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Spatial patterns of the pathological changes in neuronal intermediate filament inclusion disease (NIFID): an α-internexin immunohistochemical study

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Summary

Neuronal intermediate filament inclusion disease (NIFID) is characterized by α-internexin positive neuronal cytoplasmic inclusions (NCI), swollen achromatic neurons (SN), neuronal loss, and gliosis. This study tested: 1) whether the spatial patterns of the lesions was topographically organized in areas of the frontal and temporal lobe and 2) whether a spatial relationship exists between the NCI and SN. The NCI were distributed in regular clusters and in a quarter of these areas, the clusters were 400–800 µm in diameter approximating to the size of the cells of origin of the cortico-cortical pathways. Variations in the density of the NCI were positively correlated with the SN. Hence, cortical degeneration in NIFID appears to be topographically organized and may affect the cortico-cortical projections, the clusters of NCI and SN developing within the same vertical columns of cells.

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References

  • RA Armstrong (1993a) ArticleTitleThe usefulness of spatial pattern analysis in understanding the pathogenesis of neurodegenerative disorders, with particular reference to plaque formation in Alzheimer’s disease Neurodegen 2 73–80

    Google Scholar 

  • RA Armstrong (1993b) ArticleTitleIs the clustering of neurofibrillary tangles in Alzheimer’s patients related to the cells of origin of specific cortico-cortical projections? Neurosci Lett 160 57–60 Occurrence Handle10.1016/0304-3940(93)90916-9 Occurrence Handle1:STN:280:ByuD28jlsFE%3D

    Article  CAS  Google Scholar 

  • RA Armstrong (1996) ArticleTitleCorrelations between the morphology of diffuse and primitive β-amyloid (Aβ) deposits and the frequency of associated cells in Down’s syndrome Neuropath Appl Neurobiol 22 527–530 Occurrence Handle1:STN:280:ByiC2Mjps1A%3D

    CAS  Google Scholar 

  • RA Armstrong (1997) ArticleTitleAnalysis of spatial patterns in histological sections of brain tissue J Neurosci Meth 73 141–147 Occurrence Handle10.1016/S0165-0270(96)02221-2 Occurrence Handle1:STN:280:ByiA3sbmvVI%3D

    Article  CAS  Google Scholar 

  • RA Armstrong (2003) ArticleTitleMeasuring the degree of spatial correlation between histological features in thin sections of brain tissue Neuropathology 23 245–253 Occurrence Handle14719538 Occurrence Handle10.1046/j.1440-1789.2003.00516.x

    Article  PubMed  Google Scholar 

  • RA Armstrong (2006) ArticleTitleMethods of studying the planar distribution of objects in histological sections of brain tissue J Microsci 221 153–158 Occurrence Handle10.1111/j.1365-2818.2006.01559.x Occurrence Handle1:STN:280:DC%2BD287mtlSitw%3D%3D

    Article  CAS  Google Scholar 

  • RA Armstrong NJ Cairns PL Lantos (2001) ArticleTitleWhat does the study of spatial patterns tell us about the pathogenesis of neurodegenerative disorders? Neuropathology 224 41–44

    Google Scholar 

  • RA Armstrong PL Lantos NJ Cairns (2004) ArticleTitleSpatial patterns of α-synuclein positive glial cytoplasmic inclusions in multiple system atrophy Mov Disord 19 109–112 Occurrence Handle14743371 Occurrence Handle10.1002/mds.10637

    Article  PubMed  Google Scholar 

  • EH Bigio AM Lipton CL White DW Dickson A Hirano (2003) ArticleTitleFrontotemporal dementia and motor neurone degeneration with neurofilament inclusion bodies: additional evidence for overlap between FTD and ALS Neuropathol Appl Neurobiol 29 239–253 Occurrence Handle12787321 Occurrence Handle10.1046/j.1365-2990.2003.00466.x Occurrence Handle1:STN:280:DC%2BD3szgt1GisA%3D%3D

