A Case of Recurrent Painful Ophthalmoplegic Neuropathy

Yan, Yaping; Zhang, Bo; Lou, Zhuocong; Liu, Kai-Ming; Lou, Ming; Ding, Meiping; Xu, Yongfeng

doi:10.3389/fneur.2020.00440

Cited by 6 publications

(6 citation statements)

References 17 publications

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“…16 In a case report by Yan et al, the focal enhancement of the cisternal portion of the oculomotor nerve was first suspected to be a schwannoma, but that was disproven by a follow-up MRI, which revealed enhancement regression. 2 Falsaperla et al reported a similar case, where the symptom regression coincided with the complete resolution of nerve thickening and enhancement, which is incompatible with the presentation of schwannoma. 17 Ophthalmoplegia, ptosis, and mydriasis can be indicative of both RPON and schwannoma, but these conditions differ in their clinical course.…”

Section: Discussionmentioning

confidence: 96%

“…4,8 However, the aforementioned MRI findings associated with ophthalmoplegia can sometimes be confused with schwannoma, while some oculomotor schwannomas can mimic the symptoms of RPON. 2,9 Here, we report a patient with ipsilateral headache, ophthalmoplegia, and ptosis in whom the MRI findings showed thickening and postcontrast enhancement of the oculomotor nerve during an acute episode, which was interpreted as possible schwannoma. The follow-up MRI showed regression of nerve thickening and decreased contrast uptake, which corresponded to symptom resolution and was consistent with the clinical features of RPON.…”

Section: Introductionmentioning

confidence: 91%

“…1 RPON used to be referred to as ophthalmoplegic migraine and was classified as a migraine variant in the first edition of the International Classification of Headache Disorders, but it was renamed and reclassified as a cranial neuralgia in subsequent editions. 1,2 Most patients report their first attack in child-hood, but age of onset can range from 3 months to 74 years. 3,4 The exact gender ratio is unknown, with different authors reporting a predominance of either gender or an approximately equal ratio.…”

Section: Introductionmentioning

confidence: 99%

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Recurrent Painful Ophthalmoplegic Neuropathy Mistaken for Oculomotor Schwannoma: A Case Report

Mahović

Bračić

Jovanović

et al. 2022

Journal of Pediatric Neurology

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Recurrent painful ophthalmoplegic neuropathy (RPON) is a headache disorder classified as a cranial neuropathy. It is characterized by episodes of unilateral headache followed by ipsilateral ophthalmoplegia due to ocular cranial nerve palsy. Symptoms usually subside spontaneously within weeks to months. RPON commonly occurs in childhood, though it is overlooked at times as a possible differential diagnosis. The pathophysiology of RPON is not entirely clear. Typical magnetic resonance imaging (MRI) findings in children include nerve thickening and gadolinium enhancement. However, these findings are not specific to this condition. Nerve thickening in RPON can resemble schwannomas, thereby causing a diagnostic dilemma. Here, we present a 13-year-old boy with a history of unilateral headaches associated with ipsilateral ptosis and diplopia. The first MRI indicated thickening and gadolinium enhancement of the right oculomotor nerve, which was interpreted as a schwannoma. However, his symptoms resolved after a few weeks and the follow-up MRI showed resolution of nerve changes. This clinical presentation was consistent with RPON. This case demonstrates the need for a careful follow-up of RPON patients.

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Section: Discussionmentioning

confidence: 96%

Section: Introductionmentioning

confidence: 91%

Section: Introductionmentioning

confidence: 99%

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Recurrent Painful Ophthalmoplegic Neuropathy Mistaken for Oculomotor Schwannoma: A Case Report

Mahović

Bračić

Jovanović

et al. 2022

Journal of Pediatric Neurology

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“…Similar to our cases, cranial nerve involvements have been reported as contrast enhancing nodular lesions. 8,9 Hashimoto's encephalopathy and myelin oligodendrocyte glycoprotein-associated disease, which may present with recurrent ophthalmoplegia, were not considered in our patients with long-term follow-up due to their clinical and radiological features. Imaging findings are more commonly seen in pediatric cases and the disappearance of lesion usually takes 12 weeks.…”

Section: Discussionmentioning

confidence: 99%

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

Günay¹,

Edem²,

Kurul³

et al. 2022

Turk J Pediatr

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Background. Recurrent painful ophthalmologic neuropathy (RPON), formerly known as ophthalmoplegic migraine, is characterized by repeated attacks of one or more ocular cranial nerve palsies with an ipsilateral headache. While steroid therapy has been reported to be beneficial for attacks, no clear consensus on prophylactic treatments exists. We present two cases emphasizing the diagnostic significance of the loss of enhancement during the symptom-free period and valproate as a beneficial option in prophylaxis. Case 1. A 4-year-old girl presented with a one-week right frontal headache, vomiting and photophobia. Neurological examination revealed ptosis, oculomotor nerve paresis, and delay in light reflex in the right eye. 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. The enhancement regressed after a 6-month symptom-free period. While propranolol, topiramate and flunarizine were inefficacious in prophylaxis, the patient responded to valproate prophylaxis and benefited from the administration of steroids for one week during the attacks. Case 2. A 7-year-old girl presented with a ten-day right-sided, throbbing headache in the frontal region, oneday eye deviation and double vision. Neurological examination revealed inward gaze restriction and ptosis in the ipsilateral eye to the headache. Brain MRI revealed a 4.5 mm, enhancing, nodular lesion in the 3rd cranial nerve lodge in the right perimesencephalic area. Her symptoms regressed in one week with dexamethasone and she received prophylactic propranolol. Neuroimaging findings disappeared after a 3-month symptom-free period. After valproate was added because of a relapse, she did not experience any further attacks. Conclusions. RPON is an uncommon disease in childhood with unknown etiology. On brain MRI with contrast during the symptom-free period, regression of the enhancement or complete resolution of the lesion are guiding features in the diagnosis. Valproate may have beneficial effects on RPON treatment.

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“…Imaging finding reported with RPON includes thickening and enhancement of one or more of the ocular cranial nerves, most commonly the oculomotor nerve [3,4,8,[16][17][18]. The nerve thickening can be smooth or nodular [3,4,8,16,17]. Follow-up imaging is important.…”

Section: Diagnostic Criteriamentioning

confidence: 99%

Recurrent Painful Ophthalmoplegic Neuropathy in an Adult Patient: A Case Report With Literature Review

Ansari¹,

Dua²,

Mafraji³

2022

Cureus

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A Case of Recurrent Painful Ophthalmoplegic Neuropathy

Cited by 6 publications

References 17 publications

Recurrent Painful Ophthalmoplegic Neuropathy Mistaken for Oculomotor Schwannoma: A Case Report

Recurrent Painful Ophthalmoplegic Neuropathy Mistaken for Oculomotor Schwannoma: A Case Report

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

Recurrent Painful Ophthalmoplegic Neuropathy in an Adult Patient: A Case Report With Literature Review

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