• Acta Neurol. Scand. · Sep 1992

    Case Reports

    Carbamyl phosphate synthetase-1 deficiency discovered after valproic acid-induced coma.

    • H B Verbiest, J S Straver, J P Colombo, J C van der Vijver, and T C van Woerkom.
    • Department of Neurology, Municipal Hospital Leyenburg, The Hague, The Netherlands.
    • Acta Neurol. Scand. 1992 Sep 1;86(3):275-9.

    AbstractValproic acid induced coma is presented in an adult patient without a history of metabolic disease. Liver biopsy revealed a reduction in activity of carbamyl phosphate synthetase-I, an enzyme obligated for transformation of ammonia to urea in the urea cycle. After recovery CT scan follow-up showed marked cerebral atrophy which did not exist prior to the state of coma. Risk factors are discussed.

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