Can Ehlers-Danlos syndrome cause paresthesias?

Ehlers-Danlos Syndrome Can Cause Paresthesias

Yes, Ehlers-Danlos syndrome (EDS) can cause paresthesias as part of its neurological manifestations. Paresthesias (abnormal sensations like tingling, numbness, or pins and needles) are recognized neurological symptoms that can occur in patients with various types of EDS, particularly in the hypermobile type.

Neurological Manifestations in EDS

Common Neurological Symptoms

• Paresthesias
• Musculoskeletal pain
• Fatigue
• Headache
• Muscle weakness

According to a comprehensive review, neurological manifestations in EDS are "unexpectedly common and potentially disabling" 1. Although the nervous system is not considered the primary target of the underlying molecular defect in EDS, neurological symptoms are increasingly recognized as important clinical features.

Mechanisms of Paresthesias in EDS

Several mechanisms may contribute to paresthesias in EDS patients:

1. Peripheral Neuropathy: Abnormal connective tissue surrounding nerves may lead to compression or traction injuries

2. Autonomic Dysfunction: Many EDS patients have comorbid POTS (Postural Orthostatic Tachycardia Syndrome), which can cause paresthesias 2

3. Mast Cell Activation: EDS is associated with mast cell activation syndrome (MCAS), which can trigger neurological symptoms including paresthesias 2

4. Cervical Instability: In some EDS patients, cervical instability can lead to compression of nerve roots or the spinal cord, resulting in paresthesias 3

Clinical Presentation

Paresthesias in EDS patients may present as:

• Tingling sensations in extremities
• Numbness
• "Pins and needles" sensations
• Burning sensations

These symptoms may be exacerbated by:

• Positional changes
• Physical activity
• Prolonged static postures
• Flares of autonomic dysfunction

Management of Paresthesias in EDS

Pharmacological Approaches

• Neuromodulators: The American Academy of Pain Medicine suggests that gabapentin and pregabalin may help manage neuropathic symptoms in EDS patients 3
• Low-dose antidepressants: Medications like mirtazapine may help with pain and neurological symptoms 3
• Avoid opioids: The American Society of Regional Anesthesia and Pain Medicine recommends avoiding opioids for chronic pain management in EDS due to high risk of addiction and limited efficacy 3

Non-Pharmacological Approaches

• Physical therapy: Targeted exercises to improve joint stability and reduce pressure on nerves
• Occupational therapy and bracing: These have shown to be among the most effective treatment options, with 70% of patients reporting improvement 4
• Complementary/alternative treatments: These are commonly used by EDS patients, though efficacy varies 4

Management of Associated Conditions

• POTS management: Increasing fluid and salt intake, exercise training, and compression garments 2
• MCAS treatment: Histamine receptor antagonists and mast cell stabilizers, plus avoiding triggers 2

Special Considerations

Diagnostic Evaluation

When paresthesias are present in suspected EDS patients:

• Comprehensive clinical evaluation focusing on joint hypermobility using the Beighton scale
• Skin examination for hyperextensibility, fragility, and poor wound healing
• Family history and targeted genetic testing based on suspected EDS subtype

Multidisciplinary Approach

The Ehlers-Danlos Society recommends a comprehensive approach to managing EDS patients with neurological symptoms, including:

• Neurology
• Physical medicine
• Rheumatology
• Pain management
• Genetics

Clinical Pitfalls to Avoid

1. Misdiagnosis: Paresthesias in EDS are often misattributed to other conditions, leading to diagnostic delays

2. Inappropriate treatments: Neuropathic modulators are the least well-tolerated treatment option, with 47% of patients reporting adverse effects 4

3. Overlooking comorbidities: Failing to recognize and treat associated conditions like POTS or MCAS that may contribute to paresthesias

4. Excessive testing: Unnecessary neuroimaging or invasive procedures when symptoms are consistent with known EDS manifestations

5. Inadequate pain management: Underestimating the impact of paresthesias on quality of life and functional status

By recognizing paresthesias as a legitimate manifestation of EDS and implementing appropriate management strategies, clinicians can improve outcomes and quality of life for these patients.