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

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.

A Case of Recurrent Painful Ophthalmoplegic Neuropathy

“…However, the increased thickening of the oculomotor nerve observed after 9 years of RPON attacks may serve as evidence in support of the tumor theory. This hypothesis extends the inflammatory theory, proposing that repeated bouts of inflammation may lead to cycles of demyelination and remyelination of the affected nerve, and such a process may encourage the transformation of Schwann cells to schwannoma (5). Yet, as Kim et al, Akimoto et al, and Shin et al, have proposed, follow-up surveillance via MRI is required to exclude tumors, especially in patients with suspected RPON with persistent post-contrast enhancement during times of remission (10,11,20).…”

“…As for the tumor pathogenesis, it has been reported that transient or recurrent oculomotor nerve deficits may be the primary manifestation in some cases of cranial nerve schwannoma (10). Recently, Petruzzelli et al have reported a case of a 16-year-old boy with a diagnosis of OM initially but which turned out to have the presentation of a schwannoma after 7year follow-up (5). In her review, three cases of schwannoma of the affected nerve had been reported, which included a long history of follow-up, and only one was histologically proven (17).…”

“…In 2013, OM was replaced by recurrent painful ophthalmoplegic neuropathy (RPON) in IHCD-3 based on headache, subsequent cranial neuropathies, MRI features, and the fact that some cases showed response to corticosteroid treatment (4). However, the etiology remains controversial, but mainly involves schwannoma or recurrent bouts of demyelination of the affected nerve (5,6).…”

Recurrent Painful Ophthalmoplegic Neuropathy: Diagnostic Controversies. Single Case Report with a Systematic Review

“…We analyzed the relationship between RPON and schwannoma. As proposed by Petruzzelli et al (15), we are performing a long-term follow-up at our institute in order to prevent any complications.…”

Headache with cranial nerve palsy – a diagnostic and therapeutic challenge in three cases

“…Egyik elmélet szerint a tumor intermittensen olyan kémiai anyagokat bocsájt ki már akkor is, amikor imagisztikailag még nem detektálható, amelyek stimulálják a trigeminus receptorait, ami migrénes fejfájáshoz vezet, így a RPON a tumor kezdeti megnyilvánulásának tekinthető [32,34]. A másik hipotézis szerint a RPON hátterében álló visszatérő gyulladás sorozatos demyelinisatiohoz és remyelinisatiohoz vezet, amelyek elindíthatják a Schwann-sejtek proliferációját és átalakulását schwannomává, így ez a tumor a RPON hosszútávú komplikációjának tekinthető [35].…”