Advertisement

Acta Neuropathologica

, Volume 85, Issue 5, pp 553–559 | Cite as

The topographic distribution of brain atrophy in Huntington's disease and progressive supranuclear palsy

  • D. M. A. Mann
  • R. Oliver
  • J. S. Snowden
Regular Papers

Summary

The topographic distribution of brain atrophy was quantified by image analysis of fixed coronal brain slices from 12 patients dying with Huntington's disease (HD) and from 4 other patients dying with progressive supranuclear palsy (PSP). In HD, atrophy was maximal within the caudate nucleus, putamen and globus pallidus. However, the cerebral cortex was also atrophied with reductions in cross-sectional area within frontal, temporal and parietal lobes. In general, more white matter than grey matter was lost leading to an elevation in the grey/white matter ratio. The amygdala and thalamus were reduced in area. In PSP, lesser reductions in cortical area than those of HD were seen, these again being mostly due to a loss of white matter, resulting in an elevation of the grey/white ratio. The globus pallidus and thalamus were decreased in area, but no changes in the caudate nucleus and putamen were measured.

Key words

Brain atrophy Huntington's disease Progressive supranuclear palsy Subcortical dementia 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Albert ML, Feldman RG, Willis AL, (1974) The “subcortical dementia” of progressive supranuclear palsy. J Neurol Neurosurg Psychiat 37:121–130Google Scholar
  2. 2.
    Aquilonius S-M, Eckernas S-A, Sundwall A (1975) Regional distribution of choline acetyltransferase in the human brain: changes in Huntington's chorea. J Neurol Neurosurg Psychiat 38:669–677Google Scholar
  3. 3.
    Arendt T, Bigl V, Arendt A, Tennstedt A (1983) Loss of neurones from the nucleus basalis of Meynert in Alzheimer's disease, paralysis agitans and Korsakoff's disease. Acta Neuropathol (Berl) 61:101–108Google Scholar
  4. 4.
    Bamford KA, Caine ED, Kido DK, Plassche WM, Shoulson I (1989) Clinico-pathologic correlation in Huntington's disease. A neuropsychological and computed tomography study. Neurology 39:796–801Google Scholar
  5. 5.
    Berent S, Giordani B, Lehtinen S, Markel D, Penney JB, Buchtel HA, Starosta-Rubinstein S, Hichwa R, Young AB (1988) Positron emission tomographic scan investigations of Huntington's disease: cerebral metabolic correlates of cognitive function. Ann Neurol 23:541–546Google Scholar
  6. 6.
    Bird ED, Iversen LL (1974) Huntington's chorea: postmortem measurement of glutamic acid decarboxylase, choline acetyltransferase and dopamine in basal ganglia. Brain 97:457–472Google Scholar
  7. 7.
    Braak H, Jellinger K, Braak E, Bohl J (1992) Allocortical neurofibrillary changes in progressive supranuclear palsy. Acta Neuropathol 84:478–483Google Scholar
  8. 8.
    Brandt J, Folstein SE, Folstein MF (1988) Differential cognitive impairment in Alzheimer's disease and Huntington's disease. Ann Neurol 23:555–561Google Scholar
  9. 9.
    Bruyn GW (1968) Huntington's Chorea; historical, clinical and laboratory synopsis. Handb Clin Neurol 6:298–378Google Scholar
  10. 10.
    Bruyn GW (1973) Neuropathological changes in Huntington's Chorea. Adv Neurol 1:399–403Google Scholar
  11. 11.
    Bruyn GW, Bots TAM, Dom R (1979) Huntington's Chorea: current neuropathological status. Adv Neurol 23:83–93Google Scholar
  12. 12.
    Clark AW, Parhad IM, Folstein SE, Whitehouse PJ, Hedreen JC, Price DL, Chase GA (1983) The nucleus basalis in Huntington's disease. Neurology 33:1262–1267Google Scholar
  13. 13.
    Cross R (1982) Demonstration of neurofibrillary tangles in paraffin sections: a quick and simple method using Palmgrens technique. Med Lab Sci 39:67–69Google Scholar
  14. 14.
    Cudkowicz M, Kowall NW (1990) Degeneration of pyramidal projection neurons in Huntington's disease cortex. Ann Neurol 27:200–204Google Scholar
  15. 15.
    Cummings JL, Benson DF (1984) Subcortical dementia: review of an emerging concept. Arch Neurol 41:874–879Google Scholar
  16. 16.
