Dados do Trabalho

Título

RECURRENT PAINFUL OPHTHALMOPLEGIC NEUROPATHY: A REPORT OF TWO CASES

Apresentação dos casos

Case 1: A 14-year-old girl with a history of recurrent episodes since the age of 4 presented with left parietal throbbing headaches. Each episode was associated with photophobia, phonophobia, nausea, vomiting, and ipsilateral ptosis and ophthalmoplegia. The pain typically lasted about 14 days and recurred every 3-4 months. Brain MRI revealed thickening and enhancement of the cisternal portion of the left third cranial nerve. Laboratory tests and cerebrospinal fluid (CSF) examination were normal. Case 2: A 2-year-old boy presented with right ptosis, mydriasis, and exotropia, but without headache. Brain MRI showed enhancement and thickening of the cisternal portion of the right third cranial nerve, with a 3mm nodular appearance. Laboratory tests and CSF examination were normal. The child received Intravenous pulse methylprednisolone (IVMP) at 30 mg/kg/day for 5 days, leading to complete recovery, though MRI findings remained altered.

Discussão

Recurrent Painful Ophthalmoplegic Neuropathy (RPON) is a rare and poorly understood neurological syndrome characterized by recurrent headaches and ophthalmoplegia. The third cranial nerve is most commonly affected, presenting with symptoms such as mydriasis and ptosis. While RPON typically presents in children, it can persist into adulthood. Diagnosis is made by excluding other similar disorders, and symptoms generally resolve with corticosteroid therapy.

Comentários finais

The pathophysiology of RPON remains largely theoretical, based on case reports and clinical observations. These cases highlight the diverse presentations of RPON and the challenges associated with its diagnosis and treatment.

Referências

Yokoyama T, Yamamiya M, Takakuwa M, Taniguchi C, Koizumi E, Jinkawa A, Obata M, Miyashita K, Inoue M, Sakazume S, Ohta K. Recurrent painful ophthalmoplegic neuropathy. J Paediatr Child Health. 2021 Aug;57(8):1303-1304. doi: 10.1111/jpc.15147. Epub 2020 Sep 1. PMID: 32870577.
Aleksic DZ, Drakulic SM, Ljubisavljevic S. Recurrent Painful Ophthalmoplegic Neuropathy: Migraine, Neuralgia, or Something Else? J Oral Facial Pain Headache. 2020 Fall;34(4):374-378. doi: 10.11607/ofph.2656. PMID: 33290443.
Mrabet S, Nasri A, Kessentini N, Ben Djebara M, Gargouri-Berrechid A, Kacem I, Gouider R. Recurrent painful ophthalmoplegic neuropathy revealing oculomotor nerve schwannoma. Tunis Med. 2021 Aout;99(8):919-923. PMID: 35261021; PMCID: PMC9003581.

Palavras Chave

ophthalmoplegic migraine; headache; neuralgia