Abstract
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.
Similar content being viewed by others
Author information
Authors and Affiliations
Additional information
Received: 18 September 1997 / Revised, accepted: 15 January 1998
Rights and permissions
About this article
Cite this article
Leverenz, 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:
DOI: https://doi.org/10.1007/s004010050914