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Phenylpyruvic Oligophrenia

  • Valerie Cowie (a1)


“The study of phenylpyruvic amentia may throw light on the whole problem of mental deficiency.”—Jervis.

Phenylpyruvic oligophrenia is a syndrome in which mental deficiency is accompanied by the excretion of phenylpyruvic acid in the urine.



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Bates, R. M., “Three Cases of Phenylpyruvic Oligophrenia,” J. Ment. Sci., 1938, 85, 273.
Block, R. J., Jervis, G. A., Bolling, D., and Webb, M., “Chemical and Metabolic Studies on Phenylalanine; Amino Acid Content of Tissue Proteins of Normal and Phenylpyruvic Oligophrenic Individuals,” J. Biol. Chem., 1940, 134, 567572.
Brugger, C., “Die Ausscheidung von Phenylbrenztraubensäure im Urin von Schwachsinnigen,” Schweiz. Arch. f. Neurol. u. Psychiat., 1942, 49, 6269.
Cowie, V. A., “The Galactose Tolerance Test in Phenylketonuria,” J. Ment. Sci., 1950, July.
Delay, J., Pichot, P., “Etude de quelques aspects biologiques de l'oligophrénie phénylpyruvique,” Ann. med. Psychol., 1947, ii, 6164.
Iidem, Desgrez, P., and Delbarre, F., “L'oligophrénie phénylpyruvique,” Bull. Mem. Soc. Med. Hâp. Paris, 1946, 62, 300302.
Iidem and Polonowski, M., “L'oligophrénie phénylpyruvique,” Sem. H⊚p. Paris, 1947, 23, 17491758.
Idem, Pichot, P., Delbarre, F., and Tasseel, J., “L'oligophrénie phénylpyruvique; nouvelles observations,” Bull. Mem. Soc. Med. H⊚p. Paris, 1948, 64, 669673.
Embden, G., and Baldes, K., Biochem. Z., 1913, 55, 301.
Föllings, A., “Utzkillelse av fenylpyroduesyre i urinen som stofskiftesanomali forbindelse med imbecilitet,” Nord. med. tideskr., 1934, 8, 1054.
Idem, Mohr, O. L., and Rund, L., “Oligophrenia Phenylpyrouvica; a Recessive Syndrome in Man,” Norske Videnskaps-Akad. i. Oslo Mat. Naturv. Klasse 1944, I945, 13.
Frazier, R. L., “Phenylpyruvic Amentia,” Am. J. Ment. Def., 1947, 51, 577586.
Gibbs, F. A., and Gibbs, E. L., “Phenylpyruvic Amentia,” Atlas of Electroencephalography, 1941.
Himwich, H. E., and Fazekas, J. F., “Cerebral Metabolism in Mongolian Idiocy and Phenylpyruvic Oligophrenia,” Arch. Neurol. Psychiat., 1940, 44, 12131218.
Jervis, G. A., “Inherited Biochemical Alterations in Certain Types of Mental Deficiency,” Proc. Am. Assoc. Ment. Def., 1937a, 61, 101115.
Idem, “Phenylpyruvic Oligophrenia; Introductory Study of 50 Cases of Mental Deficiency Associated with Excretion of Phenylpyruvic Acid,” Arch. Neurol. Psychiat., 1937b, 38, 944963.
Idem, “Metabolic Investigations on Cases of Phenylpyruvic Oligophrenia,” J. Biol. Chem., 1938, 126, 305313.
Idem, “Genetics of Phenylpyruvic Oligophrenia : A Contribution to Study of Influence of Heredity on Mental Defect,” J. Ment. Sci., 1939, 85, 719762.
Idem, Block, R. J., Bolling, D., and Kanze, E., “Chemical and Metabolic Studies on Phenylalanine; Phenylalanine Content of Blood and Spinal Fluid in Phenylpyruvic Oligophrenia,” J. Biol. Chem., 1940, 134, 105113.
