楓糖尿症（Branched Chain Ketonuria）の1例—病理学的ならびに神経化学的研究

Pathological and neurochemical studies on a case of maple syrup urine disease (branched chain ketonuria)

　楓糖尿症（MSUD）古典型の1症例について病理学的検索と脳髄指質分析（本学順応医研生化学部門所見）を行なったので報告する。

A case of maple syrup urine disease (branched chain ketonuria) of classical type was examined pathologically and neurochemically.

This male infant died at 4 months, 11 days of age. He was born at term after a normal pre-gnancy. He weighed 3060 g at birth. He was the first child of unrelated and healthy parents, both 28 years old.