Ocular myositis

Ocular myositis is a rare unilateral or bilateral inflammation of the extraocular muscles.

We report on nine ocular myositis patients from three families and describe the course.

Patient 1: 13-year-old girl. Movement of right eye painful, particularly when looking to the left. MRT of orbit: inflamed swelling of the right m. rectus med. Therapy of 100 mg Urbason for 5 days, slowly reduced over a period of 8 weeks. Condition improved rapidly within a few days, MRT showed normal right m. rectus int. after 3 months. No relapse.

Patient 2: 15-year-old girl; undulant course over 2 years accompanied by painful eye movement on both sides, left > right, slight protrusio bulbi on left side. MRT of orbit: marked inflammation of entire extraocular muscles, particularly left m. rectus sup. Steroid therapy of 1 mg/kg body weight. Patient was pain-free within 3 days, glancing up to the left remained painful for a further week, in correspondence with the MRT findings of the orbit after 14 days treatment.

Patients 3 to 9: Italian-German family, five women and two men from three generations affected. All patients displayed a highly undulant course of disease, beginning with painful eye movement between 9 and 17 years of age. The grandmother and mother on the Italian side of the family can no longer reliably remember the exact date of onset. Symptoms were arrested in all cases following the birth of the first child. All patients responded to steroids immediately: 1 mg/kg body weight for varying durations. Whereas the boys in the family only experienced one episode, several recurrences were reported for the girls. Two of the three women from the third generation are free of symptoms since taking oral contraceptives.

Ocular myositis is a rare condition occurring most frequently before puberty. Disease progression is markedly undulant and responds immediately to treatment with steroids. Women conceivably experience a more severe progression than men. The observation of continued improvement under the influence of female hormones (contraceptives) is a new development which could indicate a protective effect.