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. 2006 Apr 5;8(2):403–406. doi: 10.1111/j.1750-3639.1998.tb00163.x

Establishing a Pathological Diagnosis in Degenerative Dementias

James Lowe 1,, DM FRCPath 1
PMCID: PMC8098334  PMID: 9546296

Abstract

While clinicopathological studies have confirmed that Alzheimer's disease (AD) is the most common neurodegenerative cause of dementia, these same studies have also revealed that other degenerative pathologies account for a significant proportion of patients with cognitive decline. Because pathological assessment of non‐Alzheimer neurodegenerative diseases now demands routine use of a costly panel of immunohistochemical techniques a scheme for staged examination of brain tissue has been developed. This scheme is weighted to initially screen out cases of Alzheimer's disease, dementia with Lewy bodies and vascular dementia using conventional staining methods and established diagnostic protocols, bringing in immunochemical techniques to discriminate between non‐Alzheimer degenerative dementias. Diagnosis of pathologies causing the clinical syndrome of frontotemporal dementia can be ascertained using conventional staining supplemented by immunochemical detection of ubiquitin, tau protein and α B crystallin. The diagnosis of prion disease is reliably confirmed by immunohistochemical detection of prion protein. This morphological assessment complements emerging genetic insights into many of these neurodegenerative diseases.

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References

  • 1. Alexander EM, Wagner EH, Buchner DM, Cain KC, Larson EB (1995) Do surgical brain lesions present as isolated dementia? A population‐based study. Journal of the American Geriatrics Society 43: 138–43. [DOI] [PubMed] [Google Scholar]
  • 2. Bell JE, Gentleman SM, Ironside JW, McCardle L, Lantos PL, Doey L, Lowe J, Fergusson J, Luthert P, McQuaid S, Allen IV (1997) Prion protein immunocytochemistry–UK five centre consensus report. Neuropathology & Applied Neurobiology 23: 26–35. [PubMed] [Google Scholar]
  • 3. Bergeron C, Davis A, AE L (1998) Corticobasal ganglionic degeneration and progressive supranuclear palsy presenting with cognitive decline. Brain Pathology 8: 355–365. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 4. Budka H, Aguzzi A, Brown P, Brucher JM, Bugiani O, Collinge J, Diringer H, Gullotta F, Haltia M, Hauw JJ, Ironside JW, Kretzschmar HA, Lantos PL, Masullo C, Pocchiari M, Schlote W, Tateishi J, Will RG (1995) Tissue handling in suspected Creutzfeldt‐Jakob disease (CJD) and other human spongiform encephalopathies (Prion diseases). Brain Pathology 5: 319–322. [DOI] [PubMed] [Google Scholar]
  • 5. Coker SB (1991) The diagnosis of childhood neurodegenerative disorders presenting as dementia in adults. Neurology 41: 794–8. [DOI] [PubMed] [Google Scholar]
  • 6. Cooper PN, Jackson M, Lennox G, Lowe J, Mann DMA (1995) tau, ubiquitin, and alphaB‐crystallin immunohistochemistry define the principal causes of degenerative frontotemporal dementia. Archives of Neurology 52: 1011–1015. [DOI] [PubMed] [Google Scholar]
  • 7. CoreyBloom J, Sabbagh MN, Bondi MW, Hansen L, Alford MF, Masliah E, Thai LJ (1997) Hippocampal sclerosis contributes to dementia in the elderly. Neurology 48: 154–160. [DOI] [PubMed] [Google Scholar]
  • 8. Dickson D (1998) Pick's disease ‐ a modern approach. Brain Pathology 8: 339–354. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 9. Dickson DW, Davies P, Bevona C, van Hoeven KH, Factor SM, Grober E, Aronson MK, Crystal HA (1994) Hippocampal sclerosis: A common pathological feature of dementia in very old (80 years of age) humans. Acta Neuropathologica 88: 212–221. [DOI] [PubMed] [Google Scholar]
  • 10. Ince P, Perry E, Morris C (1998) Dementia with Lewy Bodies ‐ a distinct non‐Alzheimer dementia syndrome Brain Pathology 00: 000–000. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 11. Jackson M, Lennox G, Lowe J (1996) Motor neurone disease‐inclusion dementia. Neurodegeneration 5: 339–350. [DOI] [PubMed] [Google Scholar]
  • 12. Jackson M, Lowe J (1996) The new neuropathology of degenerative frontotemporal dementias. Acta Neuropathologica 91: 127–134. [DOI] [PubMed] [Google Scholar]
  • 13. Jellinger K (1998) Dementia with Grains (Argyrophilic Grain Disease). Brain Pathology 8: 377–386. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 14. Jellinger K, Bancher C (1998) Senile Dementia with Tangles (Tangle predominant form of senile dementia). Brain Pathology 8: 367–376. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 15. Lowe J (1997) Degenerative Non‐Alzheimer dementias. Brain Pathology 7: 1047–1051. [Google Scholar]
  • 16. Mann D (1998) Dementia of frontal types and dementia with subcortical gliosis. Brain Pathology 8: 325–338. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 17. McKeith IG, Galasko D, Kosaka K, Perry EK, Dickson DW, Hansen LA, Salmon DP, Lowe J, Mirra SS, Byrne EJ, Lennox G, Quinn NP, Edwardson JA, Ince PG, Bergeron C, Burns A, Miller BL, Lovestone S, Collerton D, et al (1996) Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): Report of the consortium on DLB international workshop. Neurology 47: 1113–1124. [DOI] [PubMed] [Google Scholar]
  • 18. Mirra SS, Hart MN, Terry RD (1993) Making the diagnosis of Alzheimer's disease. A primer for practicing pathologists. Archives of Pathology & Laboratory Medicine 117: 132–44. [PubMed] [Google Scholar]
  • 19. Mirra SS, Heyman A, McKeel D, Sumi SM, Crain BJ, Brownlee LM, Vogel FS, Hughes JP, van Belle G, Berg L (1991) The Consortium to Establish a Registry for Alzheimer's Disease (CERAD). Part II. Standardization of the neuropathologic assessment of Alzheimer's disease. Neurology 41: 479–486. [DOI] [PubMed] [Google Scholar]
  • 20. National Institue on Aging and Reagan Institute Working Group on Diagnostic Criteria for the Neuropathological Assessment of Alzheimer's Disease (1997) Consensus recommendations for the postmortem diagnosis of Alzheimer's disease. Neurobiol Aging 18 (Suppl): S1–2. [PubMed] [Google Scholar]
  • 21. Papp Ml, Lantos PL (1992) Accumulation of tubular structures in oligodendroglial and neuronal cells as the basic alteration in multiple system atrophy. J Neurol Sci 107: 172–82. [DOI] [PubMed] [Google Scholar]
  • 22. Roman GC, Tatemichi TK, Erkinjuntti T, Cummings JL, Masdeu JC, Garcia JH, Amaducci L, Orgogozo JM, Brun A, Hofman A, et al. (1993) Vascular dementia: diagnostic criteria for research studies. Report of the NINDS‐AIREN International Workshop. Neurology 43: 250–60. [DOI] [PubMed] [Google Scholar]
  • 23. Spillantini M, Bird T, Ghetti B (1998) Frontotemporal Dementia and Parkinsonism linked to chromosome 17: A new group of Tauopathies. Brain Pathology 8: 387–402. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 24. Tolnay M, Probst A (1995) Frontal lobe degeneration: Novel ubiquitin‐immunoreactive neurites within frontotemporal cortex. Neuropathology and Applied Neurobiology 21: 492–497. [DOI] [PubMed] [Google Scholar]

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