Recurrent painful ophthalmoplegic neuropathy
- the headache localises around one eye and it is followed hours later by a IIIrd or VIth nerve palsy on that side. The nerve palsy gradually improves over several days or weeks.
- a Horner's syndrome may develop after several episodes
- must be distinguished from a posterior communicating artery aneurysm. The aneurysm can compress the IIIrd cranial nerve
- ophthalmoplegic migraine was the previous term - this old and inappropriate term was rejected because this syndrome is not migrainous but rather a recurrent painful neuropathy)
Description: Repeated attacks of paresis of one or more ocular cranial nerves (commonly the IIIrd), with ipsilateral headache.
A. At least two attacks fulfilling criterion B
B. Both of the following:
1. unilateral headache
2. ipsilateral paresis of one, two or all three ocular motor nerves1
C. Orbital, parasellar or posterior fossa lesion has been excluded by appropriate investigation
D. Not better accounted for by another ICHD-3 diagnosis
1. Some 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.
- Ashina M. Migraine. N Engl J Med 2020;383:1866-76. DOI: 10.1056/NEJMra1915327
- Headache Classification Committee of the International Headache Society (IHS). The international classification of headache disorders, 3rd edition. Cephalalgia 2018; 38: 1-211
Last edited 11/2020 and last reviewed 11/2020