Session: Poster session B

Ophthalmoplegic migraine: a retrospective cohort study

Denis GEORGELIN¹, Melodie AUBART¹, Isabelle DESGUERRE¹, Nathalie BODDAERT¹, Dominique BREMOND-GIGNAC¹, Robert MATTHIEU¹

¹Necker-Enfants malades Hospital, Paris, France

INTRODUCTION

Recurrent painful ophthalmoplegic neuropathy (RPON) of the III cranial nerve, formerly known as“ophthalmoplegic migraine”, is a condition characterized by recurrent crises of headaches and III nerve palsy, which pathophysiology remains unclear.

METHODS

Cases of typical ophthalmoplegic migraines examined in our department between 2011 and 2023 were retrospectively retrieved and studied. These included III nerve RPON fitting the International Classification of Headache Disorders definition, and cases of non-recurrent painful ophthalmoplegic neuropathy (NRPON) fitting all other characteristics of the definition.

RESULTS

Twelve cases were retrieved – 11 males, 1 female, median age at first presentation: 2.9 years –, consisting in 6 cases of RPON and 6 of NRPON.

The left eye was involved in 7 patients and the right in 5. The median number of “crises” was 1.5 per patient [1-7]. Ophthalmoplegia and/or ptosis occurred in all patients and pupil was involved, in association with the previous ones, in 9 patients (75%). Six patients (50%) had a complete resolutive episode, whereas 6 (50%) had persistent sequelae of diverse degrees.

All patients had a brain MRI and ten of them (83%) (5 RPON and 5 NRPON) revealed an abnormality of the III cranial nerve on the side of the crisis, which always consisted in a round lesion at the anatomic emergence of the III in the interpeduncular cistern, enhancing with gadolinium. This enhancement vanished after the crisis in 1 case, while it persisted in 9 cases.

CONCLUSIONS

Most of the patients who presented with ophthalmoplegic migraine were males. The presence of a characteristic lesion of the III at the time of the crisis was present in the vast majority of cases. The pathophysiological value of such lesions, often described as III nerve “schwannomas”, is debated. Its value as a diagnostic criterion of a specific pathological entity, however, should probably be considered, be it or not followed by a recurrence.