13.9 Recurrent painful ophthalmoplegic neuropathy

Previously used term:
Ophthalmoplegic migraine.

Repeated attacks of paresis of one or more ocular cranial nerves (commonly the IIIrd), with ipsilateral headache.

Diagnostic criteria:
A. At least two attacks fulfilling criterion B
B. Unilateral headache accompanied by ipsilateral paresis of one, two or all three ocular motor nerves
C. Orbital, parasellar or posterior fossa lesion has been excluded by appropriate investigation
D. Not better accounted for by another ICHD-3 diagnosis.

The old and inappropriate term ophthalmoplegic migraine was rejected because this syndrome is not migrainous but rather a recurrent painful neuropathy.

Recent data suggest that headache can develop up to 14 days prior to ocular motor paresis. Gadolinium enhancement or nerve thickening can be demonstrated using MRI. Treatment with corticosteroids is beneficial in some patients.