Sanger Brown (1892) described a kindred with 21 persons in 4 generations who showed symmetric ataxia of gait and limb movement, dysarthria, and pyramidal signs in the limbs. Three had impaired pupillary reaction to light; at least 1 ... Sanger Brown (1892) described a kindred with 21 persons in 4 generations who showed symmetric ataxia of gait and limb movement, dysarthria, and pyramidal signs in the limbs. Three had impaired pupillary reaction to light; at least 1 developed a disorder of conjugate eye movement. The proband had normal development until age 18 years, when he developed an ataxic gait associated with lower limb weakness and hyperreflexia. Upper limbs became involved later. He also reported dysarthria, dysphagia, and difficulties seeing at a distance. Physical examination as an adult showed significant ataxia, ankle clonus, hyperreflexia, and some abnormal movements suggestive of chorea. Ophthalmic examination showed ptosis, slow pupillary reaction, myopia, optic disc pallor, retinal atrophy, and color blindness. His 26-year-old sister developed ataxia in her twenties and also had poor visual acuity in the light. A brother of the 2 had similar features. In another branch of the family, 3 members had adult onset of progressive ataxia, dysarthria, and poor vision. Nystagmus was not present, but many had optic atrophy. Dick et al. (1983) described a mother and 3 of her 5 children (2 males, 1 female) with hereditary spastic ataxia combined with congenital miosis. The affected persons were late in walking unaided and had slurred speech, small nonreacting pupils, and nystagmus. Deep tendon reflexes were increased and the plantar reflexes were often extensor. Timby et al. (2008) reported a Swedish family in which 4 individuals spanning 3 generations had early childhood-onset spastic ataxia and pupillary miosis. Onset of independent walking occurred by age 2 or 3 years, but all showed truncal and gait ataxia. All also had miotic pupils that did not dilate in the dark. Most patients had balance problems and were unable to stand on 1 leg or with closed eyes. Other features included dysdiadochokinesis, hyperreflexia mainly affecting the legs, and mild dysarthria. The ataxia was present from the first years of life, but the miosis started between ages 4 and 16 years.