Werner syndrome (WS) is an autosomal recessive disorder associated with evidence of accelerated systemic aging, but generally thought not to involve the central nervous system. We examined two WS cases utilizing a sensitive Bielschowsky silver stain and immunohistochemistry for amyloid β peptide (Aβ) and hyperphosphorylated tau. Extensive frontal and temporal lobe Aβ deposition was observed in the oldest (age 57 years) WS case and restricted neurofibrillary pathology was seen in the medial temporal lobe of both cases. The severity of Aβ deposition in the medial temporal lobe of the oldest case exceeded that observed in our control cases and that reported in the literature. Our findings suggest that the apparent accelerated aging observed in WS can involve the central nervous system and may implicate the recently observed WRN locus mutation associated with WS in the neuropathology of aging and aging-associated diseases.
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Similar content being viewed by others Explore related subjectsDiscover the latest articles and news from researchers in related subjects, suggested using machine learning. Author information Authors and AffiliationsDepartment of Neurology, Box 356465, University of Washington School of Medicine, Seattle, WA 98195-6465, USA Tel.: 1-206-685-7687, Fax: 1-206-685-8100, , , , , , US
J. B. Leverenz & Gerard D. Schellenberg
Department of Medicine, University of Washington School of Medicine, Seattle, WA 98195-6465, USA, , , , , , US
Chang-En Yu
Received: 18 September 1997 / Revised, accepted: 15 January 1998
About this article Cite this articleLeverenz, J., Yu, CE. & Schellenberg, G. Aging-associated neuropathology in Werner syndrome. Acta Neuropathol 96, 421–424 (1998). https://doi.org/10.1007/s004010050914
Issue Date: September 1998
DOI: https://doi.org/10.1007/s004010050914
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