Purpose or Case Report: Recurrent Painful Ophthalmoplegic Neuropathy (RPON), formerly known as ophthalmoplegic migraine, is a rare pediatric disorder characterized by recurrent episodes of headache followed by ophthalmoplegia involving one or more ocular motor nerves. The International Classification of Headache Disorders (ICHD-3, 2018) now classifies this entity as a cranial neuropathy rather than a migraine variant, reflecting its inflammatory and demyelinating features. This educational exhibit highlights the clinical presentation and MRI features of RPON, emphasizing the imaging role in diagnosis and in distinguishing it from other causes of painful ophthalmoplegia.
Methods & Materials: We reviewed teaching data base at a tertiary care pediatric hospital for pediatric patients presenting with headache and ophthalmoplegia, all of whom underwent detailed clinical evaluation and MRI imaging. MRI protocols included high-resolution steady-state free precession (bFFE) and thin-section post-contrast T1-weighted sequences focused on the cranial nerves. Clinical and imaging findings were correlated to identify consistent features and exclude other etiologies such as aneurysm, schwannoma, and demyelinating disease.
Results: Patients typically presented with ipsilateral headache, diplopia, ptosis, and photophobia. MRI demonstrated focal thickening and enhancement of the affected oculomotor nerve during symptomatic episodes, which resolved or improved on follow-up imaging; findings consistent with transient inflammatory neuropathy. No vascular or mass lesions were identified. The clinical course was self-limited, and all patients improved with conservative management. These MRI findings are key in differentiating RPON from persistent or progressive lesions such as schwannomas, where enhancement does not resolve.
Conclusions: RPON is an uncommon but critical diagnostic consideration in children presenting with painful ophthalmoplegia. Recognition of its reversible MRI features and benign clinical course prevents misdiagnosis and unnecessary interventions. High-resolution MRI sequences are indispensable for detecting subtle cranial nerve changes and guiding accurate diagnosis. Increased awareness among radiologists and pediatric neurologists is essential for early recognition and appropriate management of this underrecognized condition.