Acta Neuropathologica

, Volume 97, Issue 2, pp 170–176

Cytoskeletal pathology in familial cerebral amyloid angiopathy (British type) with non-neuritic amyloid plaque formation

  • T. Revesz
  • J. L. Holton
  • B. Doshi
  • B. H. Anderton
  • F. Scaravilli
  • G. T. Plant
Regular paper

DOI: 10.1007/s004010050970

Cite this article as:
Revesz, T., Holton, J., Doshi, B. et al. Acta Neuropathol (1999) 97: 170. doi:10.1007/s004010050970

Abstract

The histological features of familial cerebral amyloid angiopathy (British type) with non-neuritic amyloid plaque formation (FAB) include deposition of amyloid, (supposedly associated with the C-terminal fragments of both α- and β-tubulin), in small cerebral and spinal arteries, hippocampal amyloid plaques and neurofibrillary tangles (NFTs) as well as ischaemic white matter changes. In the present study we report on the cytoskeletal pathology that occurs in association with FAB. Sections from the hippocampus and cerebellum of three cases from three unrelated families were stained with silver impregnation methods and antibodies to antigens including tau, neurofilaments, ubiquitin and glial fibrillary acidic protein. Electron microscopic examination of the hippocampus was carried out in one case. All hippocampal subregions contained large numbers of NFTs and neuropil threads (NT), which were stained with both phosphorylation-dependent and phosphorylation-independent tau antibodies and ultrastructurally were found to be composed of paired helical filaments (PHFs). Although the majority of the amyloid plaques were of the non-neuritic type, distended PHF-containing and tau-positive neurites were seen in close proximity of a minority of the hippocampal plaques. The perivascular amyloid deposits of the cerebellum contained numerous ubiquitin-positive granular elements similar to those seen in cerebellar Aβ amyloid plaques in Alzheimer’s disease. In FAB severe cytoskeletal pathology is present in areas most affected by amyloid plaque deposits, thus suggesting a localised neurotoxic effect of the poorly characterised amyloidogenic peptide characteristic of this condition.

Key words Amyloid angiopathy Amyloid plaque British type Neurofibrillary tangle Paired helical filament 

Copyright information

© Springer-Verlag Berlin Heidelberg 1999

Authors and Affiliations

  • T. Revesz
    • 1
  • J. L. Holton
    • 1
  • B. Doshi
    • 2
  • B. H. Anderton
    • 3
  • F. Scaravilli
    • 1
  • G. T. Plant
    • 4
  1. 1.Department of Neuropathology, Institute of Neurology, Queen Square, London WC1N 3 BG, UK e-mail: T.Revesz@ion.ucl.ac.uk, Fax: +44-171-916 9546GB
  2. 2.Department of Neuropathology, Institute of Psychiatry, London, UKGB
  3. 3.Department of Neuroscience, Institute of Psychiatry, London, UKGB
  4. 4.National Hospital for Neurology and Neurosurgery, London, UKGB