The illness of Augustus d'Este

Written by  Enrico Granieri
TWe owe our first record of multiple sclerosis to a descendent of the Este.
''An 'Estense' neurologist is not complete without this book especially when his main love is multiple sclerosis''. With this dedication, my friend George Ebers, professor of Neurology at Oxford University and renowned researcher into multiple sclerosis, gave a me a book some years ago containing the notes in which a descendent of the House of Este describes the symptoms of his disease. It is a singular fact that the earliest account of the symptoms and clinical history of multiple sclerosis was written by Sir Augustus d'Este, a grandson of King George III of England, who kept a personal diary and record of his disease between 1822 and 1848. Today Augustus d'Este's account is universally recognised as the first document describing the typical course of the disease in its entirety.
Augustus d'Este was born on 13 January 1794. His mother was the Lady Augusta Murray,a Roman Catholic and daughter of John, 4th Earl of Dunmore; his father was Prince Augustus Frederick, Duke of Sussex, the sixth son of the Hanoverian King George III and a descendent of the Este family.


Augustus had a normal childhood for the period; he was vaccinated against smallpox, and suffered from green faeces, colic and shingles. His mother, with whom he lived, yielded to his every whim and brought him up in accordance with the customs of the English aristocracy, which justly enjoyed a less than excellent reputation at the time.
While at Harrow school, he had measles in February 1808; in 1812 he joined the 7th Royal Fusiliers and reached the rank of lieutenant colonel.
The part of his diary which comes down to us begins with the description of bilateral optic neuritis which developed in 1822, when he was aged 28. Here is how Augustus described the first days of his illness: ''In the month of December 1822 I travelled from Ramsgate to the Highlands of Scotland for the purpose of passing some days with a Relation for whom I had the affection of a Son. On my arrival I found him dead. Shortly after the funeral I was obliged to have my letters read to me, and their answers written for me, as my eyes were so attacked that when fixed upon minute objects indistinctness of vision was the consequence: Until I attempted to read, or to cut my pen, I was not aware of my Eyes being in the least attacked. Soon after, I went to Ireland, and without anything having been done to my Eyes, they completely recovered their strength and distinctness of vision.''
Optic neuritis recurred in 1826, when he was aged 32.
A year later, during a voyage in Italy, he suffered from episodes of double vision due paralysis of the ocular muscles. ''17 October 1827: To my surprise (in Venice) I one day found a torpor or indistinctness of feeling about the Temple of my left Eye. At Florence I began to suffer from a confusion of sight:about the 6th of November the malady increased to the extent of my seeing all objects double. Each eye had its separate visions. (Dr. Kissock supposed bile to be the cause: I was twice blooded from the temple by leeches); purges were administered; One Vomit, and twice I lost blood from the arm: one of the times it was with difficulty that the blood was obtained. The Malady in my Eyes abated, I again saw all objects naturally in their single state. I was able to go out and walk. Now a new disease began to show itself: every day I found gradually (by slow degrees) my strength leaving me. A torpor or numbness and want of sensation became apparent about the end of the Back-bone and the Perinaeum. At length about the 4th of December my strength of legs had quite left me. I remained in this extreme state of weakness for about 21 days.The problem with my eyes receded, and I recovered the vision of each object in its single state in the normal manner. I was once more able to go out and take a walk,''
In the same year there appeared the first symptoms of problems in the nerve paths affecting movement and feeling: ''Now a new disease began to show itself: every day I found gradually (by slow degrees) my strength leaving me. A torpor or numbness and want of sensation became apparent about the end of the Back-bone and the Perinaeum. At length about the 4th of December my strength of legs had quite left me. I remained in this extreme state of weakness for about 21 days.''
Augustus d'Este was never again able to run fast or dance properly. In 1828 he related further unpleasant sensory symptoms and fatigue, but he continued his military career until developing urinary retention. He also became constipated, and had a single episode of faecal incontinence. In 1830, during his holidays in Ramsgate, in Kent, he discovered that he had become impotent.
From this point onwards, his diary records various visit to European spas, medical consultations and the therapeutic prescriptions and treatments suggested.
Given that parts of the diary are missing, it is presumed that Augustus d'Este subsequently also suffered from disturbed balance, ataxia, and episodes of numbness below the waist; he later also recorded night-time spasms. In 1843, as well as these more or less persistent symptoms, he suffered an acute episode of vertiginous syndrome and faulty motor coordination, from which he made a slow partial recovery. In a short time, the disease took on a slow progressive course with relapses and remissions, characterised by new acute symptoms which eventually led to the loss of the use of his arms. Finally, in December 1848, Augustus d'Este died at the age of 54 years, unmarried and without heirs, having suffered for 26 years from an initially intermittent disease with clinical relapses and remission of the symptoms, but with a gradual neurological deterioration.
The disease had not, of course, been identified at that time. In the years after 1830, studies of morbid anatomy in diseases similar to that described by Augustus d'Este began to a signal the presence of inflammatory lesions throughout the central nervous system, typical of the diseases which in 1864 Jean-Martin Charcot would call 'sclerose en plaque'.
There is no doubt that Charcot's account was an accurate description of the course of multiple sclerosis. Multiple sclerosis is disease of unknown origin which causes attacks of inflammation in the brain and spinal cord involving the 'white matter' made up of the myelin-covered nerve fibres that are responsible for transmitting motor, visual and sensory signals. In most cases multiple sclerosis hits young people and young adults in the prime of their professional, personal and social lives.
It is significant that the diary kept by Augustus d'Este-little less than two hundred years ago-records clearly and in detail the clinical data that are recognised today as the signs and typical case history identified by neurologists in the onset and course of multiple sclerosis in young people.