PSP is easy to say, clearly stands for something else, but does not yet fall into the realm of commonly understood abbreviations. Indeed it may not sound familiar to many medics, yet it represents a syndrome (a well defined collection of symptoms and signs) first described in 1964. PSP stands for Progressive Supranuclear Palsy. The first word is fairly clear – people with this condition have median survival of 9 years from onset, that is to say, half will die less than 9 year for onset, and half will live longer. The second word is complicated – the “nuclei” are a part of the brain stem, and in the post-mortem studies of the brains of these patients, abnormailities are found in this part of the brain stem, above the bits tnat control eye movements. “Palsy” is an old word for paralysis, or weakness, and this is a key feature of the condition.
PSP seems to be casued by the accumulation of an abnormal substance, “tau” protein, leading to loss of brain cells. The reason for this “tauopthy” is unknown, but the genetics are being explored. There is no suggestion that the condition is inherited or passed down the generations.
My illness, initially labelled, is is commonly the case, as Parkinson’s Disease, first showed itslelf when I kept falling off my bicycle, more than two years ago. The second neurologist who saw me, Professor Bandmann in Sheffield, said at out first meeting that he wondered whather PSP was the diagnosis. On our third meeting, last week, he expressed a high degree of confidence that this is what I’ve got.
The main features of my illness that fit ths diagnosis of PSP are akinesia (slowness in inititatng movement), loss of control of balance, seen mainly in frequent backwards falls, and possibly a reduction in downward gaze.
I have been greatly helped by using a walking frame, initially loaned by the excellent physio Department, and more recently a 4-wheeler bought from the very excellent Wicker store in Sheffield. This has brakes and a seat and goes outdoors, and fits easily into Ton’s Smart car. More controversially, before the PSP label was so firmly attached I acquired an electric assisted tricycle, which I ride with complete abandon, travelling independently across Sheffield. The “complete abandon” is quite possibly another feature of PSP – a psychological dysfunction due to the brain disease where control is lost of risk.
I’ll add more later.