Progressive Supranuclear Palsy classically

In RS, two-thirds of cases are men, whereas the sex distribution in PSP-P is even. Disease duration in RS is significantly shorter (5.9 versus 9.1 years, P < 0.001) and age at death earlier (72.1 versus 75.5 years, P = 0.01) than in PSP-P. The isoform composition of insoluble tangle-tau isolated from the basal pons also differed significantly. In RS, the mean four-repeat: three-repeat tau ratio was 2.84 and in PSP-P it was 1.63 (P < 0.003). The effect of the H1,H1 PSP susceptibility genotype appeared stronger in RS than in PSP-P (odds ratio 13.2 versus 4.5). The difference in genotype frequencies between the clinical subgroups is not significant. There are no differences in apolipoprotein E genotypes. The different tau isoform deposition in the basal pons suggests that PSP-P may ultimately prove to be a discrete diagnostic category (Williams DR, de Silva R, et al. The Queen Square Brain Bank for Neurological Disorders, London, UK; Brain. 2005 Mar 23).

Thomas E. Radecki, M.D., J.D.

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