Abnormal glutamate metabolism in an adult-onset degenerative neurological disorder

Andreas Plaitakis, Soll Berl, Melvin D. Yahr

Research output: Contribution to journalArticlepeer-review

187 Scopus citations


In patients with recessive, adult-onset olivopontocerebellar degeneration associated with a partial deficiency of glutamate dehydrogenase, the concentration of glutamate in plasma was significantly higher than that in controls. Plasma α-ketoglutarate was significantly lower. Oral administration of monosodium glutamate resulted in excessive accumulation of this amino acid in plasma and lack of increase in the ratio of plasma lactate to pyruvate in the glutamate dehydrogenase - deficient patients. Decreased glutamate catabolism may result in an excess of glutamate in the nervous system and cause neuronal degeneration.

Original languageEnglish
Pages (from-to)193-196
Number of pages4
Issue number4542
StatePublished - 1982


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