Malamud and Cohen (1958) described a male infant with developed ataxia at age 10 months after normal early development. He also had head tremor, unilateral sixth nerve palsy, and mental retardation. At age 3 years, he developed spasticity ... Malamud and Cohen (1958) described a male infant with developed ataxia at age 10 months after normal early development. He also had head tremor, unilateral sixth nerve palsy, and mental retardation. At age 3 years, he developed spasticity and extrapyramidal rigidity. He was incontinent and died at age 7 years. Postmortem examination showed severe atrophy of the cerebellum, diffuse small cysts, pale inferior olives, and gliosis. The pons was not atrophied. Purkinje cells in the cerebellum were absent, but the granular layer remained intact. Family history revealed multiple other affected males related through females, consistent with X-linked recessive inheritance.