    D'Antona R, Baron JC, Samson Y, Serdaru M, Viader F, Agid Y, Cambier J (1985) Subcortical dementia. Frontal cortex hypometabolism detected by positron tomography in patients with progressive supranuclear palsy. Brain 108:785–799Google Scholar
  17. 17.
    De La Monte SM, VonSattel J-P, Richardson EP (1988) Morphometric demonstration of atrophic changes in the cerebral cortex, white matter, and neostriatum in Huntington's disease. J Neuropathol Exp Neurol 47:516–525Google Scholar
  18. 18.
    Dom R, Baro F, Brucher JM (1973) A cytometric study of the putamen in different types of Huntington's Chorea. Adv Neurol 1:369–385Google Scholar
  19. 19.
    Dom R, Malfroid M, Baro F (1976) Neuropathology of Huntington's Chorea. Studies of the ventrobasal complex of the thalamus. Neurology 26:64–68Google Scholar
  20. 20.
    Dunlap CB (1927) Pathologic changes in Huntington's Chorea with special reference to the corpus striatum. Arch Neurol Psychiat 18:867–943Google Scholar
  21. 21.
    Ellison DW, Beal MF, Mazurck MF, Malloy ED, Bird ED, Martin JB (1987) Amino acid neurotransmitter abnormalities in Huntington's disease and the quinolinc acid animal model of Huntington's disease. Brain 110:1657–1673Google Scholar
  22. 22.
    Forno LS, Jose C (1973) Huntington's Chorea: a pathological study. Adv Neurol 1:453–470Google Scholar
  23. 23.
    Graveland GA, Williams RS, DiFiglia M (1985) Evidence for degenerative and regenerative changes in neostriatal spiny neurones in Huntington's disease. Science 227:770–773Google Scholar
  24. 24.
    Hedreen JC, Peyser CE, Folstein SE, Ross CA (1991) Neuronal loss in lavers V and VI of cerebral cortex in Huntington's disease. Neurosci Lett 133:257–261Google Scholar
  25. 25.
    Heinsen H (1991) Cortical nerve cell loss in Huntington's disease: a stereological investigation in five cases. Clin Neuropathol 10:257–263Google Scholar
  26. 26.
    Jellinger K, Bancher C (1992) Neuropathology. In: Litvan I, Agid Y (eds) Progressive supranuclear palsy. Clinical and research approaches. Oxford University Press, New York, pp 44–88Google Scholar
  27. 27.
    Jellinger K, Riederer P, Tomonaga M (1980) Progressive supranuclear palsy: clinico pathological and biochemical studies. J Neural Transm [Suppl] 16:111–128Google Scholar
  28. 28.
    Klintworth GK (1973) Huntington's Chorea: morphologic contributions of a century. Adv Neurol 1:353–368Google Scholar
  29. 29.
    Kowall NW, Ferrante RJ, Martin JB (1987) Neuropathology of Huntington's disease. Trends Neurosci 10:404–406Google Scholar
  30. 30.
    Kuwert T, Lange HW, Langen K-J, Herzog H, Aulich A, Feinendegen LE (1990) Cortical and subcortical glucose consumption measured by PET in patients with Huntington's disease. Brain 113:1405–1423Google Scholar
  31. 31.
    Lange H, Thörner G, Hopf A, Schröder KF (1976) Morphometric studies of the neuropathological changes in choreatic diseases. J Neurol Sci 28:401–425Google Scholar
  32. 32.
    Leenders KL, Frackowiak RSJ, Lees AJ (1988) Steele-Richardson-Olszewski syndrome. Brain energy metabolism, blood flow and fluorodopa uptake measured by positron emission tomography. Brain 111:615–630Google Scholar
  33. 33.
    Mann DMA (1989) Subcortical afferent projection systems in Huntington's Chorea. Acta Neuropathol 78:551–554Google Scholar
  34. 34.
    Mann DMA (1991) The topographic distribution of brain atrophy in Alzheimer's disease. Acta Neuropathol 83:81–86Google Scholar
  35. 35.
    Mann DMA, Brown AMT, Prinja D, Jones D, Davies CA (1990) A morphological analysis of senile plaques in the brains of non-demented persons of different ages using silver, immunocytochemical and lectin histochemical staining techniques. Neuropathol Appl Neurobiol 16:17–25Google Scholar
  36. 36.
    McCaughey WTE (1961) The pathologic spectrum of Huntington's chorea. J Nerv Ment Dis 133:91–103Google Scholar
  37. 37.
    McGeer PL, McGeer EG (1976) Enzymes associated with the metabolism of catecholamines, acetylcholine and GABA in human controls and patients with Parkinson's disease and Huntington's Chorea. J Neurochem 26:65–76Google Scholar
  38. 38.
