The History of HD

Huntington’s Disease was named after George Huntington, not because his was the first to describe the disease, but because his report was a brief, insightful and very specific description of the symptoms and inheritance. (The full portion of Huntington’s paper that describes the disease is linked here). Huntington was only 22 when his paper was published - even more surprising, he had first observed and noted patients with chorea and cognitive deficits characteristic of HD when he was only 9, accompanying his physician father on visits to patients. Later in life, Huntington offered the following poignant description of his experiences with HD:

‘Driving with my father through a wooded road leading from East Hampton to Amagansett, we suddenly came upon two women, mother and daughter, both tall, thin, almost cadaverous, both bowing, twisting, grimacing. I stared in wonderment, almost in fear. What could it mean? My father paused to speak with them, and we passed on. Then my Gamaliel like instruction began; my medical education had its inception. From this point my interest in the disease has never wholly ceased. Then came the hanging of D.H. in his blacksmith shop. He was a victim of incipient chorea, knew it, possibly had been waiting for it, the “Sanctus Invictus,” and well knowing the character of the foe he must meet, the so dreaded, the long expected, the conquering, he cut short the taper and his life went out. Other victims had sought the same refuge again and again by drowning. Others, of a different nervous organization perhaps, lived on if not content, still seemingly reconciled to Fate, until mind and body both exhausted they fell asleep.’  

The next chapter in the history of HD, unfortunately, is a dark one.  The re-discovery of Mendel’s laws of inheritance initiated the eugenics movement starting during the late 19th century and lasting into the mid-20th century. Eugenics had the stated objective of improving the overall genetic makeup of society. Leaders of the movement, which was supported by many prominent academics, scientists, and public figures, encouraged reproduction among those deemed “most fit” and discouraged reproduction, often by forced sterilization, for those with traits perceived as undesirable, such as mental illnesses, criminal tendencies, or disabilities – including people with Huntington’s Disease! At its peak, the Eugenics movement had succeeded in convincing more than 30 states to adopt laws requiring forced sterilization for some “unfit” portions of society. Following exposure of the Nazi atrocities, there was a powerful backlash against eugenics, with repudiation of its principles. Nevertheless, and shockingly, some forced sterilization programs in the US were active into the late 1900’s and early 2000’s.