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Abstract Background Tolosa-Hunt syndrome (THS) and recurrent painful ophthalmoplegic neuropathy (RPON) are rare diseases reported within the “Painful lesions of the cranial nerves” section of the International Classification of Headache Disorders-3rd edition (ICHD-3). In case of a first painful attack, differential diagnosis could be challenging and many pitfalls are due to the rarity of the disorders and the lack of information about correct medical management in youngsters. Case presentation Our main purpose was to report a new case of THS and a new case of RPON describing management and diagnostic investigation at the time of the first episode. In both cases of THS (13 years old) and RPON (14 years old) a unilateral periorbital headache associated with acute onset of ipsilateral third cranial nerve paresis, scarcely responding to non-steroidal anti-inflammatory drugs (NSAID), was present at the beginning of the first attack. Brain MRI with "time-of-flight" (TOF) angiography and the need to administer steroids (after 72 h from onset) in order to stop pain were the most important handles allowing us to adopt the correct management both in THS or RPON since onset and to face recurrences in RPON by avoiding useless therapy during follow-up. Conclusion Unilateral periorbital headache associated with third-fourth or sixth cranial nerve paresis should ideally be investigated with a full work-up, comprehensive of brain MRI with TOF angiography since the first attack. In cases with negative brain MRI spontaneous resolution should be considered and watchful waiting might be advisable before starting steroid therapy.

Background: Tolosa-Hunt syndrome (THS) and recurrent painful ophthalmoplegic neuropathy (RPON) are rare diseases reported within the "Painful lesions of the cranial nerves" section of the International Classification of Headache Disorders-3rd edition (ICHD-3). In case of a first painful attack, differential diagnosis could be challenging and many pitfalls are due to the rarity of the disorders and the lack of information about correct medical management in youngsters. Case presentation: Our main purpose was to report a new case of THS and a new case of RPON describing management and diagnostic investigation at the time of the first episode. In both cases of THS (13 years old) and RPON (14 years old) a unilateral periorbital headache associated with acute onset of ipsilateral third cranial nerve paresis, scarcely responding to non-steroidal anti-inflammatory drugs (NSAID), was present at the beginning of the first attack. Brain MRI with "time-of-flight" (TOF) angiography and the need to administer steroids (after 72 h from onset) in order to stop pain were the most important handles allowing us to adopt the correct management both in THS or RPON since onset and to face recurrences in RPON by avoiding useless therapy during follow-up. Conclusion: Unilateral periorbital headache associated with third-fourth or sixth cranial nerve paresis should ideally be investigated with a full work-up, comprehensive of brain MRI with TOF angiography since the first attack. In cases with negative brain MRI spontaneous resolution should be considered and watchful waiting might be advisable before starting steroid therapy. [ABSTRACT FROM AUTHOR]

Recurrent painful ophthalmoplegic neuropathy is a rare clinical syndrome, included into the International Classification of Headache Disorders, 3rd edition, and characterized by recurrent headache episodes associated with dysfunction of one or more ocular motor nerves. The diagnosis implies mandatory exclusion of other potential causes of the symptoms. The paper presents a clinical case of a 49-year old female patient with a unilateral headache paroxysms and subsequent paresis of the oculomotor nerve. A thorough clinical assessment, with neuroimaging (brain magnetic resonance imaging and magnetic resonance angiography of arteries and veins of the head) showed no morphological abnormalities that could cause clinical symptoms of oculomotor neuropathy. Her past history was remarkable for a similar episode of prolonged unilateral headache and oculomotor paresis about 10 years before, which were at that time considered as an acute cerebrovascular accident. Thus, two episodes of similar clinical signs of painful neuropathy, the absence of other possible causes of the disease, and the complete regression of symptoms after treatment (ibuprofen and gabapentin) confirmed the diagnosis of recurrent painful ophthalmoplegic neuropathy.

