Rare headache syndromes:: ophthalmoplegic and retinal migraine

Ophthalmoplegic and retinal migraine are rare headache disorders of unknown pathophysiology. Ophthalmoplegic migraine is characterised by a painful ophthalmoplegia, with the headache phase typically preceding the ophthalmoplegia. It occurs most frequent in infants and children. The symptoms of retinal migraine include monocular scotoma and visual loss associated with migraine headache. According to the anamnestic descriptions it is often difficult to separate clearly bilateral homonymous defects from monocular events. Both syndromes are diagnoses of exclusion and require an extensive diagnostic workup. This review summarises the clinical manifestations and the diagnostic evaluation according to the spectrum of differential diagnoses.