Recurrent Painful Ophthalmoplegic Neuropathy with Unilateral Oculomotor and Trochlear Nerve Palsy in an 8-year-old Girl. Issue 4 (2nd October 2022)

• Record Type: Journal Article
• Authors: Akhondian, Javad; Ashrafzadeh, Farah; Seilanian Toosi, Farrokh; Esmaeilzadeh, Mahla; Beiraghi Toosi, Mehran; Imannezhad, Shima; Saeedi Zand, Nazanin; Hashemi, Narges
• Is Part Of: Journal of binocular vision and ocular motility. Volume 72:Issue 4(2022)
• Page Start: 199
• Page End: 204
• Publication Date: 2022-10-02
• Subjects: Trochlear nerve palsy -- fourth cranial nerve palsy -- oculomotor cranial nerve palsy -- third cranial nerve palsy -- ophthalmoplegic migraine -- recurrent painful ophthalmoplegic neuropathy -- migraine; Eye -- Movements -- Periodicals; Eye -- Movement disorders -- Periodicals; Binocular vision -- Periodicals; Binocular vision; Eye -- Movements; Vision, Binocular; Ocular Motility Disorders; Ophthalmology; Periodicals; Periodical; Electronic journals; 617.762
• DOI: Https://www.tandfonline.com/doi/10.1080/2576117X.2022.2082219
• Languages: English
• ISSNs: 2576-117X

Abstract

ABSTRACT: Background: Recurrent painful ophthalmoplegic neuropathy (RPON) is a rare disorder with a unilateral headache accompanied by ipsilateral episodes of painful ocular cranial nerve neuropathy, which typically occurs in childhood. Case report: We report an 8-year-old female with four episodes of RPON involving unilateral third and fourth cranial nerves. Right eye exotropia and complete ptosis were detected on examination. Brain MRI images revealed right third nerve enhancement where it exits from the brainstem. She completely recovered after 5 weeks with the administration of prednisolone and indomethacin. Discussion and conclusion: Due to the rarity of this condition in children, recurrent painful ophthalmoplegic neuropathy should be considered as a differential diagnosis of unilateral or bilateral painful ophthalmoplegia, particularly with a history of migrainous headache. Since it is a treatable entity, and repeated attacks may lead to permanent sequela, early intervention is crucial.