Friday, Aug. 01, 1969

Royal Malady

When Britain's King George III died in 1820, he was blind, deaf and apparently mad. His physicians, limited in their medical knowledge and hindered by protocol in examining their royal patient (they could not inquire how he felt unless he spoke to them first), had long since concluded that the King was "under an entire alienation of mind." George III went down in history as the mad monarch, a judgment accepted by generations of historians and buttressed by psychiatric studies.

Now, however, two British psychiatrists who re-examined George's medical records in the light of new medical knowledge are proposing a radically different interpretation. Drs. Ida Macalpine and Richard Hunter, her son, suggest in the British Medical Journal that George III suffered from porphyria, a rare hereditary metabolic disorder that can lead to severe mental disturbances.

George apparently inherited porphyria from Mary Queen of Scots (1542-87), who passed the genetic disorder on to 16 generations of European aristocracy. Her son, James I of England, was affected, as are several living European aristocrats who cooperated in the study but asked that they not be identified. In a simliar fashion, Queen Victoria--whose father, the Duke of Kent, showed signs of porphyria--passed hemophilia on to generations of male European royalty.

Textbook Case. Porphyria was unknown in clinical jargon before the 20th century, and is still not fully understood. It is a group of diseases with many different signs and symptoms. "In some of them the only problem is the undue sensitivity of the skin to sunlight," wrote Professor Abe Goldberg of Glasgow's Western Infirmary in 1966. In others, "the normal life of the patient may be shattered by devastating attacks of abdominal pain, paralysis of limbs, and profound mental upset."

One sign, however, may occur in any variety of porphyria--darkening of the urine, which frequently turns the color of port wine. The discoloration is caused by the presence of porphyrins, purple-red pigments contained in every cell of the human body and responsible for the red color of blood. In porphyria a metabolic defect results in an excess of porphyrins and their byproducts.

George Ill's illness, say Macalpine and Hunter, reads "like a textbook case," His first severe attack occurred in 1788, when he was 50 years old, and lasted for seven months. Starting with acute abdominal pain, weakness of the limbs and the classic discolored urine, his symptoms progressed through insomnia, headache and restlessness to delirium, convulsions and stupor. Even after his condition improved, George suffered periods during which his doctors said "wrong ideas" took hold of him. In 1810, he became so ill that he was incapacitated for the rest of his life, and his son, as Prince Regent, assumed the King's duties, George died at 81, one month after a turbulent attack during which he went 58 hours without sleep.

Equally Unkind. The standard treatment for insanity in George Ill's day was coercion and restraint. If he refused to eat because he had difficulty swallowing, or if he was too restless to lie down, his attendants would put him in a straitjacket.

Historians have been equally unkind, characterizing him as neurotically irresolute at some times and unrealistically stubborn at others. Some attribute his firm anticolonial policy during the American Revolution to outright madness. The findings of Drs. Macalpine and Hunter require a modification of this view to take his physical illness into account. The new evidence may also explain the mysterious deaths of several of his ancestors and collateral relatives, including James I's son Henry and George's sister Caroline Matilda, Queen of Denmark and Norway. Both were rumored to have been poisoned by close relatives. Both actually may have died of the royal malady.

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