Three puzzling cases

The clinicopathological conference is a time honoured exercise in which a participant is given a clinical protocol and tries to make a diagnosis. It has been variously lauded as an instructive teaching exercise or derided as an unreal situation.

The cases are often puzzling. What, for example, is one to make of a wasted man, deathly pale, with web-like thin hair, exaggerated sensitivity to noise, odours, light, or touch, where the course of the disease fluctuates with intermittent agitation or depression?

Or what sudden epidemic illness would cause high fever, profuse generalised bleeding, and death within half an hour?

And what about a dishevelled 39 year old man with a history of alcohol and possibly opiate use, presenting with lethargy and confusion but remaining afebrile, becoming delirious and combative, and dying within four days.

The first two cases, products of Edgar Allan Poe's lugubrious imagination, defy diagnosis. The symptoms of the cadaverous looking man in The Fall of the House of Usher have been attributed, perhaps not too convincingly, to acute intermittent porphyria (JAMA 1989;261: 863-4). One can only speculate what might have caused the hideous pestilence with “sharp pains, sudden dizziness, and then profuse bleeding at the pores with dissolution… the incidents of half an hour” (The Mask of the Red Death).

For the third case, a Dr Michael Benitez at one time suggested rabies. It is a case unsolved since 1849, when the patient boarded a train in Richmond, disappeared for five days, was found confused in a Baltimore tavern, dishevelled, in someone else's clothes, then taken to a city hospital, where he died delirious after several days. Delirium tremens, encephalitis, and malaria have been considered, but some think he was robbed and mugged. Others postulated that this being during an election, Poe (for it was the author himself) was drugged by a “repeater” gang and taken to vote at different polling booths, then abandoned.

There are further mysteries: firstly, the body was exhumed in 1875 and relocated, but there is speculation that the gravediggers dug up the wrong body; and secondly, for several years after 1949 an anonymous visitor would come on Poe's birthday and place a half bottle of Martel cognac on his grave.