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Multiple sclerosis (MS) is a neurodegenerative malady characterized by recurrent neural attacks that are followed by a partial waning of symptoms. The disease is chronic and, over time, the patient accumulates neuronal damage manifest by a gradual loss of physical capacities. There is no cure for MS and the common causes of death are infections such as pneumonia or urinary tract infections. In the mid-19th century Charcot, in France, intensively studied and described the disease now called “multiple sclerosis”. By the mid-20th century researchers had discovered, in the journal of an obscure member of the British Royal family, a trove of information consistent with MS. Though the man involved lived through the sequelae of this disease in the early 1800s and died nearly 20 years before Charcot’s work, he kept such meticulous notes of his signs and symptoms in his journal that, when reviewed a century later, physicians retrospectively diagnosed him with multiple sclerosis. He became the first known patient with MS. His name was Augustus Frederick D’Este.

A brief background of Augustus D’Este

D’Este was of royal, but tarnished, blood. His father was Prince Augustus Frederick, Duke of Sussex, and his mother, a Catholic, was lady Augusta Murray of Scotland. His parent’s marriage was in stark violation of the 1772 Royal Marriages Act because it did not have the king’s blessing. The king disapproved thereby annulling the marriage and leaving Augustus Frederick an “illegitimate” child. He was given the last name of D’Este, an older European name, but was disinherited by the crown, his grandfather, King George III of Britain.

His disease and treatments

Throughout his lifetime, D’Este struggled with obtaining royal recognition while plagued by his illness. As a young man of 28 years, his MS first manifested as an inability to focus his vision. He then began experiencing “attacks” of “spots” floating about his visual fields. He stated that he had improvement in his symptoms after he began drinking, bathing with and immersing his eyes in a tonic called “steel-water”. But a year or so later he developed diplopia and an odd feeling around the lateral portion of his left eye. His physician treated him with a course of leeches and he noticed some improvement.

However, after a short time he began having problems with periodic falls. He wrote that this was not actual syncope but falling due to profound weakness and fatigue. Soon he couldn’t carry his own weight and required assistance for mobility. He also developed numbness around the lower spine and perineum and bouts of uncontrollable defecation. He changed physicians and his new primary care provider prescribed a course of beef steak consumption concurrent with imbibing either London Porter, sherry or Madeira wine. His legs and back were also rubbed with a liniment. This protocol appeared most tenable, his compliance was very good, and he experienced a short-lived recovery. The response rate seemed, overall, subjectively better than the program of leeches.

Sometime later the lower back and perineal numbness returned but now compounded by urogenital difficulties. He developed urinary retention. A bougie was inserted into the bladder reportedly rupturing a membranous stricture; this procedure was accompanied by a great deal of pain and cold sweats. He was prescribed a tonic of herbs and flowers. Despite all these treatments he was still bothered by a modicum of fatigue which, combined with the back/perineal numbness, made it impossible for him to walk, ride a horse, or even sit in a carriage for long distances. The latter would also provoke a numbness in the legs. To his disfavor, he found himself impotent though his urinary tract was evaluated and found to be faultless. He was given “pills for vigor” with little effect. He was subsequently given a course of medical electricity. Nothing helped.

Death and legacy

In the time leading up to his death D’Este found that his lower extremity numbness continued but now was accompanied by lower abdominal numbness. He also noted a progressive dysfunction in his hands and eventually lost the use of his arms. He suffered from periodic leg spasms which kept him awake at night. During his final years he was totally bedbound. When his father died in 1843 D’Este moved to claim the dukedom of Sussex as his own but the House of Lords dismissed his plea. He died of unknown causes in 1848 at 54 years of age.

In spite of his multiple sclerosis, Augustus D’Este maintained an active, very productive life. He enlisted as an Aide-de-Camp with the British army during the ill-fated Battle of New Orleans in the War of 1812. He served in the 9th Light Dragoons, the 4th Royal Irish Dragoons and, as a colonel, he conducted numerous military inspections. He was a Freemason, a “Knight Commander” of the Hanoverian Guelphic order (a British order of Chivalry) and a Ranger of St. James and Hyde parks. He was an active member of the Aborigines Protection Society with a particular interest in protecting the rights of Native Americans in Canada. D’Este chose to live even with his encumbrances. He served well his country, the crown and the people. His only true defeat, the loss of his birthright, was due to an act of Parliament…NOT to his MS.

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Posted by Robert Killeen

Dr. Killeen is a physician practicing Internal Medicine and Hematology primarily in the Tampa Bay area of Florida.