1
- Myofascial release techniques combined with low-resistance exercise are specifically recommended during acute flares 2
Why This Works Better
- The goal in hEDS is to strengthen periarticular muscles to compensate for ligamentous laxity, not to relax them 1, 5
- Occupational therapy and bracing were the most effective treatment options in a retrospective study of 98 EDS patients, with 70% reporting improvement 5
- Swimming and low-impact weight-bearing exercise are preferred activities that improve muscle tone without excessive joint stress 1
When Pharmacotherapy Is Considered
Neuropathic Pain Modulators (If Pain Predominates)
- If the "spasms" are actually neuropathic pain or central sensitization, neuropathic modulators may be considered, though they were the least well-tolerated option in one study with 47% reporting adverse effects 5
- This should only be attempted after physical therapy has been optimized and under multidisciplinary guidance 1, 7
Critical Comorbidities to Address First
- Screen for POTS (postural orthostatic tachycardia syndrome) by measuring postural vital signs with heart rate increase ≥30 beats/min within 10 minutes of standing, as autonomic dysfunction affects up to 37.5% of hEDS patients and can cause muscle symptoms 6, 1
- Test for celiac disease earlier in hEDS patients with any GI symptoms, as the risk is elevated and may contribute to fatigue and muscle weakness 8
- Check vitamin D levels and supplement if deficient, as this is common and contributes to musculoskeletal symptoms 1
Multidisciplinary Coordination Algorithm
Step 1: Confirm Diagnosis and Assess Severity
- Apply the 2017 diagnostic criteria for hEDS using the Beighton score (≥5/9 for adults under 50) 8
- Perform echocardiography to evaluate aortic root diameter, as dilation occurs in 25-33% of hEDS cases 8, 1
Step 2: Initiate Physical Therapy
- Refer to physical therapy with specific instructions for low-resistance strengthening exercises focusing on joint stabilization 1, 2
- Consider occupational therapy for bracing and activity modification 5
Step 3: Screen for Treatable Comorbidities
- Measure postural vital signs for POTS 6, 1
- Check celiac serology if any GI symptoms present 8
- Obtain vitamin D level and supplement if <30 ng/mL 1
Step 4: Psychological Support
- Provide or refer for psychological support and brain-gut behavioral therapies, as anxiety and psychological distress are common and significantly impact quality of life 1, 2
Step 5: Reassess After 8-12 Weeks
- If symptoms persist despite optimized physical therapy and comorbidity management, coordinate with pain management specialists for interventional procedures such as trigger point injections or peripheral nerve blocks, not systemic muscle relaxants 9
Critical Pitfalls to Avoid
- Never prescribe opioids for chronic pain management in hEDS, particularly when GI manifestations are present 2
- Avoid invasive diagnostic procedures unnecessarily, as tissue fragility increases complication risk 1
- Do not use muscle relaxants as a substitute for physical therapy, as this undermines the primary therapeutic goal of strengthening periarticular muscles 1, 4
- Ensure nutritional counseling accompanies any dietary interventions to prevent restrictive eating patterns and avoidant/restrictive food intake disorder (ARFID) 2