Diagnosis and Treatment

AVM-associated headache may or may not present with other symptoms. “Intractable, unilateral headache should increase suspicion for brain AVM and prompt [magnetic resonance imaging] MRI evaluation,” according to the review.8 According to the International Classification of Headache Disorders (ICHD-3) diagnostic criteria for AVM-related headaches, they “have a clinical course that parallels that of a coexisting AVM in terms of temporality, severity, and location.” 

The most well-known example is occipital AVM, which is believed to be a risk factor for headache. Headache occurs in patients with occipital AVM at rates ranging from 15% to 61%, and it is often characterized by visual symptoms such as field cuts and blurring, as well as other migraine-like symptoms.12-14

If AVM is detected, the Spetzler-Martin grading system can help estimate risk and guide treatment selection, which is influenced by the size, location, blood vessel supply, and drainage of the AVM. Grading is based on a 1 to 5 scale, with 1 representing the simplest cases and 5 representing the most complex cases. A 2010 study observed surgical risk levels ranging from 0.7% (95% CI, 0%-3%) in grade 1-2 AVMs to 21% (95% CI, 15%-28%) in grade 3-5 AVMs.15


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If lesion eradication is identified as the treatment goal, it may be achieved by several methods. Various “case series have shown that intervention for AVM with microsurgical resection, endovascular embolization, stereotactic radiosurgery, or a combination of these can be effective in decreasing or even eliminating headaches –however, there could be bias in case reporting,” said Dr Kung.

  • Microsurgical resection, embolization, or both led to significant or complete headache improvement across a number of studies in 39% to 100% of patients.16,17
  • Radiosurgery shows improvement rates ranging from 47% to 75% across numerous studies, including a retrospective study demonstrating a 69.5% headache reduction 5 years after radiosurgery for AVM.18-20
  • Multimodal treatment, such a combination of surgery, embolization, and/or radiosurgery embolization, was associated with improvement in 50% to 67% of cases in earlier research, and the improvement rate was 87.8% in a new study of multidisciplinary care for AVM-associated headache.21  

Future Directions

Research on AVM has mainly focused on hemorrhage risk vs treatment risk, as well as seizure control, rather than headache. “There is a lot we don’t know about AVM-related headache,” said Dr Kung. “I believe that the next step should be to study the effect of pharmacological agents on achieving headache relief for unruptured AVM, targeting each hypothetical mechanism.” 

References

  1. Friedlander RM. Clinical practice. Arteriovenous malformations of the brain. New Engl J Med. 2007; 356:2704–12. doi:10.1056/NEJMcp067192
  2. Mohr JP, Kejda-Scharler J, Pile-Spellman J. Diagnosis and treatment of arteriovenous malformations. Current Neurol Neurosci Rep. 2013; 13:324. doi:10.1007/s11910-012-0324-1
  3. Brown Jr RD, Wiebers DO, Torner JC. Incidence and prevalence of intracranial vascular malformations in Olmsted County, Minnesota, 1965 to 1992. Neurology. 1996; 46:949–52. doi:http:/​/​dx.​doi.​org/​10.​1212/​WNL.​46.​4.​949
  4. Evans RW. Diagnostic testing for the evaluation of headaches. Neurol Clin. 1996; 14:1-26. doi:http://dx.doi.org/10.1016/S0733-8619(05)70240-1
  5. Erdener SE, Dalkara T. Modelling headache and migraine and its pharmacological manipulation. Br J Pharmacol. 2014;171:4575-4594. doi: 10.1111/bph.12651
  6. Chimowitz MI, Little JR, Awad IA, et al. Intracranial hypertension associated with unruptured cerebral arteriovenous malformations. Ann Neurol. 1990;27:474-479. doi:10.1002/ana.410270504
  7. Bernstein C, Burstein R. Sensitization of the trigeminovascular pathway: perspective and implications to migraine pathophysiology. J Clin Neurol. 2012;8:89-99. doi:10.3988/jcn.2012.8.2.89
  8. Ellis JA, Mejia Munne JCLavine SDMeyers PMConnolly ES JrSolomon RAArteriovenous malformations and headache. J Clin Neurosci. 2016;23:38-43. doi:10.1016/j.jocn.2015.08.003
  9. Okabe T, Meyer JS, Okayasu H, et al. Xenon-enhanced CT CBF measurements in cerebral AVM’s before and after excision. Contribution to pathogenesis and treatment. J Neurosurg. 1983;59:21-31.
  10. Mast H, Mohr JP, Thompson JL, et al. Transcranial Doppler ultrasonography in cerebral arteriovenous malformations. Diagnostic sensitivity and association of flow velocity with spontaneous hemorrhage and focal neurological deficit. Stroke. 1995; 26:1024-1027. doi:10.1161/01.STR.26.6.1024
  11. Ferrari MD, Klever RR, Terwindt GM, et al. Migraine pathophysiology: lessons from mouse models and human genetics. Lancet Neurol. 2015;14:65-80. doi:10.1016/S1474-4422(14)70220-0
  12. Troost BT, Newton TH. Occipital lobe arteriovenous malformations. Clinical and radiologic features in 26 cases with comments on differentiation from migraine. Arch Ophthalmol. 1975;93:250-256. doi:10.1001/archopht.1975.01010020260002
  13. Dehdashti AR, Thines L, Willinsky RA, et al. Multidisciplinary care of occipital arteriovenous malformations: effect on nonhemorrhagic headache, vision, and outcome in a series of 135 patients. Clinical article. J Neurosurg. 2010;113:742-748. doi:10.3171/2009.11.JNS09884
  14. Kupersmith MJ, Vargas ME, Yashar A, et al. Occipital arteriovenous malformations: visual disturbances and presentation. Neurology. 1996; 46:953-957. doi:10.​1212/​WNL.​46.​4.​953
  15. Davidson AS, Morgan MK. How safe is arteriovenous malformation surgery? A prospective, observational study of surgery as first-line treatment for brain arteriovenous malformations. Neurosurgery. 2010;66(3):498-504; discussion 504-505. doi:10.1227/01.NEU.0000365518.47684.98.
  16. Steiger HJ, Etminan N, Hanggi D. Epilepsy and headache after resection of cerebral arteriovenous malformations. Acta Neurochir Suppl. 2014;119:113–115. doi:10.1007/978-3-319-02411-0_19
  17. Martin NA, Wilson CB. Medial occipital arteriovenous malformations. Surgical treatment. J Neurosurg. 1982;56:798-802. doi:10.3171/jns.1982.56.6.0798
  18. Hadjipanayis CG, Levy EI, Niranjan A, et al. Stereotactic radiosurgery for motor cortex region arteriovenous malformations. Neurosurgery. 2001;48:70-76 [discussion 76-77].
  19. Lunsford LD, Kondziolka D, Flickinger JC, et al. Stereotactic radiosurgery for arteriovenous malformations of the brain. J Neurosurg. 1991;75:512-5124. doi:10.3171/jns.1991.75.4.0512
  20. Bowden G, Cavaleri J, Kano H, et al. Radiosurgery for arteriovenous malformations and the impact on headaches. Headache. 2017;57(5):737-745. doi:10.1111/head.13055
  21. Lai LF, Chen M, Chen JX, et al. Multidisciplinary care of unruptured brain arteriovenous malformations to improve symptomatic headache and the onset, progression, and outcomes of unruptured brain arteriovenous malformations. Pain Physician. 2017; 20(1):E127-E136.