[Acute encephalopathy in inherited metabolic diseases].

Acute encephalopathy, regardless of the cause, is a medical emergency. In addition to being a common manifestation of a variety of acquired medical or surgical conditions, it is a presenting feature of number of inherited metabolic diseases, particularly in young children. Because of the importance of identifying treatable inherited metabolic diseases early, initial investigation must not be delayed. In addition, it is important to keep their serum and urine for investigation later.