Recurrent painful ophthalmoplegic neuropathy: a report of two new pediatric cases

• Published in: Turkish journal of pediatrics Vol. 64; no. 3; pp. 592 - 598
• Main Authors: Günay, Çaǧatay, Edem, Pınar, Kurul, Ayşe Semra Hız, Yaşar, Elif, Yiş, Uluç
• Format: Journal Article
• Language: English
• Published: Ankara Hacettepe University Faculty of Medicine 01-05-2022
• Subjects: Disease prevention; Family medical history; Headaches; Magnetic resonance imaging; Medical imaging; Migraine; Neuroimaging; Patients; Pediatrics; Steroids; Vomiting
• ISSN: 0041-4301
• DOI: 10.24953/turkjped.2021.n24

Brain magnetic resonance imaging (MRI) revealed a 5.5 mm nodular enhancement in the cisternal part of the 3rd cranial nerve in the right premesencephalic area. Recurrent painful ophthalmoplegic neuropathy (RPON), previously known as ophthalmoplegic migraine, is a rare syndrome characterized by repeating attacks of one or more ocular cranial nerve palsies with an ipsilateral headache, in which secondary causes have been excluded.1 The first contributions to the literature on this disease date back to the 19th century with Note in 1854 and Gubler in 1860, but Charcot named it "migraine ophthalmology" for the first time in 1890. Magnetic resonance angiography (MRA) revealed reduced flow at P1 segment of the right posterior cerebral artery (PCA), moderate narrowing of the P1 segment posteriorly, compared to the left P1 segment, secondary to the lesion originating from the 3rd cranial nerve (Fig.1b). The attacks restricting daily activity, became more pronounced with physical activity, benefited from analgesics, and were not accompanied by photophobia, phonophobia, nausea, vomiting and ocular symptoms.