Abstract Background Ophthalmoplegic migraine, renamed "Recurrent Painful Ophthalmoplegic Neuropathy" (RPON) in 2013 by the International Headache Society is a rare neurologic disorder characterized by recurrent attacks of ophthalmoplegia associated to ipsilateral headache. The etiology is still unknown. Typical magnetic resonance imaging findings show a focal nerve thickening and contrast enhancement. In the majority of cases, there is a full recovery within days or weeks. There is no evidence supporting a specific treatment. The review defines the characteristics of the recurrent painful ophthalmoplegic neuropathy in patients within 2 years of age underlying the importance of the role of magnetic resonance imaging even in presence of the first attack. Thus, an emblematic case report is presented. Case presentation The authors present a case of third cranial nerve paresis in a 17-month-old male child, presenting a neuroradiological pattern highly suggestive of schwannoma, aneurism or recurrent painful ophthalmoplegic neuropathy. Thus, a review of the literature with the pediatric casuistry of recurrent painful ophthalmoplegic neuropathy occurred within 2 years of age focusing on diagnostic considerations is presented. The authors highlight the importance to consider recurrent painful ophthalmoplegic neuropathy in presence of magnetic resonance imaging findings and clinical symptoms referable to aneurysm or schwannoma. Thus, the review defines the characteristics and the neuroradiological findings at the first RPON attack occurred under 2 years of age. Conclusion Although two attacks are necessary, the review strongly suggests to consider recurrent painful ophthalmoplegic neuropathy even at the first attack, in presence of described characteristics and the aforementioned magnetic resonance imaging findings.

Introduction: Recurrent Painful Ophthalmoplegic Neuropathy, previously known as Ophthalmoplegic Migraine, is a poorly characterized disorder mainly because there are few cases described. We report a new case of Recurrent Painful Ophthalmoplegic Neuropathy and a review of the literature to contribute to increasing the knowledge of the clinical features of this disorder. Case report and review of literature: A 45-year-old woman presented with adult-onset recurrent attacks of abducens and oculomotor palsy associated with diplopia followed by headache. Most notably, pain always presented many days after oculomotor impairment, a feature never described in the literature. A diagnosis of possible Recurrent Painful Ophthalmoplegic Neuropathy was made after excluding other possible mimicking disorders. Symptoms usually resolved gradually with corticosteroid therapy, albeit without a clear-cut benefit. Clinical data collected from 1989 to 2022 showed that adult onset in Recurrent Painful Ophthalmoplegic Neuropathy is not uncommon. While III cranial nerve palsy is typical, VI and IV nerve palsy have also been described. Pathophysiology and diagnosis: Several hypotheses have been proposed, including nerve compression, ischemia or inflammation/demyelination, but none has been completely accepted. Diagnosis remains of exclusion; magnetic resonance imaging and blood exams are key in differential diagnosis. Conclusions: Our case gives us the possibility to expand the clinical features of Recurrent Painful Ophthalmoplegic Neuropathy, also contributing to updating the pathophysiological hypotheses. [ABSTRACT FROM AUTHOR]

Background: Ophthalmoplegic migraine, renamed "Recurrent Painful Ophthalmoplegic Neuropathy" (RPON) in 2013 by the International Headache Society is a rare neurologic disorder characterized by recurrent attacks of ophthalmoplegia associated to ipsilateral headache. The etiology is still unknown. Typical magnetic resonance imaging findings show a focal nerve thickening and contrast enhancement. In the majority of cases, there is a full recovery within days or weeks. There is no evidence supporting a specific treatment. The review defines the characteristics of the recurrent painful ophthalmoplegic neuropathy in patients within 2 years of age underlying the importance of the role of magnetic resonance imaging even in presence of the first attack. Thus, an emblematic case report is presented. Case presentation: The authors present a case of third cranial nerve paresis in a 17-month-old male child, presenting a neuroradiological pattern highly suggestive of schwannoma, aneurism or recurrent painful ophthalmoplegic neuropathy. Thus, a review of the literature with the pediatric casuistry of recurrent painful ophthalmoplegic neuropathy occurred within 2 years of age focusing on diagnostic considerations is presented. The authors highlight the importance to consider recurrent painful ophthalmoplegic neuropathy in presence of magnetic resonance imaging findings and clinical symptoms referable to aneurysm or schwannoma. Thus, the review defines the characteristics and the neuroradiological findings at the first RPON attack occurred under 2 years of age. Conclusion: Although two attacks are necessary, the review strongly suggests to consider recurrent painful ophthalmoplegic neuropathy even at the first attack, in presence of described characteristics and the aforementioned magnetic resonance imaging findings. [ABSTRACT FROM AUTHOR]