    Article  PubMed  CAS  Google Scholar 

  • NJ Cairns RA Armstrong (2003) ArticleTitleClustering of neuronal inclusions in patients with ‘dementia with neurofilament inclusions’ Acta Neuropathol 106 125–128 Occurrence Handle12732935 Occurrence Handle10.1007/s00401-003-0710-5 Occurrence Handle1:STN:280:DC%2BD3szmsVGntw%3D%3D

    Article  PubMed  CAS  Google Scholar 

  • NJ Cairns RH Perry E Jaros D Burn IG McKeith JS Lowe J Holton MN Rossor K Skullerud C Duyckaerts FF Cruz-Sanchez PL Lantos (2003) ArticleTitlePatients with a novel neurofilamentopathy: dementia with neurofilament inclusions Neurosci Lett 341 177–180 Occurrence Handle12697277 Occurrence Handle10.1016/S0304-3940(03)00100-9 Occurrence Handle1:CAS:528:DC%2BD3sXivFCqtro%3D

    Article  PubMed  CAS  Google Scholar 

  • NJ Cairns E Jaros RH Perry RA Armstrong (2004a) ArticleTitleTemporal lobe pathology of human patients with neurofilament inclusion disease Neurosci Lett 354 245–247 Occurrence Handle10.1016/j.neulet.2003.10.020 Occurrence Handle1:CAS:528:DC%2BD3sXhtVWisrjK

    Article  CAS  Google Scholar 

  • NJ Cairns V Zhukareva K Uryu B Zhang E Bigio IRA Mackenzie M Gearing C Duyckaerts H Yokoo Y Nakazato E Jaros RH Perry VMY Lee JQ Trojanowski (2004b) ArticleTitleα-Internexin is present in the pathological inclusions of neuronal intermediate filament inclusion disease Am J Pathol 164 2153–2161 Occurrence Handle1:CAS:528:DC%2BD2cXltFymsb4%3D

    CAS  Google Scholar 

  • NJ Cairns M Grossman SE Arnold DJ Burn E Jaros RH Perry C Duyckaerts B Stankoff B Pillon K Skullerud FF Cruz-Sanchez EH Bigio IRA Mackenzie M Gearing J Juncos JD Glass H Yokoo Y Nakazato S Mosaheb JR Thorpe K Uryu VMY Lee JQ Trojanowski (2004c) ArticleTitleClinical and neuropathologic variation in neuronal intermediate filament inclusion disease (NIFID) Neurology 63 1376–1384 Occurrence Handle1:STN:280:DC%2BD2crjsVCjuw%3D%3D

    CAS  Google Scholar 

  • GY Ching RKH Liein (1998) ArticleTitleRoles of head and tail domains in alpha-internexin’s self-assembly and coassembly with the neurofilament triplet proteins J Cell Sci 111 321–333 Occurrence Handle9427681 Occurrence Handle1:CAS:528:DyaK1cXhsFCgur0%3D

    PubMed  CAS  Google Scholar 

  • GY Ching CL Chien R Flores RKH Liein (1999) ArticleTitleOverexpression of alpha-internexin causes abnormal neurofilamentous accumulations and motor coordination deficits in transgenic mice J Neurosci 19 2974–2986 Occurrence Handle10191315 Occurrence Handle1:CAS:528:DyaK1MXitlGnurw%3D

    PubMed  CAS  Google Scholar 

  • M Goedert MG Spillantini LC Serpell J Berriman MJ Smith R Jakes RA Crowther (2001) ArticleTitleFrom genetics to pathology: tau and alpha-synuclein assemblies in neurodegenerative diseases Philos Trans R Soc Lond B Biol Sci 356 213–227 Occurrence Handle11260802 Occurrence Handle10.1098/rstb.2000.0767 Occurrence Handle1:CAS:528:DC%2BD3MXjt1WjtrY%3D

