Clinical Approach to Progressive Supranuclear Palsy
نویسندگان
چکیده
منابع مشابه
Clinical Approach to Progressive Supranuclear Palsy
Sixty years ago, Steele, Richardson and Olszewski designated progressive supranuclear palsy (PSP) as a new clinicopathological entity in their seminal paper. Since then, in addition to the classic Richardson's syndrome (RS), different clinical phenotypic presentations have been linked with this four-repeat tauopathy. The clinical heterogeneity is associated with variability of regional distribu...
متن کاملClinical genetics of familial progressive supranuclear palsy.
Recent studies have shown that progressive supranuclear palsy (PSP) could be inherited, but the pattern of inheritance and the spectrum of the clinical findings in relatives are unknown. We here report 12 pedigrees, confirmed by pathology in four probands, with familial PSP. Pathological diagnosis was confirmed according to recently reported internationally agreed criteria. The spectrum of the ...
متن کاملFamilial 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.
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ژورنال
عنوان ژورنال: Journal of Movement Disorders
سال: 2016
ISSN: 2005-940X,2093-4939
DOI: 10.14802/jmd.15060