Diagnosing Clapham Junction syndrome

Abstract

People who have dementia in this country are not heard. I’m fortunate; I can be heard. Regrettably, it’s amazing how people listen if you stand up in public and give away $1million for research into the disease, as I have done. Why did I do it? I regarded finding I had a form of Alzheimer’s as an insult and decided to do my best to marshal any kind of forces I could against this wretched disease. I have posterior cortical atrophy or PCA. They say, rather ingenuously, that if you have Alzheimer’s it’s the best form of Alzheimer’s to have. This is a moot point, but what it does do, while gradually robbing you of memory, visual acuity and other things you didn’t know you had until you miss them, is leave you more or less as fluent and coherent as you always have been. I spoke to a fellow sufferer recently (or as I prefer to say, ‘‘a person who is thoroughly annoyed with the fact they have dementia’’) who talked in the tones of a university lecturer and in every respect was quite capable of taking part in an animated conversation. Nevertheless, he could not see the teacup in front of him. His eyes knew that the cup was there; his brain was not passing along the information. This disease slips you away a little bit at a time and lets you watch it happen. When I look back now, I suspect there may be some truth in the speculation that dementia (of which Alzheimer’s is the most common form) may be present in the body for quite some time before it can be diagnosed. For me, things came to a head in the late summer of 2007. My typing had been getting progressively worse and my spelling had become erratic. I grew to recognize what I came to call Clapham Junction days when the demands of the office grew too much to deal with. I was initially diagnosed not with Alzheimer’s but with an ischemic change, a simple loss of brain cells due to normal ageing. That satisfied me until the next Clapham Junction day. I went back to my GP and said I knew there was something more going on. Fortunately, she knew well enough not to bother with the frankly pathetic MMSE test (the 30-point questionnaire used to determine brain function) and sent me to Addenbrooke’s Hospital in Cambridge, where, after examination of my MRI scan and an afternoon of complex tests, I was diagnosed with PCA, an uncommon variant of dementia, which had escaped the eagle eye of the original diagnostician. When in Paradise Lost Milton’s Satan stood in the pit of hell and raged at heaven, he was merely a trifle miffed compared to how I felt that day. I felt totally alone, with the world receding from me in every direction and you could have used my anger to weld steel. Only my family and the fact I had fans in the medical profession, who gave me useful advice, got me through that moment. I feel very sorry for, and angry on behalf of, the people who don’t have the easy ride I had.