In 1931 Alpers described a condition in which there was a peculiar form of degeneration of the cerebral grey matter. Subsequently other examples of this disorder in association with epilepsy, spasticity, myoclonus and dementia in early life have been published. These 24 cases, together with eight of their own, have been summarized by Wolf and Cowen (1954) under the name of 'diffuse progressive cerebral cortical atrophy', although the cerebellum and basal ganglia were also involved. As the condition is uncommon it was thought worth while to record five cases, occurring in three families, in which cerebral biopsy was performed; on the four which subsequently died, histological and chemical examination of the brain was carried out.Case Reports Case 1. The health of the parents had been good. There was no known family history of epilepsy or mental illness. An only sibling constitutes the second case of this report.This full-term male infant was the product of the mother's first pregnancy and weighed 6i lb. (2-9kg.). The membranes ruptured 48 hours before delivery and labour lasted 24 hours. The child was shocked at birth and appeared cyanotic one hour later; oxygen was administered. He was apparently well when discharged from hospital on the tenth day. Diphtheria-pertussis inoculations were given at 6, 7 and 8 months, and vaccination was carried out at 10 months, without complications.At 10 months of age the child broke a femur and was in hospital for six weeks. There was no apparent head injury.The parents thought that the child developed normally until the age of 15 months. He sat at 6 months if supported. At 10 months he could form simple phrases but was unable to crawl. However, the clinicians considered from a review of home movies, that motor retardation had begun at about 8 months of age.The first convulsion, which occurred at 15 months, began with extension of the right hand in a claw position; the child 'shivered', fell forward limp and remained unconscious for four hours; clonic movements of the extremities then occurred. The patient was observed in hospital for 10 days; temperature and cerebrospinal fluid were normal. During the next two months the patient became less active and more irritable. A second convulsion, similar to the first, occurred at 17 months, and subsequently there were progressively more frequent episodes ofjerking of the extremities with hyperextension of the back, without loss of consciousness.At 23 months of age focal seizures began. These were characterized by loss of consciousness and twitching of the left side of the face and left arm, each lasting 30 to 60 seconds. These occurred with increasing frequency, being almost continuous when the patient was admitted to hospital.The patient was admitted to the National Hospital, Queen Square, at the age of 24 months, on January 13, 1958, under the care of Dr. E. A. Carmichael. He was a physically well-developed child who was inattentive to his surroundings, did not speak, and was unable to maintain the sitting position. There were frequen...