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Metabolic Disorders

  • Chapter
Neuropsychiatric Features of Medical Disorders

Part of the book series: Critical Issues in Psychiatry ((CIPS))

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Abstract

The term porphyria refers to a group of diseases, each with characteristic manifestations, that have in common excessive excretion of one or more porphyrins, porphyrinogens, and/or porphyrin precursors in the urine and/ or feces. The term porphyrinuria refers to porphyrins appearing in the urine and is thus a sign rather than a disease. Classification of these disorders is not entirely satisfactory, but most commonly they are separated into two general groups. The first is erythropoietic porphyria, a blood disorder in which excessive quantities of porphyrins are accumulated in the normoblasts and erythrocytes, the blood forming tissues. The second group is that of the hepatic porphyrias, which includes porphyria variegata, porphyria cutanea tarda, hereditary coproporphyria, and acute intermittent porphyria. It is this latter syndrome that has the most important psychiatric implications and, consequently, is the one most often described in the psychiatric literature.

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References

  • Ackner B, Cooper JE, Gray CH, et al: Acute porphyria: A neuropsychiatric and biochemical study. J Psychosom Res 6: 1–24, 1962.

    Article  PubMed  CAS  Google Scholar 

  • Atcheson SG, Ward JR: Ptosis and weakness after start of D-penicillamine therapy. Ann Intern Med 89: 939–940, 1978.

    PubMed  CAS  Google Scholar 

  • Becker DM, Kramer S: The neurological manifestations of porphyria: A review. Medicine 56: 411–422, 1977.

    Article  PubMed  CAS  Google Scholar 

  • Broomfield B: Acute intermittent porphyria treated with chlorpromazine. Proc R Soc Med 55: 799–800, 1962.

    PubMed  CAS  Google Scholar 

  • Carney MP: Hepatic porphyria with mental symptoms. Lancet 2: 100–101, 1972.

    Article  PubMed  CAS  Google Scholar 

  • Cartwright GE: Disorders of porphyrin metabolism, In Thorn GW, Adams RD, Braunwald E, et al (eds): Harrison’s Principals of Internal Medicine, ed 8. New York, McGraw-Hill, 1977a, pp 655–661.

    Google Scholar 

  • Cartwright GE: Hepatolenticular degeneration (Wilson’s disease), In Thorn GW, Adams RD, Braunwald E, et al (eds): Harrison’s Principals of Internal Medicine ed 8. New York, McGraw-Hill, 1977b, pp 661–664.

    Google Scholar 

  • Cartwright GE: Diagnosis of treatable Wilson’s disease. N Engl J Med 298: 1347–1350, 1978.

    Article  PubMed  CAS  Google Scholar 

  • Cashman MD: Psychiatric aspects of acute porphyria. Lancet 1: 115–116, 1961.

    Article  PubMed  CAS  Google Scholar 

  • Copeman SM: In Proceedings of the Pathological Society of London. Lancet 1: 197–200, 1891.

    Google Scholar 

  • Cross TN: Porphyria-A deceptive syndome. Am J Psychiatry 112: 1010–1014, 1956.

    PubMed  CAS  Google Scholar 

  • Denny-Brown D: Hepatolenticular degeneration (Wilson’s disease), two different components. N Engl J Med 270: 1149–1156, 1964.

    Article  PubMed  CAS  Google Scholar 

  • Dobyns WB, Goldstein NP, Gordon H: Clinical spectrum of Wilson’s disease (hepatolenticular degeneration). Mayo Clin Proc 54: 35–42, 1979.

    PubMed  CAS  Google Scholar 

  • Duret-Cosyns S, Duret RL: Etudy psychiatrique de la porphyrie essentielle. Ann Med-Psychol 2: 193–197, 1959.

    Google Scholar 

  • Ebaugh FG, Holt JW: Porphyria and neuronal dysfunction. Am J Med Sci 245: 95–108, 1963.

    Google Scholar 

  • Eilenberg MD, Scobie BA: Prolonged neuropsychiatric disability and cardio-myopathy in acute intermittent porphyria. Lancet 1: 858–860, 1960.

    CAS  Google Scholar 

  • Eldahl A: A case of acute porphyria developing during hospitalization. Acta Med Scand 97: 415–417, 1938.

    Article  Google Scholar 

  • Fleming CR, Dickson ER, Wahner HW, et al: Pigmented corneal rings in non-Wilsonian liver disease. Ann Intern Med 86: 285–288, 1977.

    PubMed  CAS  Google Scholar 

  • Francone CA: “My battle against Wilson’s disease.” Am J Nurs 76: 247–249, 1976.

    PubMed  CAS  Google Scholar 

  • Goldstein NP, Ewert JC, Randall RV, et al: Psychiatric aspects of Wilson’s disease (hepatolenticular degeneration): Results of psychometric tests during long-term therapy. Am J Psychiatry 124: 1555–1561, 1968.

    PubMed  CAS  Google Scholar 

  • Gunther H: Porphyrie (Hamatoporphyrie). Neue Dtsch Klin 14: 256–260, 1936.

    Google Scholar 

  • Hirsch S, Dunsworth FA: An interesting case of porphyria. Am J Psychiatry 111: 703, 1955.

