13.10 Recurrent painful ophthalmoplegic neuropathy

Previously used term:

Ophthalmoplegic migraine (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.

Diagnostic criteria:
  1. At least two attacks fulfilling criterion B
  2. Both of the following:
    1. unilateral headache
    2. ipsilateral paresis of one, two or all three ocular motor nerves1
  3. Orbital, parasellar or posterior fossa lesion has been excluded by appropriate investigation
  4. Not better accounted for by another ICHD-3 diagnosis.
Note:

Some data suggest that headache can develop up to 14 days prior to ocular motor paresis.

Comments:

Gadolinium enhancement or nerve thickening can be demonstrated using MRI.

Treatment with corticosteroids is beneficial in some patients.