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

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.

This is a preview of subscription content, log in to check access.

References

  1. 1.

    Albert ML, Feldman RG, Willis AL, (1974) The “subcortical dementia” of progressive supranuclear palsy. J Neurol Neurosurg Psychiat 37:121–130

    Google 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 38:669–677

    Google 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) 61:101–108

    Google 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 39:796–801

    Google 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 23:541–546

    Google 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 97:457–472

    Google Scholar 

  7. 7.

    Braak H, Jellinger K, Braak E, Bohl J (1992) Allocortical neurofibrillary changes in progressive supranuclear palsy. Acta Neuropathol 84:478–483

    Google Scholar 

  8. 8.

    Brandt J, Folstein SE, Folstein MF (1988) Differential cognitive impairment in Alzheimer's disease and Huntington's disease. Ann Neurol 23:555–561

    Google Scholar 

  9. 9.

    Bruyn GW (1968) Huntington's Chorea; historical, clinical and laboratory synopsis. Handb Clin Neurol 6:298–378

    Google Scholar 

  10. 10.

    Bruyn GW (1973) Neuropathological changes in Huntington's Chorea. Adv Neurol 1:399–403

    Google Scholar 

  11. 11.

    Bruyn GW, Bots TAM, Dom R (1979) Huntington's Chorea: current neuropathological status. Adv Neurol 23:83–93

    Google 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 33:1262–1267

    Google Scholar 

  13. 13.

    Cross R (1982) Demonstration of neurofibrillary tangles in paraffin sections: a quick and simple method using Palmgrens technique. Med Lab Sci 39:67–69

    Google Scholar 

  14. 14.

    Cudkowicz M, Kowall NW (1990) Degeneration of pyramidal projection neurons in Huntington's disease cortex. Ann Neurol 27:200–204

    Google Scholar 

  15. 15.

    Cummings JL, Benson DF (1984) Subcortical dementia: review of an emerging concept. Arch Neurol 41:874–879

    Google 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 108:785–799

    Google 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 47:516–525

    Google 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 1:369–385

    Google Scholar 

  19. 19.

    Dom R, Malfroid M, Baro F (1976) Neuropathology of Huntington's Chorea. Studies of the ventrobasal complex of the thalamus. Neurology 26:64–68

    Google Scholar 

  20. 20.

    Dunlap CB (1927) Pathologic changes in Huntington's Chorea with special reference to the corpus striatum. Arch Neurol Psychiat 18:867–943

    Google 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 110:1657–1673

    Google Scholar 

  22. 22.

    Forno LS, Jose C (1973) Huntington's Chorea: a pathological study. Adv Neurol 1:453–470

    Google 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 227:770–773

    Google 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 133:257–261

    Google Scholar 

  25. 25.

    Heinsen H (1991) Cortical nerve cell loss in Huntington's disease: a stereological investigation in five cases. Clin Neuropathol 10:257–263

    Google 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 44–88

    Google Scholar 

  27. 27.

    Jellinger K, Riederer P, Tomonaga M (1980) Progressive supranuclear palsy: clinico pathological and biochemical studies. J Neural Transm [Suppl] 16:111–128

    Google Scholar 

  28. 28.

    Klintworth GK (1973) Huntington's Chorea: morphologic contributions of a century. Adv Neurol 1:353–368

    Google Scholar 

  29. 29.

    Kowall NW, Ferrante RJ, Martin JB (1987) Neuropathology of Huntington's disease. Trends Neurosci 10:404–406

    Google 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 113:1405–1423

    Google Scholar 

  31. 31.

    Lange H, Thörner G, Hopf A, Schröder KF (1976) Morphometric studies of the neuropathological changes in choreatic diseases. J Neurol Sci 28:401–425

    Google 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 111:615–630

    Google Scholar 

  33. 33.

    Mann DMA (1989) Subcortical afferent projection systems in Huntington's Chorea. Acta Neuropathol 78:551–554

    Google Scholar 

  34. 34.

    Mann DMA (1991) The topographic distribution of brain atrophy in Alzheimer's disease. Acta Neuropathol 83:81–86

    Google 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 16:17–25

    Google Scholar 

  36. 36.

    McCaughey WTE (1961) The pathologic spectrum of Huntington's chorea. J Nerv Ment Dis 133:91–103

    Google 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 26:65–76

    Google 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 50:1101–1109

    Google Scholar 

  39. 39.

    Neary D, Snowden JS, Northen B, Goulding P (1988) Dementia of frontal lobe type. J Neurol Neurosurg Psychiat 51:353–361

    Google 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 988–1045

    Google 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 41:634–643

    Google Scholar 

  42. 42.

    Reynolds GP, Pearson SJ (1987) Decreased glutamic acid and increased 5-hydroxytryptamine in Huntington's disease brain. Neurosci Lett 78:233–238

    Google 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 113:95–100

    Google Scholar 

  44. 44.

    Roos RAC (1986) Neuropathology of Huntington's Chorea. Handb Clin Neurol 49:315–326

    Google 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 46:1204–1208

    Google Scholar 

  46. 46.

    Siegel S (1956) Non parametric statistics for the behavioural sciences. McGraw-Hill, New York

    Google 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 41:1117–1123

    MathSciNet  MATH  Google Scholar 

  48. 48.

    Spokes EGS (1980) Neurochemical alterations in Huntington's Chorea. A study of post-mortem brain tissue. Brain 103:179–210

    Google Scholar 

  49. 49.

    Stahl WL, Swanson PD (1974) Biochemical abnormalities in Huntington's Chorea brains. Neurol 24:813–819

    Google Scholar 

  50. 50.

    Steele JC, Richardson JD, Olszewski J (1964) Progressive supranuclear palsy. Arch Neurol 10:333–359

    CAS  PubMed  Google 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 62:243–260

    Google 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 44:37–42

    Google 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 42:1169–1175

    Google 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 33:308–332

    Google 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 44:559–577

    Google 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 51:91–104

    Google 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 31:151

    Google 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 49:152–156

    Google Scholar 

Download references

Author information

Affiliations

Authors

Additional information

Supported in part by an intercalated BSc studentship (RO) from the Medical Research Council

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Mann, D.M.A., Oliver, R. & Snowden, J.S. The topographic distribution of brain atrophy in Huntington's disease and progressive supranuclear palsy. Acta Neuropathol 85, 553–559 (1993). https://doi.org/10.1007/BF00230496

Download citation

Key words

  • Brain atrophy
  • Huntington's disease
  • Progressive supranuclear palsy
  • Subcortical dementia