Porphyria: Difference between revisions
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===Historical patients=== | ===Historical patients=== | ||
The insanity exhibited by [[George III of the United Kingdom|King George III]] evidenced in the regency crisis of 1788 has inspired several attempts at retrospective diagnosis. The first, written in 1855, thirty-five years after his death, concluded he suffered from acute mania. M. Guttbacker, in 1941, suggested manic-depressive psychosis as a more likely diagnosis, The first suggestion that a physical illness was the cause of King George's mental derangements came in 1966, in a paper "The Insanity of King George III: A Classic Case of Porphyria"<ref>Ida Macalpine & Richard Hunger, "The Insanity of King George III: A Classic Case of Porphyria", ''British Medical Journal'', 1966, pp. 65-71.</ref>, with a follow-up in 1968, "Porphyria in the Royal Houses of Stuart, Hanover and Prussia"<ref>Ida Macalpine, Richard Hunger, & Claude Rimington, "Porphyria in the Royal Houses of Stuart, Hanover and Prussia: A Followup Study of George III's Illness", ''British Medical Journal'', 1968, pp. 7-18.</ref>. The papers, by a mother/son psychiatrist team, were written as though the case for porphyria had been proven, but the response demonstrated that many, including those more intimately familiar with actual manifestations of porphyria, were unconvinced. The theory is treated in ''Purple Secret''<ref>Röhl, John C.G., Warren Martin,& David Hunt, ''Purple Secret'', Bantam Press, London, 1998 ISBN 0-593-04148-8</ref>, which documents the ultimately unsuccessful search for genetic evidence of porphyria in the remains of royals suspected to suffer from it.<ref>The authors demonstrated a single point mutation in the PPOX gene, but not one which has been associated with disease.</ref> In 2005 it was suggested that [[arsenic]] (which is known to be porphyrogenic) given to George III with antimony may have caused his porphyria.<ref>Cox TM, Jack N, Lofthouse S, Watling J, Haines J, Warren MJ. King George III and porphyria: an elemental hypothesis and investigation. ''Lancet'' 2005;366(9482):332-5. PMID 16039338.</ref> Despite the lack of direct evidence, the notion that George III (and other members of the royal family) suffered from porphyria has achieved such popularity that many forget that it is merely a hypothesis. | The insanity exhibited by [[George III of the United Kingdom|King George III]] evidenced in the regency crisis of 1788 has inspired several attempts at retrospective diagnosis. The first, written in 1855, thirty-five years after his death, concluded he suffered from acute mania. M. Guttbacker, in 1941, suggested manic-depressive psychosis as a more likely diagnosis, The first suggestion that a physical illness was the cause of King George's mental derangements came in 1966, in a paper "The Insanity of King George III: A Classic Case of Porphyria"<ref>Ida Macalpine & Richard Hunger, "The Insanity of King George III: A Classic Case of Porphyria", ''British Medical Journal'', 1966, pp. 65-71.</ref>, with a follow-up in 1968, "Porphyria in the Royal Houses of Stuart, Hanover and Prussia"<ref>Ida Macalpine, Richard Hunger, & Claude Rimington, "Porphyria in the Royal Houses of Stuart, Hanover and Prussia: A Followup Study of George III's Illness", ''British Medical Journal'', 1968, pp. 7-18.</ref>. The papers, by a mother/son psychiatrist team, were written as though the case for porphyria had been proven, but the response demonstrated that many, including those more intimately familiar with actual manifestations of porphyria, were unconvinced. The theory is treated in ''Purple Secret''<ref>Röhl, John C.G., Warren Martin,& David Hunt, ''Purple Secret'', Bantam Press, London, 1998 ISBN 0-593-04148-8</ref>, which documents the ultimately unsuccessful search for genetic evidence of porphyria in the remains of royals suspected to suffer from it.<ref>The authors demonstrated a single point mutation in the PPOX gene, but not one which has been associated with disease.</ref> In 2005 it was suggested that [[arsenic]] (which is known to be porphyrogenic) given to George III with antimony may have caused his porphyria.<ref>Cox TM, Jack N, Lofthouse S, Watling J, Haines J, Warren MJ. King George III and porphyria: an elemental hypothesis and investigation. ''Lancet'' 2005;366(9482):332-5. PMID 16039338.</ref> Despite the lack of direct evidence, the notion that George III (and other members of the royal family) suffered from porphyria has achieved such popularity that many forget that it is merely a hypothesis. | ||
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Other commentators have suggested that [[Vincent van Gogh]] may have suffered from acute intermittent porphyria.<ref>Loftus LS, Arnold WN. Vincent van Gogh's illness: acute intermittent porphyria? ''BMJ'' 1991;303:1589-91. PMID 1773180.</ref> | Other commentators have suggested that [[Vincent van Gogh]] may have suffered from acute intermittent porphyria.<ref>Loftus LS, Arnold WN. Vincent van Gogh's illness: acute intermittent porphyria? ''BMJ'' 1991;303:1589-91. PMID 1773180.</ref> | ||
Revision as of 10:51, 13 November 2007
Historical patients
The insanity exhibited by King George III evidenced in the regency crisis of 1788 has inspired several attempts at retrospective diagnosis. The first, written in 1855, thirty-five years after his death, concluded he suffered from acute mania. M. Guttbacker, in 1941, suggested manic-depressive psychosis as a more likely diagnosis, The first suggestion that a physical illness was the cause of King George's mental derangements came in 1966, in a paper "The Insanity of King George III: A Classic Case of Porphyria"[1], with a follow-up in 1968, "Porphyria in the Royal Houses of Stuart, Hanover and Prussia"[2]. The papers, by a mother/son psychiatrist team, were written as though the case for porphyria had been proven, but the response demonstrated that many, including those more intimately familiar with actual manifestations of porphyria, were unconvinced. The theory is treated in Purple Secret[3], which documents the ultimately unsuccessful search for genetic evidence of porphyria in the remains of royals suspected to suffer from it.[4] In 2005 it was suggested that arsenic (which is known to be porphyrogenic) given to George III with antimony may have caused his porphyria.[5] Despite the lack of direct evidence, the notion that George III (and other members of the royal family) suffered from porphyria has achieved such popularity that many forget that it is merely a hypothesis.
It is also suspected that Mary Queen of Scots--George III's grandmother six times removed--also suffered from acute intermittent porphyria, although this is subject to much debate.
Other commentators have suggested that Vincent van Gogh may have suffered from acute intermittent porphyria.[6]
- ↑ Ida Macalpine & Richard Hunger, "The Insanity of King George III: A Classic Case of Porphyria", British Medical Journal, 1966, pp. 65-71.
- ↑ Ida Macalpine, Richard Hunger, & Claude Rimington, "Porphyria in the Royal Houses of Stuart, Hanover and Prussia: A Followup Study of George III's Illness", British Medical Journal, 1968, pp. 7-18.
- ↑ Röhl, John C.G., Warren Martin,& David Hunt, Purple Secret, Bantam Press, London, 1998 ISBN 0-593-04148-8
- ↑ The authors demonstrated a single point mutation in the PPOX gene, but not one which has been associated with disease.
- ↑ Cox TM, Jack N, Lofthouse S, Watling J, Haines J, Warren MJ. King George III and porphyria: an elemental hypothesis and investigation. Lancet 2005;366(9482):332-5. PMID 16039338.
- ↑ Loftus LS, Arnold WN. Vincent van Gogh's illness: acute intermittent porphyria? BMJ 1991;303:1589-91. PMID 1773180.