Thursday, March 3, 2016

Augustus d'Este 1794-1848

Sir Augustus Frederick  d'Este, Grandson of King George III is accepted to be the first recorded case of MS multiple sclerosis. His personal diary gives a vivid account of his own complaint, disseminated sclerosis, the clinical picture including transitory blindness and diplopia, absolute loss of power of locomotion followed by almost complete recovery, what followed later would be the gradual onset of paralegia, tremors, sphincter troubles and sensory symptoms. 
This description is the even more remarkable because the period covered in his diary, 1822 to 1846, is at least twenty years before any description comparable to this appeared in the medical press.

Among the observations Sir Augustus noted in his diary were:

  • December 1822, I was obliged to have my letters read to me, and their answers written for me, as my eves were so attacked that when fixed upon minute objects indistinctness of vision was the consequence.
  • Soon after I went to Ireland, and without anything having been done to my eyes, they completely recovered their strength and distinctness of vision.
  • I sometimes saw imagined spots floating before my eyes. 
  • January 1826 the most painful Chapter up to that period of my life occurred. I was beset by afflictions on all sides. My eyes were again attacked in the same manner as they had been in Scotland...my eyes again recovered. 
  • In June 1827 I found my Mother in bad health at Lausanne; the heat of the country I found intolerable.
  • My Mother and I traveled to Venice. To my surprise I there one day found a torpor (Inability to see clearly in dim light) or indistinctness of feeling about the Temple of my left eye.
  • About the 6th of November the malady increased to the extent of my seeing all objects double. Each eye had its separate vision. -The Maladv of my eyes abated, I again saw all objects naturally in their single state.
  • Now a new disease began to shew itself: every day I found gradually (by slow degrees) my strength leaving me: I could clearly perceive each succeeding day that I went up and down the staircase with greater difficulty. Numbness and want of sensation became apparent about the end of the Backbone and the Perineum.
  • About the 4th of December mv strength of legs had quite left me, and twice in one day I fell down upon the floor...I was obliged to remain on the floor until my Servant came in and picked me up. stance; I was obliged to remain on the floor until my Servant came in and picked me up. I remained in this extreme state of weakness for about 21 days, during which period I fell down about 5 times (never fainting) from my legs not being strong enough to carry my body. I never once fainted or had any sort of fit. Debility, extreme debility was the only cause of my falling...December the 14th, 15th, and 16th 1827 I was at my greatest degree of weakness;
  • December the 25th I was able to take a short walk, and on the 21st of January (1828) I was strong enough to begin a journey from Florence (where I was during my Illness) to Rome.-On the journey I was able to walk up some steep Hills. I rode out on horseback most days, and my strength gradually returned. I never was able to run so fast as formerly, nor could I venture to dance.
  • June 19th, 1843. What I complain of now is numbness all down the back part of my Thighs and Legs, and when standing or walking I cannot keep my balance without a Stick.
  • I sleep well when I am not annoyed with little nervous twitching in my Legs or Feet, which sometimes, but seldom, is the case.
Sir Augustus d'Este died in early 1846 at the age of 52.

I find it remarkable that this malady had not been observed prior to this yet 20 years later multiple sclerosis was recognized and it has assumed the following traits.
  • It is more common in Northern and Southern latitudes.
  • It is more common in persons of European descent.
  • It affects more women then men.
Were there environmental triggers that Augustus d'Este and subsequent generations face that were and are different from previous generations?







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