From ophthalmoplegic migraine to cranial neuropathy

Ophthalmoplegic migraine (OM)/recurrent painful ophthalmoplegic neuropathy (RPON) is a rare disease consisting of recurrent unilateral headache accompanied or followed by ipsilateral ophthalmoplegia. Because MRI findings suggest neuropathy and the relationship to typical migraine remains unclear, the disease has been renamed from "ophthalmoplegic migraine" to "recurrent painful oculomotor neuropathy" in the third edition of the International Classification of Headache Disorders (ICHD). However, it remains a fact that most cases of OM/RPON described in the literature have a history of migraine and that the headache during OM/RPON often has migrainous features. A more detailed clinical description of the headache during OM/RPON and additional results from imaging and possibly histology will be needed to better understand the pathophysiology of the disease and its relationship to typical migraine.