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Progressive Supranuclear Palsy
Background
Progressive Supranuclear Palsy: PSP; Steele Richardson Olszewski syndrome
PSP, otherwise known as Steele Richardson Olszewski syndrome after the three Canadian Doctors, who first described it in 1962 as a distinct disorder, is a neurodegenerative disease, which involves the progressive death of neurons, mainly in the basal ganglia and brainstem. In pathological examination of brain tissue, characteristic neurofibrillary tangles of a protein called tau distinguishes this disorder from other neurodegenerative disorders, particularly from Parkinson's disease where lewy body deposits are a characteristic hallmark. However, in the early stages PSP can mimic Parkinson's disease and be misdiagnosed as such. The two disorders have historically been linked.
Average life expectancy is some seven years from onset, the last two often wheelchair-bound, with twenty-four hour care becoming necessary. The onset of PSP is mainly in the fifty to seventy age bracket, but patients as young as late thirties have been diagnosed. Recent studies indicate a prevalence of 5 in 100,000, which is nearly four times as many as earlier studies had indicated. Leading neurologists now assess there could be as many as five to ten thousand cases in the UK alone, many misdiagnosed or undiagnosed.
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