Abstract Background Abducens nerve is the most common nerve affected among other cranial nerves in terms of isolated ocular palsy. Despite its prevalence and progression made in neuroimaging, incidence of idiopathic cases continues to rise. Case presentation We report a young adult male with no previous illnesses, who presented with sudden onset of binocular diplopia and headache and was later diagnosed with an isolated ipsilateral abducens nerve palsy secondary to an unknown cause and treated with steroids. The palsy and diplopia had resolved in 2-month time but the patient still continued to suffer from a migraine, such as headache. Conclusion Although, we labelled the patient as idiopathic initially, another plausible cause for this kind of presentation could be recurrent painful ophthalmoplegic neuropathy (RPON) owing to the nature of the presentation. Cases labelled as idiopathic could be attributed to a defined pathogenesis with detailed history and follow-up. Follow-up is vital for all patients, especially those labelled as idiopathic as recurrence of the illness have the chance of changing the diagnosis if supported by the detailed history and physical examination findings.

The article presents a case study of a 18-year-old male with right eyelid ptosis and horizontal diplopia. Topics include developing severe throbbing and sharp pain along the right frontal and lateral aspect of the head, associated with photophobia; and denied blurry vision, visual loss, speech difficulty, and weakness of his extremities.

Recurrent painful ophthalmoplegic neuropathy (RPON) is a rare disorder, which typically occurs in children, and causes headaches and unilateral oculomotor palsy. Early high-dose corticosteroid therapy is recommended to rapidly resolve acute episodes. However, the pathophysiology and therapeutic options for this disorder remain to be fully elucidated. We report a case with typical clinical features of RPON successfully treated with beta-blocker eye drop instillation after the effects of high-dose corticosteroid and other therapies were not sufficient. We propose that beta-blocker eye drop instillation should be considered for patients with corticosteroid-resistant RPON. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Abducens nerve is the most common nerve affected among other cranial nerves in terms of isolated ocular palsy. Despite its prevalence and progression made in neuroimaging, incidence of idiopathic cases continues to rise. Case presentation: We report a young adult male with no previous illnesses, who presented with sudden onset of binocular diplopia and headache and was later diagnosed with an isolated ipsilateral abducens nerve palsy secondary to an unknown cause and treated with steroids. The palsy and diplopia had resolved in 2-month time but the patient still continued to suffer from a migraine, such as headache. Conclusion: Although, we labelled the patient as idiopathic initially, another plausible cause for this kind of presentation could be recurrent painful ophthalmoplegic neuropathy (RPON) owing to the nature of the presentation. Cases labelled as idiopathic could be attributed to a defined pathogenesis with detailed history and follow-up. Follow-up is vital for all patients, especially those labelled as idiopathic as recurrence of the illness have the chance of changing the diagnosis if supported by the detailed history and physical examination findings. [ABSTRACT FROM AUTHOR]

Recurrent painful ophthalmoplegic neuropathy (RPON) replaced the term 'ophthalmoplegic migraine' as the condition behaves more like an inflammatory cranial neuropathy than a primary headache disorder. RPON may be associated with cranial nerve thickening on MR imaging and persistent oculomotor paresis. Oculomotor tumours have rarely been described in cases of relapsing painful ophthalmoplegia with and without persistent paresis. Here, we present a case of relapsing painful left ophthalmoplegia that gradually became persistent. MR imaging after 14 years of symptoms revealed an enhancing tumour of the left oculomotor nerve. It is unclear whether the tumour was the cause of the attacks or whether repeated cycles could lead to tumour development. MR imaging is indicated in patients with RPON who develop persistent deficits to screen for associated oculomotor tumour. [ABSTRACT FROM AUTHOR]

