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Clinical symptoms and enzyme measurement (in one patient) excluded 3\u2010PGDH deficiency. Deficiencies in other serine biosynthesis enzymes were highly unlikely on clinical grounds. On basis of the fasting state, ketone bodies and lactate in plasma, urine and CSF, we speculate that reduced serine levels were due to its use as gluconeogenic substrate, conversion to pyruvate by brain serine racemase or decreased L\u2010serine production because of a lack of glucose. These are the first strikingly similar cases of patients with a clear secondary serine deficiency associated with a toxic encephalopathy.<\/jats:p>","DOI":"10.1007\/s10545-010-9067-9","type":"journal-article","created":{"date-parts":[[2010,3,18]],"date-time":"2010-03-18T09:51:38Z","timestamp":1268905898000},"page":"181-185","source":"Crossref","is-referenced-by-count":4,"title":["Fatal cerebral edema associated with serine deficiency in CSF"],"prefix":"10.1002","volume":"33","author":[{"given":"Irene M. L. 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