Abstract
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.
Highlights
Includes the recurrent painful ophthalmoplegic neuropathy (RPON), a condition characterized by repeated attacks of paresis of one or more ocular cranial nerves, with ipsilateral headache [1]. is disorder is extremely rare and can occur at any age, but the highest prevalence is in children under the age of 12 years, with the median age at onset about 8 years [5]. e diagnostical definition of Recurrent painful ophthalmoplegic neuropathy (RPON) changed throughout the history of headache classifications. e 1st edition (1987) of the International Classification of Headache Disorders (ICHD) had considered this condition as a migraine variant [6], but in the ICHD-II (2004), the disorder, called “ophthalmoplegic migraine”, was classified in the group of cranial neuralgias and central causes of facial pain [7]
In the contest of this ongoing debate [14] about the classification of RPON, we presented a pediatric case report with a 7-year follow-up and multiple brain magnetic resonance imaging (MRI) data, previously diagnosed as ophthalmoplegic migraine (OM). e last brain MRI during an acute phase of oculomotor paresis with ipsilateral headache showed a nodular lesion described as schwannoma of III cranial nerve
He showed gradual and progressive improvement of headache symptoms in about one week. This is the first case reported in the literature of an adolescent suffering from headache and ophthalmoplegia with a 7-year brain MRI follow-up, raising the question on the relationship between RPON and schwannoma. e evolutive changes in the presentation of symptoms and the different findings over time in brain MRI images observed in this patient repurpose some open issues on the classification and pathophysiological mechanisms of RPON. e patient had his first clinical manifestations of recurrent headaches at the age of 4 years agreeing to diagnostic criteria of migraine without aura
Summary
In the 3rd edition of International Classification of Headache Disorders (ICHD) (2018) has been introduced a chapter named “painful lesions of the cranial nerves and other facial pains” after a consensus between the International Headache Society (IHS) and the International Association for the Study of Pain (IASP) [1]. e IASP defines neuropathic pain (NP) as “pain arising as a direct consequence of a lesion or disease affecting the somatosensory system” [2]. ere is increasing awareness about NP in pediatric chronic pain because there are fewer information available regarding the prevalence of NP in the pediatric population than adults [3], and the affected children experience significant physical, psychological, and social sequelae that affect themselves and family and friends [4]. e chapter “painful lesions of the cranial nerves and other facial pains”includes the recurrent painful ophthalmoplegic neuropathy (RPON), a condition characterized by repeated attacks of paresis of one or more ocular cranial nerves (commonly the III nerve), with ipsilateral headache [1]. is disorder is extremely rare and can occur at any age, but the highest prevalence is in children under the age of 12 years, with the median age at onset about 8 years [5]. e diagnostical definition of RPON changed throughout the history of headache classifications. e 1st edition (1987) of the ICHD had considered this condition as a migraine variant [6], but in the ICHD-II (2004), the disorder, called “ophthalmoplegic migraine” (with the quotes around “migraine” included), was classified in the group of cranial neuralgias and central causes of facial pain [7]. In the contest of this ongoing debate [14] about the classification of RPON, we presented a pediatric case report with a 7-year follow-up and multiple brain MRI data, previously diagnosed as OM. E last brain MRI during an acute phase of oculomotor paresis with ipsilateral headache showed a nodular lesion described as schwannoma of III cranial nerve. 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. E 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. Is 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 Conclusions. is 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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