Chédiak-Higashi syndrome; a lethal familial disease with anomalous inclusions in the leukocytes and constitutional stigmata: report of a case with necropsy.

An additional fatal case of Chediak-Higashi9s syndrome, occurring in the firstborn of a Canadian family, is reported. The parents were not related and the mother has subsequently given birth to a normal healthy child. The clinical features of the Canadian case were in all essential features similar to those reported by Chediak and Higashi. The hematologic abnormalities were identical. In addition to findings referable to the blood dyscrasia, the post-mortem examination disclosed a peculiar type of "encephalomyelitis," probably secondary to a chronic degenerative process.