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Clustering of neuronal inclusions in "dementia with neurofilament inclusions"

Abstract

Dementia with neurofilament inclusions (DNI) is a new disorder characterized clinically by early-onset dementia and histologically by the presence of intraneural inclusions immunopositive for neurofilament antigens but lacking tau and α-synuclein reactivity. We studied the clustering patterns of the neurofilament inclusions (NI) in regions of the temporal lobe in three cases of DNI to determine whether they have the same spatial patterns as inclusions in the tauopathies and α-synucleinopathies. The NI exhibited a clustered distribution (mean size of clusters 400 μm, range 50–800 μm, SD 687.8) in 24/28 of the areas studied. In 22 of these areas, the clusters exhibited a regular distribution along the tissue parallel to the pia mater or alveus. In 3 cortical areas, there was evidence of a more complex pattern in which the NI clusters were aggregated into larger superclusters. In 6 cortical areas, the size of the clusters approximated to those of the cells of origin of the cortico-cortical pathways but in the remaining areas cluster size was smaller than 400 μm. Despite the unique molecular profile of the NI, their spatial patterns are similar to those shown by filamentous neuronal inclusions in the tauopathies and α-synucleinopathies.

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Acknowledgements

We would like to thank Prof. Peter Lantos, Institute of Psychiatry, King's College, London for reviewing the neuropathology of these cases. We would also like to thank Heidi Barnes and Mavis Kibble for their excellent technical assistance.

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Correspondence to R. A. Armstrong.

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Cairns, N.J., Armstrong, R.A. Clustering of neuronal inclusions in "dementia with neurofilament inclusions". Acta Neuropathol 106, 125–128 (2003). https://doi.org/10.1007/s00401-003-0710-5

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Keywords

  • Dementia with neurofilament inclusions
  • Neurofilament inclusions
  • Clustering
  • Tauopathies
  • α-Synucleinopathies