A Case of Recurrent Painful Ophthalmoplegic Neuropathy with Associated Oculomotor Nerve Tumour

Background: Recurrent painful ophthalmoplegic neuropathy (RPON), formerly known as ophthalmoplegic migraine (OM), is a poorly understood condition that presents with recurrent unilateral headaches and at least one ocular cranial nerve (CN) palsy, generally in childhood. There has been ongoing debate about whether the etiology of this disorder is neuropathic or related to migraines. The Case: We present a case about a 10-year-old male with his third presentation of RPON, repeatedly affecting his right oculomotor nerve. His treatment choices are discussed, along with associated outcomes. The patient was treated with topiramate with resolution of his symptoms occurred within one month. Conclusion: As the annual incidence of RPON is rare at fewer than 1 case per million people, clear documentation of observed cases with treatment failures and successes is key to building evidence for future management.

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Magnetic Resonance Findings in Recurrent Painful Ophthalmoplegic Neuropathy with Reversible Enhancement of Oculomotor Nerve

Electronic Journal of General Medicine ◽

10.15197/sabad.1.12.11 ◽

2015 ◽

Vol 12 (1) ◽

Cited By ~ 1

Author(s):

Ali Sami Kivrak ◽

Alaaddin Nayman ◽

Yahya Paksoy ◽

Serefnur Ozturk

Keyword(s):

Magnetic Resonance ◽

Oculomotor Nerve ◽

Recurrent Painful Ophthalmoplegic Neuropathy

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Proposed modified diagnostic criteria for recurrent painful ophthalmoplegic neuropathy: Five case reports and literature review

Cephalalgia ◽

10.1177/0333102420944872 ◽

2020 ◽

Vol 40 (14) ◽

pp. 1657-1670

Author(s):

Yinglu Liu ◽

Miao Wang ◽

Xiangbing Bian ◽

Enchao Qiu ◽

Xun Han ◽

...

Keyword(s):

Diagnostic Criteria ◽

Cranial Nerve ◽

Current Knowledge ◽

Case Reports ◽

Case Series ◽

Oculomotor Nerve ◽

Multiplanar Reformation ◽

Unilateral Headache ◽

Pubmed Database ◽

Recurrent Painful Ophthalmoplegic Neuropathy

Background Recurrent painful ophthalmoplegic neuropathy (RPON) is an uncommon disorder characterized by recurrent unilateral headache attacks associated with ipsilateral ophthalmoplegia. We intend to study the clinical picture in our case series along with the published literature to discuss the pathogenesis and propose modified diagnostic criteria for recurrent painful ophthalmoplegic neuropathy. Methods We reported five cases diagnosed as ophthalmoplegic migraine/RPON in our medical centers and reviewed the published literature related to RPON from the Pubmed database between 2000 and 2020. In one of these cases, a multiplanar reformation was performed to look at the aberrant cranial nerve. Results The mean onset age for RPON was 22.1 years, and the oculomotor nerve was the most commonly involved cranial nerve (53.9%) in 165 reviewed patients. In most patients, ophthalmoplegia started within 1 week of the headache attack (95.7%, 67/70). Additionally, 27.6% (40/145) of patients presented enhancement of the involved nerve(s) from MRI tests. Finally, 78 patients received corticosteroids, out of which 96.2% benefited from them. Conclusion This is the first time multiplanar reformation has been performed to reveal the distortion of the oculomotor nerve. Modified diagnostic criteria are proposed. We hope to expand the current knowledge and increase the detection of recurrent painful ophthalmoplegic neuropathy in the future.

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Re: Oculomotor nerve tumors masquerading as recurrent painful ophthalmoplegic neuropathy

Cephalalgia ◽

10.1177/0333102415607071 ◽

2015 ◽

Vol 36 (8) ◽

pp. 814-814 ◽

Cited By ~ 1

Author(s):

Robert K Shin ◽

Luis J Mejico ◽

Dan Boghen

Keyword(s):

Oculomotor Nerve ◽

Recurrent Painful Ophthalmoplegic Neuropathy

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Recurrent painful ophthalmoplegic neuropathy: a cause for recurrent third nerve palsy in a child

BMJ Case Reports ◽

10.1136/bcr-2021-246179 ◽

2021 ◽

Vol 14 (11) ◽

pp. e246179

Author(s):

Jayakumari Nandana ◽

Sruthi S Nair ◽

Sachin Girdhar ◽

Soumya Sundaram

Keyword(s):

Nerve Palsy ◽

Immunosuppressive Agents ◽

Rare Condition ◽

Oculomotor Nerve ◽

Third Nerve Palsy ◽

Hunt Syndrome ◽

Recurrent Painful Ophthalmoplegic Neuropathy ◽

Third Nerve

Recurrent painful ophthalmoplegic neuropathy (RPON), previously called ophthalmoplegic migraine, is a rare condition characterised by recurrent episodes of headache and ophthalmoplegia. We report a case of 11-year-old girl with recurrent painful ophthalmoplegia due to isolated right oculomotor nerve involvement. MR brain imaging showed enhancing lesion of cisternal segment of right oculomotor nerve. A possibility of Tolosa Hunt syndrome was considered and she was treated with glucocorticoids, followed by azathioprine due to recurrence. In the fourth episode, she developed migraine headache followed by right third nerve palsy, after which the diagnosis was revised to RPON. She was started on flunarizine along with short-term glucocorticoids. At 1-year follow-up, she remained asymptomatic. RPON should be considered in patients with recurrent third nerve palsy to avoid inadvertent long-term exposure to immunosuppressive agents.

