INFANTILE SUBACUTE NECROTIZING ENCEPHALOPATHY WITH PREDILECTION FOR THE BRAIN STEM：REPORT OF A CASE

　1．A 15-year-old Japanese girl with postmortem findings indicative of "Subacute Necrotizing Encephalopathy" is reported.

　2．Studying the author's own case and reviewing the available literature, it is felt that severe peripheral neuropathy frequently associated with secondary degeneration of the dorsal white columns of the spinal cord constitutes an integral part of the disorder.

　3．The presence of peripheral neuropathy, frequent familial occurrence of the disorder, and consanguinity in some of the patients with this disorder appears to support the speculation of the previous investigators thatthe disease may represent an inborn error of metabolism, possibly enzymatic, especially of the pyruvate metabolism.