    Neary D, Snowden JS, Shields RA, Burjan AWI, Northen B, MacDermott N, Prescott MC, Testa HJ (1987) Single photon emission tomography using 99m TcHMPAO in the investigation of dementia. J Neurol Neurosurg Psychiat 50:1101–1109Google Scholar
  39. 39.
    Neary D, Snowden JS, Northen B, Goulding P (1988) Dementia of frontal lobe type. J Neurol Neurosurg Psychiat 51:353–361Google Scholar
  40. 40.
    Oppenheimer DR, Esiri MM (1992) Diseases of the basal ganglia, cerebellum and motor neurones. In: Adams JH, Duchen LW (eds) Greenfields neuropathology, 5th edn. Edward Arnold, London, pp 988–1045Google Scholar
  41. 41.
    Pillon B, Dubois B, Ploska A, Agid Y (1991) Severity and specification of cognitive impairment in Alzheimer's. Huntington's Parkinson's diseases and progressive supranuclear palsy. Neurology 41:634–643Google Scholar
  42. 42.
    Reynolds GP, Pearson SJ (1987) Decreased glutamic acid and increased 5-hydroxytryptamine in Huntington's disease brain. Neurosci Lett 78:233–238Google Scholar
  43. 43.
    Reynolds GP, Pearson SJ, Heathfield KWG (1990) Dementia in Huntington's disease is associated with neurochemical deficits in the caudate nucleus not the cerebral cortex. Neurosci Lett 113:95–100Google Scholar
  44. 44.
    Roos RAC (1986) Neuropathology of Huntington's Chorea. Handb Clin Neurol 49:315–326Google Scholar
  45. 45.
    Salmon DP, Pow F, Yuen K, Heindel WC, Butters N, Thal LJ (1989) Differentiation of Alzheimer's disease and Huntington's disease with the dementia rating scale. Arch Neurol 46:1204–1208Google Scholar
  46. 46.
    Siegel S (1956) Non parametric statistics for the behavioural sciences. McGraw-Hill, New YorkGoogle Scholar
  47. 47.
    Sotrel A, Paskevich PA, Kiely DK, Bird ED, Williams RS, Myers RH (1991) Morphometric analysis of the prefrontal cortex in Huntington's disease. Neurology 41:1117–1123MathSciNetzbMATHGoogle Scholar
  48. 48.
    Spokes EGS (1980) Neurochemical alterations in Huntington's Chorea. A study of post-mortem brain tissue. Brain 103:179–210Google Scholar
  49. 49.
    Stahl WL, Swanson PD (1974) Biochemical abnormalities in Huntington's Chorea brains. Neurol 24:813–819Google Scholar
  50. 50.
    Steele JC, Richardson JD, Olszewski J (1964) Progressive supranuclear palsy. Arch Neurol 10:333–359PubMedGoogle Scholar
  51. 51.
    Tagliavini F, Pilleri G (1983) Basal nucleus of Meynert: a neuropathological study in Alzheimer's disease, simple senile dementia, Pick's disease and Huntington's Chorea. J Neurol Sci 62:243–260Google Scholar
  52. 52.
    Tagliavini F, Pilleri G, Bouras C, Constantinidis J (1984) The basal nucleus of Meynert in patients with progressive supranuclear palsy. Neurosci Lett 44:37–42Google Scholar
  53. 53.
    Tanahashi N, Meyer JS, Ishikawa Y, Kandula P, Mortel KF, Rogers RL, Gandhi S, Walker M (1985) Cerebral blood flow and cognitive testing correlate in Huntington's disease. Arch Neurol 42:1169–1175Google Scholar
  54. 54.
    Tellez-Nagel I, Johnson AB, Terry RD (1974) Studies on brain biopsies of patients with Huntington's Chorea. J Neuropathol Exp Neurol 33:308–332Google Scholar
  55. 55.
    VonSattel J-P, Myers RH, Stevens TJ, Ferrante RJ, Bird ED, Richardson EP (1985) Neuropathological classification of Huntington's disease. J Neuropathol Exp Neurol 44:559–577Google Scholar
  56. 56.
    Weinberger DR, Berman KF, Iadorola M, Driesen N, Zee RF (1988) Prefrontal cortical blood flow and cognitive function in Huntington's disease. J Neurol Neurosurg Psychiatry 51:91–104Google Scholar
  57. 57.
    Zalneraitis EL, Landis DMD, Richardson EP, Selkoe DJ (1981) A comparison of astrocytic protein in cerebral cortex and striatum in Huntington's disease. Neurology 31:151Google Scholar
  58. 58.
    Zweig RM, Ross CA, Hedreen JC, Peyser C, Cardillo JE, Folstein SE, Price DL (1992) Locus coeruleus involvement in Huntington's disease. Arch Neurol 49:152–156Google Scholar

Copyright information

© Springer-Verlag 1993

Authors and Affiliations

  • D. M. A. Mann
    • 1
  • R. Oliver
    • 1
  • J. S. Snowden
    • 2
  1. 1.Department of Pathological SciencesUniversity of ManchesterManchesterUK
  2. 2.Department of NeurologyThe Royal InfirmaryManchesterUK

Personalised recommendations