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Recurrent Painful Ophthalmoplegic Neuropathy and Oculomotor Nerve Schwannoma: A Pediatric Case Report with Long-Term MRI Follow-Up and Literature Review

Pain Research and Management ◽

10.1155/2019/5392945 ◽

2019 ◽

Vol 2019 ◽

pp. 1-11 ◽

Cited By ~ 2

Author(s):

Maria Giuseppina Petruzzelli ◽

Mariella Margari ◽

Flora Furente ◽

Maria Carmela Costanza ◽

Anna Rosi Legrottaglie ◽

...

Keyword(s):

Cranial Nerve ◽

Brain Mri ◽

Oculomotor Nerve ◽

Childhood Onset ◽

Mri Findings ◽

Long Term Follow Up ◽

Recurrent Painful Ophthalmoplegic Neuropathy

Background. Recurrent painful ophthalmoplegic neuropathy (RPON), previously known as ophthalmoplegic migraine (OM), is an uncommon disorder with repeated episodes of ocular cranial nerve neuropathy associated with ipsilateral headache. The age of presentation is most often during childhood or adolescence. MRI has a central role in the assessment of the RPON, especially to distinguish orbital, parasellar, or posterior fossa lesions that mimic symptoms of RPON. Actually, oculomotor nerve tumors may be masquerade as RPON so that MRI follow-ups are required to detect the possibility of tumor etiology. Case presentation. We report a 16-year-old boy with a 7-year follow-up and multiple brain MRI data, previously diagnosed as OM. The last brain MRI, performed during an acute phase of oculomotor paresis with ipsilateral headache, showed a nodular lesion described as schwannoma of III cranial nerve. Then, we reviewed the literature on OM and RPON in pediatric age with a focus on brain MRI findings. Conclusions. This review highlights the important role of serial brain MRIs in the long-term follow-up of RPON, especially in the cases with childhood onset, in order to not delay the diagnosis of a possible oculomotor nerve schwannoma.

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Adult-onset recurrent painful ophthalmoplegic neuropathy displaying atypical oculomotor nerve gadolinium-enhancement pattern in the orbit and cavernous sinus

Cephalalgia ◽

10.1177/0333102415583987 ◽

2015 ◽

Vol 36 (2) ◽

pp. 199-200 ◽

Cited By ~ 6

Author(s):

Tsubasa Takizawa ◽

Mamoru Shibata ◽

Hirokazu Fujiwara ◽

Toshihiko Shimizu ◽

Suketaka Momoshima ◽

...

Keyword(s):

Cavernous Sinus ◽

Oculomotor Nerve ◽

Adult Onset ◽

Enhancement Pattern ◽

Gadolinium Enhancement ◽

Recurrent Painful Ophthalmoplegic Neuropathy

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Oculomotor nerve tumors masquerading as recurrent painful ophthalmoplegic neuropathy: Report of two cases and review of the literature

Cephalalgia ◽

10.1177/0333102414558886 ◽

2014 ◽

Vol 35 (9) ◽

pp. 825-830 ◽

Cited By ~ 11

Author(s):

Ryul Kim ◽

Jae-Hyoung Kim ◽

Eunhee Kim ◽

Hee-Kyung Yang ◽

Jeong-Min Hwang ◽

...

Keyword(s):

Cranial Nerves ◽

Oculomotor Nerve ◽

Venous Angioma ◽

Ocular Motor ◽

Review Of The Literature ◽

Mri Findings ◽

Interval Change ◽

Ophthalmoplegic Migraine ◽

Recurrent Painful Ophthalmoplegic Neuropathy

Background In recurrent painful ophthalmoplegic neuropathy (RPON) that was previously termed as ophthalmoplegic migraine, enhancement of the ocular motor cranial nerves could be seen in the cisternal segment during the acute phase. However, various tumors involving the oculomotor nerve may mimic RPON. Methods We report two patients with MRI findings of oculomotor nerve schwannoma who initially presented with RPON, and found through the literature review five more patients with oculomotor nerve tumors that masqueraded as RPON. Results All patients showed an involvement of the oculomotor nerve. The radiological or pathological diagnosis included schwannoma in five, venous angioma in one, and neuromuscular harmatoma in another one. MRIs with gadolinium documented an enhancing nodule involving the cisternal portion of the oculomotor nerve in six of them, which was also observed on follow-up MRIs without an interval change. Conclusions It should be recognized that an incomplete recovery may occur during future attacks in patients with otherwise uncomplicated RPON. Follow-up MRIs are required to detect tumors involving the ocular motor cranial nerves, especially in patients with suspected RPON when the recovery is incomplete.

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