Association of ophthalmoplegic migraine with familial acute anterior uveitis.

In a family with 3 cases of acute anterior uveitis (AAU) the proband had 13 episodes of recurrent AAU associated 4 times with severe, throbbing ipsilateral headache. During one such episode he had an oculomotor palsy, hyperaesthesia of the cornea, and lack of spontaneous pulsations of the central retinal vein in the eye with AAU. An oculomotor palsy occurred twice intermittently between the AAU episodes. Carotid and vertebral angiographies confirmed the diagnosis of ophthalmoplegic migraine. Prostaglandins liberated by trauma, AAU, or other inflammation may be involved as mediators in ophthalmoplegic migraine attacks. All 3 patients with AAU had sacroiliitis and seronegative polyarthritis. One of their siblings had polyarthritis and one incomplete Reiter's disease. All of them were HLA-B27 positive whereas one healthy sibling was HLA-B27 negative. These findings support the hypothesis that HLA-B27 itself or a pleiotropic HLA-B27 linked gene predisposes the carrier to AAU, sacroiliitis, ankylosing spondylitis, and Reiter's disease.