Idem. ibid., 1947, 169, 651.
Larcomb, J. M., “Phenylketonuria and the Congenitally Deaf,” J. Pediat., 1939, 14, 348.
Lepow, H., “Oligophrénie phénylpyruvique. Etude clinique et biologique,” Monatschr. f. Psychiat. u. Neurol., 1944, 110, 161.
Medes, G., Biochem. J., 1932, 26, 917.
Morel, F., “Récherches généalogiques sur un cas d'oligophrénie phénylpyruvique,” Arch. Jul. Klaus Stift., 1944, 19.
Munro, T. A., “Phenylketonuria : Data on Forty-seven British Families,” Ann. Eugenics, 1947, 14, 6688.
Idem, Penrose, L. S., and Taylor, G. L., “A Study of the Linkage Relationship between the Genes for Phenylketonuria and the ABO Allelomorphs in Man,” Proc. Seventh Int. Genet. Congre., 1939, C.U.P.
Idem, “The Genetics of Phenylketonuria,” ibid.
Myers, B., “Statistics … of 1,400 London Children,” Brit. J. Child Dis., 1926, 23, 87.
Myle, , Coquet, , Nyssen, and Van Bogaert, , “Deuxieme cas vérifié anatomiquement d'oligophrénie phenylpyruvique,” Monatschr. f. Psychiat. u. Neurol. 1944, 1944.
Myle, G., “L'oligophrénie phénylpyruvique ou maladie de Föllings,” Ann. Med. Psychol., 1945, 11.
Penrose, L. S., “Two Cases of Phenylpyruvic Amentia,” Lancet, 1935a, i, 23.
Idem, “Inheritance of Phenylpyruvic Amentia,” ibid., 1935b, ii, 192.
Idem and Quastel, J. H., “Metabolic Studies in Phenylketonuria,” Biochem. J., 1937, 31, 266.
Idem, “Peripheral Nerve Tumours in a Case of Phenylketonuria,” Lancet, 1939, i. 572.
Idem, “A Contribution to the Genetical Study of Phenylketonuria,” Trans. Roy. Soc. Canada, 1941, 35, Sec. (5), 81.
Idem, “Search for Linkage between ABO Agglutinogens and Phenylketonuria,” Am. J. Ment. Def., 1945, 50, 4.
Idem, “Phenylketonuria : A Problem in Eugenics,” Lancet, 1946, i, 949.
Idem, “Phenylketonuria,” The Biology of Mental Defect, 1949. Sidgwick & Jackson.
Idem and Cowie, , Ann. Eugenics, 1951, 15, 297.
Pichot, , Delay, , Bertagna, , “L'oligophrénie phénylpyruvique,” Extrait des Annales Médico-Psychologiques, no. 3, 1949.
Polonowski, M., Desgrez, P., and Delbarre, F., “Recherches sur l'oligophrénie phénylpyruvique. Methods de dosage de l'acide phénylpyruvique dans des urines,” Bull. Soc. Chim. Biol., 1947, 29, 10491054.
Rhein, M., and Stoeber, R., “Conservation des urines contenant de l'acide phénylpyruvique,” C. R. Mem. Soc. Biol., 1936, 3, 807.
Shay, H., Arch. Int. Med., 1931, 47, 391.
Turpin, R., and Duchene, H., “L'oligophrénie phénylpyruvique—resultat d'une enquête en France,” Sem. H⊚p. Paris, 1945, 21, 345348.
Turpin, R., Dagand, H., Duchene, H., and Delbarre, F., “Presentation clinique d'un malade atteint d'oligophrénie phénylpyruvique,” Ann. Med. Psychol., 1947, ii, 6567.
Williams, D., “New Orientations in Epilepsy,” Brit. Med. J., 1950, i, March 25, p. 685.
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Phenylpyruvic Oligophrenia

  • Valerie Cowie (a1)
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