From the Guidelines
The best treatment approach for a patient with Chiari 1 malformation experiencing migraines typically involves a combination of surgical and medical management, with posterior fossa decompression surgery being the definitive treatment for symptomatic cases, and medical management including preventive medications such as topiramate, propranolol, or amitriptyline, as well as lifestyle modifications. For patients with symptomatic Chiari 1 malformation causing significant migraines, posterior fossa decompression surgery is often the definitive treatment, which involves removing a small portion of the skull and sometimes the C1 vertebra to relieve pressure on the cerebellum and brainstem 1. While awaiting surgery or for those who are not surgical candidates, medical management of migraines should include first-line preventive medications. According to the American College of Physicians, if recommended treatments are not tolerated or result in an inadequate response, consider an ACE inhibitor (lisinopril), an ARB (candesartan or telmisartan), or an SSRI (fluoxetine) 1. The use of a headache diary may help to determine treatment efficacy, identify analgesic overuse, and follow up on migraine progression 1. Certain behavioral interventions, such as cognitive behavioral therapy, relaxation training, or mindfulness-based treatment alone or combined with other components, may decrease the frequency of migraine headaches, and education alone that focuses on behavioral changes may also improve migraine-related disability 1. For acute migraine attacks, triptans like sumatriptan (50-100mg orally as needed) can be effective, along with NSAIDs such as ibuprofen (400-600mg every 6 hours as needed) or naproxen (500mg initially, then 250mg every 6-8 hours as needed) 1. Lifestyle modifications are also important, including maintaining regular sleep patterns, staying hydrated, avoiding known migraine triggers, and stress management techniques. This comprehensive approach addresses both the structural abnormality causing pressure on neural structures and the resulting migraine symptoms, providing relief while minimizing medication side effects. It is essential to initiate pharmacologic treatment for the prevention of migraine at a low dose and gradually increase the dose until desired outcomes are achieved, and to switch pharmacologic treatment for the prevention of episodic migraine headache if an adequate response is not achieved during a reasonable trial period (generally 2 to 3 months), or earlier if an AE occurs 1. In people of childbearing potential and in those who are pregnant or breastfeeding, discuss AEs of pharmacologic treatments during pregnancy and lactation 1. Prescribe less costly recommended medications, and consider reevaluating the balance of benefits, harms, and costs of preventive treatment with the patient 1. The 2023 U.S. Department of Veterans Affairs and U.S. Department of Defense clinical practice guideline for the management of headache provides recommendations for the abortive and preventive treatment of migraines, including the use of CGRP monoclonal antibodies, gepants, and other pharmacotherapies, as well as nonpharmacologic interventions such as physical therapy and exercise 1. The guideline also emphasizes the importance of individualized treatment plans that account for headache type or types, comorbid conditions, and values and preferences. Overall, a comprehensive treatment approach that combines surgical and medical management, lifestyle modifications, and individualized care can provide effective relief for patients with Chiari 1 malformation experiencing migraines.
From the Research
Treatment Approaches for Chiari 1 Malformation with Migraines
- The treatment of migraines in patients with Chiari 1 malformation can be challenging due to the complexity of the condition 2.
- A multidisciplinary approach, involving neurologists, neurosurgeons, and neuroradiologists, is recommended for the evaluation and treatment of headache related to Chiari 1 malformation 2.
- Topiramate has been shown to be effective in reducing and/or disappearing headache, normalizing sleep, and improving cerebral bioelectric activity in patients with Chiari type I malformation and concomitant cranio-vertebral anomaly 3.
- Anti-epileptic drugs, such as topiramate and valproate, are effective for the preventive treatment of migraine, but may have potential side effects, particularly for women of childbearing potential 4.
Considerations for Treatment
- The prevalence of migraine in patients with Chiari 1 malformation is high, with 34% of headache patients experiencing migraine 5.
- Migraine is more prevalent in patients with isolated Chiari 1 malformation, with a significant association between migraine and isolated Chiari 1 malformation 5.
- A careful headache clinical characterization, according to the International Headache Society criteria, is advised in patients with radiological evidence of Chiari 1 malformation, particularly in the absence of neurological signs 5.
- The use of anti-epileptic drugs, such as topiramate, may be beneficial in treating migraines in patients with Chiari 1 malformation, but the potential side effects and benefits should be carefully considered 3, 4.