SUNCT/SUNA and neurovascular compression: new cases and critical literature review

Cephalalgia. 2013 Dec;33(16):1337-48. doi: 10.1177/0333102413494273. Epub 2013 Jun 25.

Abstract

Background: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with cranial autonomic symptoms (SUNA) are primary headache syndromes. A growing body of literature has focused on brain magnetic resonance imaging (MRI) evidence of neurovascular compression in these syndromes.

Objective: The objective of this article is to assess whether SUNCT is a subset of SUNA or whether the two are separate syndromes and clarify the role of neurovascular compression.

Method: We describe three new SUNCT cases with MRI findings of neurovascular compression and critically review published SUNCT/SUNA cases.

Results: We identified 222 published SUNCT/SUNA cases. Our three patients with neurovascular compression added to the 34 cases previously described (16.9%). SUNCT and SUNA share the same clinical features and therapeutic options. At present, there is no available abortive treatment for attacks. Lamotrigine was effective in 64% of patients; topiramate and gabapentin in about one-third of cases. Of the 34 cases with neurovascular compression, seven responded to drug therapies, 16 patients underwent microvascular decompression of the trigeminal nerve (MVD) with effectiveness in 75%.

Conclusions: We suggest that SUNCT and SUNA should be considered clinical phenotypes of the same syndrome. Brain MRI should always be performed with a dedicated view to exclude neurovascular compression. The high percentage of remission after MVD supports the pathogenetic role of neurovascular compression.

Keywords: SUNA; SUNCT; autonomic; microvascular decompression; neurovascular compression.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Arteries / pathology
  • Cerebellum / blood supply
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Microvascular Decompression Surgery
  • Middle Aged
  • Nerve Compression Syndromes / pathology*
  • Nerve Compression Syndromes / surgery
  • SUNCT Syndrome / pathology*
  • SUNCT Syndrome / surgery
  • Trigeminal Autonomic Cephalalgias / pathology
  • Trigeminal Autonomic Cephalalgias / surgery
  • Trigeminal Nerve / pathology
  • Trigeminal Nerve / surgery