    PubMed  CAS  Google Scholar 

  • Hoogenraad TU, Vanden Hammer CJA, Koevoet R, et al: Oral zinc in Wilson’s disease. Lancet 2: 1262, 1978.

    Article  PubMed  CAS  Google Scholar 

  • Keeler MH: A case of acute intermittent porphyria with hallucinations of geometric forms. J Nery Ment Dis 134: 572–574, 1962.

    Article  CAS  Google Scholar 

  • Luby ED, Ware JG, Senf R, et al: Stress and the precipitation of acute intermittent porphyria. Psychosom Med 21: 34, 1959.

    PubMed  CAS  Google Scholar 

  • Macalpine I, Hunter R: The “insanity” of King George III: A classic case of porphyria. Br Med J 1: 65–71, 1966.

    Article  PubMed  CAS  Google Scholar 

  • McEwin R, Lawn J, Jonas CT: Survey of porphyria among psychiatric patients. Med J Aust 2: 303–306, August 1972.

    PubMed  CAS  Google Scholar 

  • Mann J: Acute porphyria provoked by barbiturates given with electroshock therapy. Am J Psychiatry 118: 509–511, 1961.

    PubMed  CAS  Google Scholar 

  • Melby JD, Street JP, Watson CJ: Chlorpromazine in the treatment of porphyria. DAMA 162: 174–178, 1956.

    CAS  Google Scholar 

  • Monaco RN, Tuper RD, Robbins JJ, et al: Intermittent acute porphyria: Treatment with chlorpromazine. N Engl J Med 256: 309–311, 1957.

    Article  PubMed  Google Scholar 

  • Olmstead EG: The neuropsychiatric aspects of abnormal porphyrin metabolism. J Nery Ment Dis 117: 300–302, 1953.

    Article  CAS  Google Scholar 

  • Rose JA, Hellman ES, Tschudy DP: Effective diet on the induction of experimental prophyria. Metabolism 10: 514–518, 1961.

    PubMed  CAS  Google Scholar 

  • Roth N: The neuropsychiatric aspects of porphyria. Psychosom Med 7: 291–301, 1945.

    Google Scholar 

  • Roth N: The psychiatric syndromes of porphyria. Int J Neuropsychiatry 4: 32–44, 1968.

    PubMed  CAS  Google Scholar 

  • Scheinberg IH: A psychogenetic anecdote. Psychosom Med 37: 368–371, 1975.

    PubMed  CAS  Google Scholar 

  • Scheinberg IH, Sternlieb I: Wilson’s disease. Annu Rev Med 16: 119–134, 1965.

    Article  PubMed  CAS  Google Scholar 

  • Schneck JM: Porphyria: Neuropsychiatric aspects in the case of a Negro. J Nery Ment Dis 104: 432–434, 1946.

    Article  CAS  Google Scholar 

  • Sternlieb IS, Scheinberg IH: Penicillamine therapy for hepatolenticular degeneration. DAMA 189: 748–754, 1964.

    CAS  Google Scholar 

  • Strickland GT, Frommer D, Leu M-L, et al: Wilson’s disease in the United Kingdom and Taiwan. Quart J Med 42: 619–638, 1973.

    PubMed  CAS  Google Scholar 

  • Strickland GT, Leu M-L: Wilson’s disease: Clinical and laboratory manifestations in 40 patients. Medicine (Baltimore) 54: 113–137, 1975.

    CAS  Google Scholar 

  • Trafford PA: Homicide in acute porphyria. Forensic Sci 7: 113–120, 1976.

    Article  PubMed  CAS  Google Scholar 

  • Vischer JS, Aldrich CK: Acute intermittent porphyria: A case study. Psychosom Med 16: 163–168, 1954.

    Google Scholar 

  • Waldenstrom J: Studien uber porphyrie. Acta Med Scand, supp 82, 1937.

    Google Scholar 

  • Werlin SL, Grand RJ, Perman JA, et al: Diagnostic dilemmas of Wilson’s disease: Diagnosis and treatment. Pediatrics 62: 47–51, 1978.

    PubMed  CAS  Google Scholar 

  • Wetterberg L, Osterberg E: Acute intermittent porphyria: A psychometric study of 25 patients. J Psychosom Res 13: 91–93, 1969.

    Article  PubMed  CAS  Google Scholar 

  • Whittaker SRF, Whitehead TP: Acute and latent porphyria. Lancet 1: 547–551, 1956.

    Article  Google Scholar 

  • Wilson SAK: Progressive lenticular degeneration: A familial nervous disease associated with cirrhosis of the liver. Brain 34: 296–509, 1912.

    Article  Google Scholar 

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Authors and Affiliations

  1. University of Wisconsin Medical School, Madison, Wisconsin, USA

    James W. Jefferson M.D. & John R. Marshall M.D.

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  1. James W. Jefferson M.D.
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  2. John R. Marshall M.D.
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© 1981 Plenum Publishing Corporation

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Jefferson, J.W., Marshall, J.R. (1981). Metabolic Disorders. In: Neuropsychiatric Features of Medical Disorders. Critical Issues in Psychiatry. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-3920-5_8

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  • DOI: https://doi.org/10.1007/978-1-4684-3920-5_8

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4684-3922-9

  • Online ISBN: 978-1-4684-3920-5

  • eBook Packages: Springer Book Archive

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