    Article  PubMed  CAS  Google Scholar 

  • RW Hiorns JW Neal RCA Pearson TPS Powell (1991) ArticleTitleClustering of ipsilateral cortico-cortical projection neurons to area 7 in the rhesus monkey Proc R Soc London 246 1–9 Occurrence Handle10.1098/rspb.1991.0117 Occurrence Handle1:STN:280:By2C3c%2FnsFE%3D

    Article  CAS  Google Scholar 

  • KA Josephs JL Holton MN Rossor H Braendgaard T Ozawa NC Fox RC Petersen GS Pearl M Ganguly P Rosa H Laursen JE Parisi G Waldemar NP Quinn DW Dickson T Revesz (2003) ArticleTitleNeurofilament inclusion body disease: a new proteinopathy? Brain 126 2291–2303 Occurrence Handle12876145 Occurrence Handle10.1093/brain/awg231

    Article  PubMed  Google Scholar 

  • JP Julien (1999) ArticleTitleNeurofilament functions in health and disease Curr Opin Neurobiol 9 554–560 Occurrence Handle10508735 Occurrence Handle10.1016/S0959-4388(99)00004-5 Occurrence Handle1:CAS:528:DyaK1MXntVOisbo%3D

    Article  PubMed  CAS  Google Scholar 

  • RC Lariviere JP Julien (2004) ArticleTitleFunctions of intermediate filaments in neuronal development and disease J Neurobiol 58 131–148 Occurrence Handle14598376 Occurrence Handle10.1002/neu.10270 Occurrence Handle1:CAS:528:DC%2BD2cXlvVemuw%3D%3D

    Article  PubMed  CAS  Google Scholar 

  • IRA MacKenzie LP Hudson (1995) ArticleTitleAchromatic neurons in the cortex of progressive supranuclear palsy Acta Neuropathol 90 615–619 Occurrence Handle8615082 Occurrence Handle1:STN:280:BymC1M7nsF0%3D

    PubMed  CAS  Google Scholar 

  • RCA Pearson MM Esiri RW Hiorns GK Wilcock TPS Powell (1985) ArticleTitleAnatomical correlates of the distribution of the pathological changes in the neocortex in Alzheimer’s disease Proc Natl Acad Sci 82 4531–4534 Occurrence Handle3859874 Occurrence Handle10.1073/pnas.82.13.4531 Occurrence Handle1:STN:280:BiqB2c3ntF0%3D

    Article  PubMed  CAS  Google Scholar 

  • JP Taylor R Slater J French G Balluch PB Crino (2001) ArticleTitleTranscription of intermediate filament genes is enhanced in focal cortical dysplasia Acta Neuropathol 102 141–148 Occurrence Handle11563628 Occurrence Handle1:CAS:528:DC%2BD3MXlslGksL4%3D

    PubMed  CAS  Google Scholar 

  • KW Tseng KS Lu CL Chien (2006) ArticleTitleA possible cellular mechanism of neuronal loss in the dorsal root ganglia of Dystonia musculorum (dt) mice J Neuropath Exp Neurol 65 336–347 Occurrence Handle16691115 Occurrence Handle1:CAS:528:DC%2BD28XltFequ78%3D Occurrence Handle10.1097/01.jnen.0000218448.39944.f1

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Vision Sciences, Aston University, Birmingham, UK

    R. A. Armstrong

  2. Departments of Neurology and Pathology & Immunology, Washington University School of Medicine, St. Louis, Missouri, USA

    N. J. Cairns

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  1. R. A. Armstrong
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  2. N. J. Cairns
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Armstrong, R., Cairns, N. Spatial patterns of the pathological changes in neuronal intermediate filament inclusion disease (NIFID): an α-internexin immunohistochemical study. J Neural Transm 114, 451–456 (2007). https://doi.org/10.1007/s00702-006-0595-2

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  • DOI: https://doi.org/10.1007/s00702-006-0595-2

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