METHODS Clinical report Family 1 We re-examined the French family first reported in 1961 by Garcin et al, 16 in which members belonging to four generations were affected by ADOAC (fig 1). Patient III. 3, a female born in 1920, suffered from a visual impairment from infancy which worsened from the age of 28. At age 38, her visual acuity was 2/10 in both eyes and ophthalmological examination revealed bilateral optic atrophy and posterior cortical cataract. On neurological examination there was resting and postural tremor of both hands and a mild extrapyramidal rigidity of both upper limbs. She had bilateral pes cavus and absence of deep tendon reflexes in the lower limbs. The cataract was operated on at age 51, when her visual acuity was 1/10 in each eye. Neurological examination at age 83 showed the same extrapyramidal signs as before, but there was no worsening of the disease. Patient IV. 1, a female born in 1943, suffered from decreased visual acuity before the age of 10. At age 15, she had a visual acuity of 5/10 in each eye, bilateral optic atrophy, and bilateral posterior cortical cataract. Neurological examination at age 15 showed mild postural tremor, mild rigidity of both upper limbs, and mild bilateral pes cavus. Her visual acuity gradually decreased to 1/10 in both eyes. Cataract lens implant surgery was done at the ages of 47 and 48. At age 58, she developed bilateral central scotomata. Neurological examination at age 60 showed a mild postural tremor of the upper extremities.