Once upon a time, I could spell. Once upon a time I could type – correctly. Those days are gone!
Is it age?
Is it the arthritis?
Does it matter?
As I age, I seem to forget everything (but is everything worth remembering??).
Before the best selling author Terry Pratchett announced he had been diagnosed with a rare form of Alheimer’s, few people would ever have heard of Posterior Cortical Atrophy (PCA). First described by Frank Benson in 1988, and sometimes referred to as Benson’s syndrome, PCA can be thought of as a visual variant of Alzheimer’s due to the degeneration of the visual center of brain tissue at the back of the brain.
When Pratchett was told of his condition, he described the diagnosis as, “an embuggerance.” With something approaching optimistic irony, Pratchett observed that if you’re going to get Alzheimer’s, it’s at least the best form to have. With PCA, the person is left both fluent and coherent, yet memory and visual acuity is gradually lost.
The symptoms of PCA occur after the age of 50. Symptoms typically involved blurred vision, problems in following lines of text when reading and problems with depth perception. As the disease progresses, previously familiar places and faces are forgotten. The disease is not easy to diagnose and no specific test exists to identify its presence. If the disease is suspected, a battery of psychological tests, neurological tests and brain imaging techniques are required before a diagnosis can be reached.
Typical daily tasks can present real problems. Dressing, for example, imitating movements, using keys, pens, or a razor are often ruled out. As the disease progresses, levels of visual agnosia progress to a point where the person is effectively blind. Eventually, language, reasoning and memory all become affected. There is no treatment for PCA. The course of the disease is between 8-12 years, from the onset of symptoms to death.
Diagnosed in 2007, Pratchett gave an interview to the Times Online in May 2008. His condition was now affecting him in many more obvious ways. “I can spell ‘transubstantiation’ and in the next bit I can’t spell ‘colour’ because it’s as if bits of the network are switching on and off,” he said. “The future is going to happen whether I’m scared of it or not so I do my best not to be. Around about five o’clock in the morning things might be different but you just have to face it.”