Diagnostic controversies in recurrent painful ophthalmoplegic neuropathy: single case report with a systematic review

Ital J Pediatr. 2022 Jun 3;48(1):82. doi: 10.1186/s13052-022-01274-x.


Background: Ophthalmoplegic migraine, renamed "Recurrent Painful Ophthalmoplegic Neuropathy" (RPON) in 2013 by the International Headache Society is a rare neurologic disorder characterized by recurrent attacks of ophthalmoplegia associated to ipsilateral headache. The etiology is still unknown. Typical magnetic resonance imaging findings show a focal nerve thickening and contrast enhancement. In the majority of cases, there is a full recovery within days or weeks. There is no evidence supporting a specific treatment. The review defines the characteristics of the recurrent painful ophthalmoplegic neuropathy in patients within 2 years of age underlying the importance of the role of magnetic resonance imaging even in presence of the first attack. Thus, an emblematic case report is presented.

Case presentation: The authors present a case of third cranial nerve paresis in a 17-month-old male child, presenting a neuroradiological pattern highly suggestive of schwannoma, aneurism or recurrent painful ophthalmoplegic neuropathy. Thus, a review of the literature with the pediatric casuistry of recurrent painful ophthalmoplegic neuropathy occurred within 2 years of age focusing on diagnostic considerations is presented. The authors highlight the importance to consider recurrent painful ophthalmoplegic neuropathy in presence of magnetic resonance imaging findings and clinical symptoms referable to aneurysm or schwannoma. Thus, the review defines the characteristics and the neuroradiological findings at the first RPON attack occurred under 2 years of age.

Conclusion: Although two attacks are necessary, the review strongly suggests to consider recurrent painful ophthalmoplegic neuropathy even at the first attack, in presence of described characteristics and the aforementioned magnetic resonance imaging findings.

Keywords: Case report; Headache; Ophthalmoplegia; Ophthalmoplegic migraine; Recurrent painful ophthalmoplegic neuropathy; Schwannoma.

Publication types

  • Case Reports
  • Systematic Review

MeSH terms

  • Child
  • Headache
  • Humans
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Neurilemmoma* / complications
  • Ophthalmoplegia* / diagnosis
  • Ophthalmoplegia* / etiology
  • Ophthalmoplegic Migraine* / complications
  • Ophthalmoplegic Migraine* / diagnosis
  • Ophthalmoplegic Migraine* / drug therapy
  • Pain
  • Peripheral Nervous System Diseases
  • Rare Diseases
  • Tolosa-Hunt Syndrome

Supplementary concepts

  • Neuropathy, Painful
  • Ophthalmoplegia, painful