Liver, fava beans, and a fine chianti
Topic: Pharmacology
Created on Thursday, August 16 2007 by dryman
Last modified on Thursday, August 16 2007.
"I ate his liver with fava beans and a fine chianti." - H. Lecter, M.D.
Hannibal Lecter is cast in a Silence of the Lambs sitcom spinoff complaining of a severe headache, nausea, dizziness, and blurred vision. His skin is a dark yellow, although his sclera are anicteric. Funduscopic evaluation demonstrates swollen, edematous optic nerves bilaterally. LP opening pressure is elevated, but CSF composition is normal and there is no evidence of infection. CT and MRI are both normal. Oddly, he has no eyebrows.
From what neurological or metabolic disorder does Dr. Lecter suffer, and what unusual dietary habits may have led to this presentation?
This question was created on August 16, 2007 by dryman.
This question was last modified on August 16, 2007.
ANSWERS AND EXPLANATIONS
A) Anemia of glucose-6-phosphate dehydrogenase deficiency / ingestion of fava beans
This answer is incorrect.
Glucose-6-phosphate dehydrogenase deficiency is an X-linked recessive genetic disorder predisposing patients to severe hemolytic anemia in response to certain drugs and foods. A particularly severe reaction, known as favism, occurs after exposure to the high levels of glycosides found in broad beans. Jaundice could be a sign of hemolytic anemia secondary to fava exposure, but the rest of Dr Lecter's presentation is not typical of this condition.
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B) Wernicke's encephalopathy / chronic abuse of a nice chianti
This answer is incorrect.
The triad of opthalmoplegia, encephalopathy, and ataxia is consistent with Wernicke's encepthalopathy. Jaundice could be a sign of hepatic cirrhosis secondary to chianti abuse.
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C) Hypervitaminosis A / overconsumption of liver
This answer is correct.
At very high levels of vitamin A consumption (>25000 IU/day), vitamin A toxicity results in decreased CSF absorption, leading to elevated intracranial pressure and papilledema (pseudotumor cerebri). Early symptoms of hypervitaminosis A include sparsely distributed, coarse hair and a specific alopecia of the eyebrows. In this case, apparent jaundice of the skin is due to high levels of carotenoid pigment.
Hypervitaminosis is most often seen due to overuse of vitamin supplements, but can also result from drastic overconsumption of specific foods. While stranded during a 1912 expedition, the Antarctic explorers Mawson and Mertz butchered and ate their sled dogs to survive, but rapidly developed vitamin A poisoning from the nutrient-rich dog livers, fatally in the case of Mertz. (see <a href="http://student.bmj.com/back_issues/0502/life/158.html">http://student.bmj.com/back_issues/0502/life/158.html</a>)
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D) Malingering in order to entrap and eat the examiner / chronic cannibalism
This answer is incorrect.
Although motive perhaps is there, Dr Lecter's physical symptoms would be difficult to simulate. (
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E) Creutzfeldt-Jakob disease / remote ingestion of infected neural tissue
This answer is incorrect.
Creutzfeldt-Jakob disease can occur either due to a genetic defect in the prion protein (PrP) or to consumption of meat containing neural tissue from infected animals. Or in Dr Lecter's case, humans. The Fore tribesman of New Guinea have a high incidence of variant CJD (kuru) due to the past prevalence of religious cannibalism. Fortunately his current symptoms do not resemble this rapidly progressive dementing illness. (
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References:
| 1. Braunwald, E., Fauci, A.S., Kasper, D.L., Hauser, S.L., Longo, D.L., and Jameson, J.L. (Eds.) (2001). Harrison's Principles of Internal Medicine, 15th Edition. McGraw Hill. New York. | |
| 2. Penniston, K.L., and Tanumihardjo, S.A. (2006). "The acute and chronic toxic effects of vitamin A." Am J Clin Nutr, 83(2) 191-201. (PMID:16469975) |  |
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pharmacology
Liver, fava beans, and a fine chianti
Question ID: 081607159
Question written by dryman. (C) FrontalCortex.com 2006-2009, all rights reserved.
Created: 08/16/2007
Modified: 08/16/2007
Estimated Permutations: 120
