MATERIALS AND METHODS

Brain tissue of two patients with MSUD was made available to us. Both patients had been studied on the Pediatric Service of the New York University School of Medicine. Case I, male: died in 1957 at the age of 20 months. DANCIS, LEVITZ and WESTALL (1960) and SILBERMAN et af.(1961) reported the clinical history and pathological findings. He was never placed on a dietary regimen and was malnourished and failed to gain weight normally. The time between death and autopsy was 27 hr. The brain weighed 1200 g. Case 22, male: died in 1961 at the age of 25 days. He arrived in extrernis and failed to respond to all emergency measures including an exchange transfusion. He was subject to intermittent spasms of the extremities during life, failed to suck, and lost weight. The time between death and autopsy was 8 hr. The brain weighed 450 g.