منابع مشابه
Familial progressive supranuclear palsy.
A progressive extrapyramidal syndrome and dementia occurred in three members of one family. The age of onset was in the seventh decade and the affected individuals showed many of the clinical features of progressive supranuclear palsy (PSP). Necropsy of one individual revealed the neuropathological features of PSP. We propose that this family has a familial form of PSP and review the evidence i...
متن کاملProgressive Supranuclear Palsy (PSP)
Introduction: Progressive supranuclear palsy (PSP) is a rare neurodegenerative disorder producing characteristic eye movement abnormalities in the classic presentation. The disease pathologically is marked by accumulation of abnormal tau proteins. There are useful MRI features, however the diagnosis remains clinical in the majority of cases, and no objective diagnostic test exists. Treatment at...
متن کاملProgressive supranuclear palsy.
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.
متن کاملProgressive supranuclear palsy
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...
متن کاملRational therapeutic approaches to progressive supranuclear palsy.
Progressive supranuclear palsy is a sporadic and progressive neurodegenerative disease, most often presenting as a symmetric, akinetic-rigid syndrome with postural instability, vertical supranuclear gaze palsy and frontal lobe deficits. It belongs to the family of tauopathies and involves both cortical and subcortical structures. Although the exact pathophysiology is not yet fully understood, s...
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ژورنال
عنوان ژورنال: Journal of Neurology, Neurosurgery & Psychiatry
سال: 1995
ISSN: 0022-3050
DOI: 10.1136/jnnp.58.3.397