Recurrent Painful Ophthalmoplegic Neuropathy and Oculomotor Nerve Schwannoma: A Pediatric Case Report with Long-Term MRI Follow-Up and Literature Review

Pain Res Manag. 2019 Sep 25:2019:5392945. doi: 10.1155/2019/5392945. eCollection 2019.

Abstract

Background: Recurrent painful ophthalmoplegic neuropathy (RPON), previously known as ophthalmoplegic migraine (OM), is an uncommon disorder with repeated episodes of ocular cranial nerve neuropathy associated with ipsilateral headache. The age of presentation is most often during childhood or adolescence. MRI has a central role in the assessment of the RPON, especially to distinguish orbital, parasellar, or posterior fossa lesions that mimic symptoms of RPON. Actually, oculomotor nerve tumors may be masquerade as RPON so that MRI follow-ups are required to detect the possibility of tumor etiology.

Case presentation: We report a 16-year-old boy with a 7-year follow-up and multiple brain MRI data, previously diagnosed as OM. The last brain MRI, performed during an acute phase of oculomotor paresis with ipsilateral headache, showed a nodular lesion described as schwannoma of III cranial nerve. Then, we reviewed the literature on OM and RPON in pediatric age with a focus on brain MRI findings.

Conclusions: This review highlights the important role of serial brain MRIs in the long-term follow-up of RPON, especially in the cases with childhood onset, in order to not delay the diagnosis of a possible oculomotor nerve schwannoma.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Cranial Nerve Neoplasms / complications
  • Cranial Nerve Neoplasms / diagnostic imaging*
  • Follow-Up Studies
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Neurilemmoma / complications
  • Neurilemmoma / diagnostic imaging*
  • Oculomotor Nerve Diseases / complications
  • Oculomotor Nerve Diseases / diagnostic imaging*
  • Ophthalmoplegic Migraine / etiology*