LOS ANGELES, Nov 24 — The American man who popularised the Ice Bucket Challenge to raise funds for research into ALS died Sunday as a result of the fatal neurological disease.
Little known outside those diagnosed with the condition, Patrick Quinn's viral challenge made amyotrophic lateral sclerosis (ALS) a household name in 2014.
The rare condition normally claims the lives of those who have it within two to three years of diagnosis.
But Quinn, who was diagnosed shortly after his 30th birthday, managed to hold off the disease for seven years.
The neurodegenerative condition attacks the motor nerve cells in the brain and spinal cord, hampering their ability to communicate with muscles and control voluntary movements, leading to eventual paralysis.
Early symptoms of stiffness and muscle weakness worsen over time as victims gradually lose the ability to walk, speak and breathe.
The deadly condition is very rare, occurring on average among two new cases per 100,000 people every year, most typically among individuals aged between 55 and 65.
There is currently no cure or treatment that halts or reverses ALS, though there are some options to can help manage symptoms.
The disease takes two main forms, according to the US-based ALS Association.
The vast majority of people suffer from a “sporadic” version that can affect anyone while up to 10 per cent of cases in the US are hereditary.
Military veterans are up to twice as likely to be diagnosed as the general public, for unknown reasons.
The average survival time for those affected by ALS is three years, according to the ALS Association.
Only five per cent of patients live for 20 years or more with the condition.
British physicist Stephen Hawking was one of the most famous people to have the disease, and his five-decade battle against it was an extraordinary exception.
Researchers have said Hawking's longevity remains a mystery, though some have noted that the disease's progression varies by patient and could be governed by genetics.
Other famous people who had it include playwright Sam Shepard, who died in August 2017, Sesame Street co-creator Jon Stone and jazz musician Charles Mingus.
ALS is commonly referred to as “Lou Gehrig's disease” in a nod to the baseball legend who is believed to have died from the illness in 1941. — AFP-Relaxnews
