The condition tends to have an onset during childhood or adolescence. An initial onset after the age of 20 is unusual and should raise doubts about the diagnosis.
Clinical features:
(1) The patient has a history of migraine (see below).
(2) Opthalmolegia occurs that may reflect involvement of the third, fourth and/or sixth cranial nerves.
(3) Slight mydriasis may occur in the pupil of the affected eye.
(4) Cerebral angiography is normal.
(5) exclusion of other explanations
The migraine headache may:
(1) precede the onset of the ophthalmoplegia by hours or days
(2) be diminished or gone by the onset of the ophthalmoplegia
The ophthalmoplegia usually resolves in a few days without residua. With repeated or prolonged episodes it may become permanent.
When to consider another diagnosis:
(1) initial onset after 20 years of age
(2) change in the level of consciousness
(3) signs and symptoms of subarachnoid hemorrhage
(4) absence of a history of migraine or family history of migraine
(5) severe, persistent headache with total ophthalmoplegia
(6) abnormal cerebral angiography
