Ophthalmoplegic "migraine" or recurrent ophthalmoplegic cranial neuropathy: new cases and a systematic review

Abstract

Ophthalmoplegic migraine is a poorly understood neurologic syndrome characterized by recurrent bouts of head pain and ophthalmoplegia. By reviewing cases presenting to our centers in whom the phenotype has been carefully dissected, and systematically reviewing all published cases of ophthalmoplegic migraine in the magnetic resonance imaging (MRI) era, this review sets out to clearly define the syndrome and discuss possible etiologies. We found that in up to one-third of patients, the headache was not migrainous or associated with migrainous symptoms. In three-quarters of the cases involving the third nerve, there was focal nerve thickening and contrast enhancement on MRI. Observational data suggest systemic corticosteroids may be beneficial acutely. The etiology remains unclear, but may involve recurrent bouts of demyelination of the oculomotor nerve. "Ophthalmoplegic migraine" is a misnomer in that it is probably not a variant of migraine but rather a recurrent cranial neuralgia. A more appropriate name might be "ophthalmoplegic cranial neuropathy."

Figure 1

Magnetic resonance images from Case 4: Axial (A) and coronal (B) post-gadolinium fluid-attenuated inversion recovery images showing thickening and enhancement of the cisternal portion of the left oculomotor nerve (arrows). This is the classic imaging finding of ophthalmoplegic migraine.

Figure 2

Age of onset of ophthalmoplegic migraine.