Sir Terry Pratchett: Author dies at 66 as Just Giving donations reach THOUSANDS in minutes

FANTASY author Sir Terry Pratchett has died at the age of 66, after suffering with early-onset Alzheimer's disease.

The hugely-popular author sold more than 85million books worldwide in 37 languages GETTY

The hugely-popular author sold more than 85million books worldwide in 37 languages

The hugely-popular author, who sold more than 70million books in over 30 languages, passed away in his home earlier today.

An update on his Twitter account, published minutes after news of his death was announced, simply stated: "The End".

Pratchett is best known for his long-running Discworld series of comic-fantasy novels. 

At the turn of the century, he was Britain's second most-read author, beaten only by Harry Potter creator JK Rowling.

The fantasy novelist died surrounded by his family with his cat asleep beside him on his bed, his publisher has revealed in a statement.

Pratchett was diagnosed with posterior cortical atrophy – a type of dementia - in late 2007.

In an online statement, the author's publisher said Pratchett had succumbed "to the final stages of the disease".

Just Giving page has been set-up in his memory, seeking donations for the Research Institute to the Care of Older People (RICE). Thousands of pounds have already been raised in the minutes following his death.

Pratchett was among the most prolific and successful authors of his generation. He finished his last book, a new entry to his successful Discworld series, last summer.

Fans across the world flooded Twitter to leave tributes to the author.

Prime Minister David Cameron said: "Sad to hear of Sir Terry Pratchett's death. His books fired the imagination of millions and he fearlessly campaigned for dementia awareness."

Alzheimer's Society chief executive Jeremy Hughes said: "Sir Terry Pratchett fundamentally changed the way dementia is seen and understood.

"Shouting from the rooftops about the absurdity of how little funding dementia research receives, and fighting for good quality dementia care, he was and will remain the truest of champions for people with the condition.

"During the many times Terry supported Alzheimer's Society, publicly and privately, I was struck by his passion, resilience and courage to fight and kill the demon of dementia. When thanked for his work, he'd simply smile and shake his head modestly, insisting it was nothing. Never dwelling on his own dementia, he used his voice to shout out for others when they could not."

My sympathies go out to Terry's wife Lyn, their daughter Rhianna, to his close friend Rob Wilkins, and to all closest to him

Larry Finlay

Larry Finlay, of Transworld Publishers, posted a heartfelt statement on the best-seller's Twitter account.

"I was deeply saddened to learn that Sir Terry Pratchett has died," he wrote. "The world has lost one of its brightest, sharpest minds.

"In over 70 books, Terry enriched the planet like few before him. As all who read him know, Discworld was his vehicle to satirize this world: he did so brilliantly, with great skill, enormous humour and constant invention. (sic)

"Terry faced his Alzheimer's disease (an 'embuggerance', as he called it) publicly and bravely. Over the last few years, it was his writing that sustained him. 

"His legacy will endure for decades to come.

"My sympathies go out to Terry's wife Lyn, their daughter Rhianna, to his close friend Rob Wilkins, and to all closest to him."

Prolific and hugely popular author Sir Terry Pratchett with his Knighthood GETTY

Prolific and hugely popular author Sir Terry Pratchett with his Knighthood

Sir Terry Pratchett suffered from a rare form of Alzheimer's disease called posterior cortical atrophy (PCA), also known as Benson's syndrome.

The progressive degenerative condition involves the loss and dysfunction of brain cells, and is thought to be behind 5% of cases of Alzheimer's representing thousands of people in the UK.

But although both diseases involve the loss and dysfunction of brain cells, they affect different parts of the brain.

Typically Alzheimer's disease first impacts the sides of the brain - areas which play an important role in memory, which is why memory problems are usually the first symptom of Alzheimer's.

In PCA, the disease first affects the back (posterior) of the brain, known as the occipital lobe.

Professor Sebastian Crutch, an expert on Alzheimer's at University College London (UCL), said this part of the brain was responsible for vision, so people with PCA often initially experienced visual problems.

"Rather than being based on memory where people forget appointments and where they've put things, for people with PCA the first thing they notice is complex visual behaviour," he said.

"They might find they're clipping wing mirrors when they're driving or having problems parallel parking.

"When they're reading, words will jump around and a lot of people will have difficulty perceiving objects such as glass doors."

These are best described as a "brainsight" problem than an "eyesight" issue, he added.

The first symptoms of PCA tend to occur when people are in their late 40s, 50s or early 60s, so it has an earlier onset than Alzheimer's disease.

But the first signs are often subtle and so it may be some time before a formal diagnosis is made.

Prof Crutch said the number of people with PCA was hard to say because it was so difficult to establish.

As damage in the brain spreads and the disease progresses, people develop the typical symptoms of Alzheimer's disease such as memory loss and confusion.

There is no specific treatment for PCA but some people use medications used for Alzheimer's disease.

Sir Terry announced he had the condition in December 2007 when he was 59.

He had been told by doctors that previous summer that he had suffered a mini-stroke at some point in the previous few years, although he had been unaware of it.

After problems with his dexterity and hand-eye co-ordination, he had a series of scans and was diagnosed with PCA.

Terence "Terry" David John Pratchett was born on April 28, 1948 in Beaconsfield, Bucks. 

He published his first short story at the age of 13, and later began work as a journalist for the Bucks Free Press newspaper.

Pratchett was working as the Press Officer for the Central Electricity Generation Board in 1983 when his first Discworld novel was published.

In 1998, he was awarded an OBE for "services to literature".

His modest response was: "I suspect the 'services to literature' consisted of refraining from trying to write any." However, he did admit he felt "mightily chuffed about it".

The author, then suffering with Alzheimer's, was knighted in 2009.

Receiving the honour at Buckingham Palace, he said multi-million pound bonuses paid to bankers should instead be spent helping to treat dementia patients.

During his lifetime, Pratchett made a significant public donation to the Alzheimer's Research Trust and filmed a hard-hitting documentary highlighting his battle with the cruel affliction.

Dressed in his customary top hat and morning suit, Pratchett said at the time: "It would appear to me that me getting up and saying 'I've got Alzheimer's', it did shake people, you cannot help but notice it's in the news an awful lot. The thing about Alzheimer's is there are few families that haven't been touched by the disease.

"People come up to me and talk about it and burst into tears, there's far more awareness about it and that was really what I hoped was going to happen.

"Everybody thinks the Government should be doing more about everything but just think how many of the bonuses which are quite rightly being dragged off certain people, just think to what good causes they could be put - wouldn't that be a lovely thought?"

Pratchett was awarded honorary doctorates of literature by the Universities of Warwick, Portsmouth, Bath and Bristol.

He leaves behind his wife Lyn and their daughter Rhianna.

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