Premise This review summarises the pain syndromes that overlap between headache and facial pain and overlap between pain and cranial nerve lesion. Problem These syndromes share two features in common. First, they show both cranial nerve impairment (e.g. palsy, autonomic dysfunction) and pain; second, they have inflammatory (and/or small vessel) processes as the underlying mechanism. A typical representative of these syndromes is recurrent painful ophthalmoplegic neuropathy, which was previously called ophthalmoplegic migraine and was regarded as a migraine subtype. It is now supposed that this syndrome is caused by an inflammation of one of the ocular motor nerves. Other syndromes discussed in this review are optic neuritis, Tolosa-Hunt syndrome, ischaemic ocular motor nerve palsy, and the very rare Raeder's syndrome. Treatment of all these syndromes is mainly based on steroids. Potential solution Management of possibly underlying disorders such as multiple sclerosis or atherosclerosis should also be considered. [ABSTRACT FROM AUTHOR]

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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 schwannomawho 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. [ABSTRACT FROM AUTHOR]

A case study is presented of a 12-year-old boy presented with diplopia and painful ophthalmoplegic neuropathy. Physical examination revealed ptosis, exotropia and mydriasis; and gadolinium enhanced magnetic resonance imaging showed enhancement of the right oculomotor nerve. He was diagnosed of the same using prednisolone drug.

Recurrent painful ophthalmoplegic neuropathy (RPON), formerly named ophthalmoplegic migraine (OM), is a rare condition characterized by the association of unilateral headaches and the ipsilateral oculomotor nerve palsy. The third cranial nerve is most commonly involved in the recurrent attacks. But it is still debated whether a migraine or an oculomotor neuropathy may be the primary cause of this disorder. Here, we report an elder patient who had a recurrent ophthalmoplegia starting with an unilateral headache circumscribed in an area shaped in a line linking the posterior-parietal region and the ipsilateral eye. And the headache had couple of features similar to that of migraine, such as past history of recurrent migraine attacks, accompaniments of nausea, vomiting, and phonophobia, response to flunarizine and sodium valproate. We may herein report a subtype of OM but not a RPON. This case report indicates that OM may exist as an entity and some OM may be wrongly grouped under the category of RPON in the current international headache classification. [ABSTRACT FROM AUTHOR]

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.

The patient was a 14-year-old boy with two previous episodes of self-remitting right ophthalmoplegia with right temporal pain at ages 9 and 12. In 2019, he developed right eyelid ptosis and diplopia 2 days after a pulsating right-sided temporoparietal headache. Recurrent headaches with ophthalmoplegia responded to high-dose steroid therapy, and the clinical features resembled recurrent painful ophthalmoplegic neuropathy (RPON). RPON generally presents with MRI findings of hypertrophy and inflammation at the root of the oculomotor nerve, a vulnerable site of the blood-brain barrier. However, the imaging features in this case were different from those in typical cases of RPON, and oculomotor nerve inflammation was found in the cavernous sinus. The order of onset of headache and oculomotor nerve palsy differed in each recurrence, suggesting that both autoimmune and vascular mechanisms may have been involved in the onset of the disease in our case.

Recurrent painful ophthalmoplegic neuropathy is a rare disorder characterized by repeated attacks of paresis of one or more ocular cranial nerves, with ipsilateral headache. We describe a 24-year-old man with recurrent painful ophthalmoplegic neuropathy and oculomotor nerve palsy, in whom clinical features and magnetic resonance imaging findings on pre and post treatment period. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 12-year-old boy who was diagnosed with recurrent painful ophthalmoplegic neuropathy (RPON).and headache. Mentioned are results of the magnetic resonance imaging (MRI) on the patient's pathogenesis and prophylaxis. Also tackled are benefits of corticosteroids on RPON.

Recurrent painful ophthalmoplegic neuropathy (RPON) is a rare condition that manifests as headache and ophthalmoplegia. It typically occurs in children. Although migraine or neuropathy have been suggested as etiologies, the precise etiology remains unclear. In the International Classification of Headache Disorders 3rd edition-beta version (ICHD3β) (code 13.9), RPON was categorized into painful cranial neuropathies and other facial pains. We encountered a 48-year-old woman who had diplopia and right ptosis. The administration of prednisolone led to the immediate improvement of her oculomotor palsy, but residual mydriasis remained. Based on this case, the pathophysiology of RPON may involve temporary nerve inflammation with migraine. Repeated and severe migraine attacks may cause irreversible nerve damage. Thus, medication for migraine prophylaxis might be needed to prevent RPON.

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., (© International Headache Society 2014.)