In one of two siblings a clinical disorder was described, consisting of a slowly progressive juvenile parkinsonism with extensor plantar responses, external ophthalmoplegia with severe ptosis and a motor and sensory polyneuropathy. The younger sibling had only juvenile parkinsonism, Unilateral ptosis and a motor and sensory polyneuropathy. Their father was neurologically normal except for a unilateral ptosis. There did not seem to be consanguinity in this family.