This review concentrates on the molecular pathology of the protein tau, including its expression in various regions of the brain. The role of post-translational modifications in fibrillogenesis is also discussed, particularly hyperphosphorylation and glycation. However, the initiating event that causes aberrant tau processing remains unclear.

Handbook of Atypical Parkinsonism, eds. Carlo Colosimo, David E. Riley, and Gregor K. Wenning. Published by Cambridge University Press. © Cambridge University Press 2011. Historical review Although the earlier literature contained several accounts of progressive supranuclear palsy (PSP) [ 1 ], including the famous case reported by Charcot in the nineteenth century [ 2 ], the fi rst detailed des...

BACKGROUND AND PURPOSE Both diffusion tensor imaging and the apparent transverse relaxation rate have shown promise in differentiating Parkinson disease from atypical parkinsonism (particularly multiple system atrophy and progressive supranuclear palsy). The objective of the study was to assess the ability of DTI, the apparent transverse relaxation rate, and their combination for differentiatin...

Spontaneous and voluntary eyelid motility is often abnormal in patients with progressive supranuclear palsy. In contrast, their eyelid reflex responses are relatively preserved, and only those generated by an acoustic startle have been found absent or severely reduced. We hypothesized that, because of their relevant brainstem pathology, patients with progressive supranuclear palsy might have ot...

OBJECTIVE To elucidate the character and distribution of the abnormal muscle tonus in the body axis in progressive supranuclear palsy. Although neck hypertonus has been well described in progressive supranuclear palsy, little is known about the involvement of the truncal muscles. METHODS Muscle tonus of the neck and trunk was separately investigated in 13 patients with progressive supranuclea...

STUDY OBJECTIVE To compare sleep characteristics, rapid eye movement (REM) sleep without atonia, and REM sleep behavior disorder (RBD) in patients with progressive supranuclear palsy (tauopathy), patients with Parkinson's disease (a synucleinopathy), and control subjects. DESIGN Sleep interview, overnight polysomnography, and Multiple Sleep Latency Tests. PATIENTS Forty-five age- and sex-ma...