Relapsing painful ophthalmoplegic neuropathy: a rare case of migraine that should be remembered

*Correspondence: [e-mail].

Abstract

Case Presentation: A 9-year-old boy who presented irritability and palpebral ptosis at the age of 9 months, associated with impaired eye movement and mydriasis on the right. He underwent corticosteroid therapy with improvement of symptoms. In the same year, in the context of steroid weaning, he had two episodes of recurrent irritability and ptosis. Episodes of headache and ptosis were recurrent, with a frequency of 3 to 4 episodes, in the subsequent two years and in 2019 there was only one episode. In the last four years, outpatient follow-up still with corticosteroid therapy, evolving with headache described as moderate intensity, pulsating, with photo and phonophobia, nausea, with weekly frequency, without associated ocular component. In March of this year, he experienced a worsening pattern of the headache associated with ptosis, mydriasis and change in eye movement to the right. It presents MRI in the period of crises with a rounded image in the cisternal segment of the oculomotor nerve in the interpeduncular cistern on the right, which improves with resolution. CSF analyzes were normal, except for HHV6 positivity.

Discussion: Recurrent Painful Ophthalmologic Neuropathy (RPON) is a rare neurological disorder. Classically described as a variant of migraine, with a change in concept, it is now accepted as an inflammatory neuropathy. It is described as ocular cranial nerve palsies within a few days and gradual resolution of symptoms over time. It is a diagnosis of exclusion and remains closely associated with migraine. The average age of the first attack is 8 years, but it varies between 7 months to 50 years. The headache is mostly of a migraine pattern and in children it can manifest itself as irritability and restlessness and can last for days to weeks. The involvement of the oculomotor nerve is the most seen, involving the pupil and with ptosis, but any oculomotor nerve can be affected. Most patients will present with abnormal oculomotor nerve enhancement in brain MRI, typically in the nerve root exit zone at the Interpeduncular cistern that tends to improve after the attack. The natural course of an episode is the spontaneous resolution of the nerve palsy over days to months. Corticosteroids are a tool that help in a faster recovery of the condition.

Final Comments: RPON is an uncommon and possibly heterogeneous disorder, the first attack is predominantly during childhood and adolescence. Overall prognosis is good for individuals, but permanent nerve damage can accumulate with repeated attacks.

Publication History

Article published online:
12 May 2025