How to manage headaches in patients (pts) with myotonic dystrophy?

Management of Headaches in Patients with Myotonic Dystrophy

Modafinil is the recommended first-line treatment for headaches in patients with myotonic dystrophy, as it has shown clinically significant improvement in excessive daytime sleepiness which can contribute to headache symptoms.

Understanding Headaches in Myotonic Dystrophy

Patients with myotonic dystrophy commonly experience headaches that may be related to:

• Sleep disturbances, including excessive daytime sleepiness (EDS), which is a prominent feature in myotonic dystrophy 1, 2
• Sleep-disordered breathing, including both central and obstructive sleep apneas 1, 3
• Periodic limb movements during sleep, which can disrupt sleep quality 2
• Central dysfunction of sleep regulation rather than solely due to sleep-related breathing disorders 3

First-Line Treatment Approach

Pharmacological Management

• Modafinil (200-400mg daily) is suggested as first-line treatment for patients with myotonic dystrophy experiencing headaches related to excessive daytime sleepiness 4
  • Has demonstrated clinically significant improvements in excessive daytime sleepiness in randomized controlled trials specifically in myotonic dystrophy patients 4
  • Addresses the underlying sleep dysfunction that may contribute to headache symptoms 5

Headache-Specific Treatment

For acute headache management in myotonic dystrophy patients:

• NSAIDs (ibuprofen 400mg or naproxen sodium) should be used as first-line treatment for acute headache episodes 4, 6
  • Consider adding an antiemetic if nausea is present 6
• Avoid medication overuse by limiting acute treatments to no more than 2-3 days per week to prevent medication overuse headache (MOH) 4

Second-Line and Adjunctive Treatments

If first-line treatments are insufficient:

• Topiramate may be considered for preventive treatment if headaches have migraine features and occur frequently 4
• Amitriptyline (starting at 10mg and titrating up to 50-100mg) may be beneficial for patients with tension-type headaches and can also address sleep disturbances 4
• Non-oral routes of medication administration should be considered if vomiting is severe 6

Management of Comorbid Sleep Disorders

Addressing underlying sleep disorders is crucial for headache management in myotonic dystrophy:

• Evaluate for sleep-disordered breathing with polysomnography, as 18-27% of myotonic dystrophy patients have obstructive sleep apnea or respiratory failure 5
• Consider CPAP or NIV for patients with documented sleep-disordered breathing 5
• Address periodic limb movements which are commonly associated with sleep disturbance in myotonic dystrophy 2

Non-Pharmacological Approaches

• Physical therapy with techniques including thermal methods, trigger point massage, and mobilization can be beneficial for reducing headache frequency and intensity 4
• Aerobic exercise or progressive strength training 2-3 times per week for 30-60 minutes may help prevent headaches 4
• Stress reduction techniques may be beneficial, as stress can exacerbate headache symptoms 4

Important Clinical Considerations

• Monitor for medication side effects: Common side effects of modafinil include insomnia, nausea, headache, and dry mouth 4
• Pregnancy precautions: Modafinil may cause fetal harm based on animal data and may reduce the effectiveness of oral contraceptives 4
• Evaluate treatment effectiveness regularly using tools like the Headache Under-Response to Treatment (HURT) questionnaire 4
• Watch for medication overuse headache which can develop in patients with pre-existing headache disorders who regularly overuse acute medications 4

When to Refer to Specialist Care

Consider referral to neurology or sleep medicine specialist when:

• Headaches are not responding to first-line treatments 4
• There are concerns about chronic migraine transformation 4
• Complex sleep disorders require specialized management 1, 5
• Respiratory failure is suspected or confirmed 5