Skip to main content
SpringerLink
Log in

Striatonigral degeneration, olivopontocerebellar atrophy and „atypical” Pick disease

  • Regular Papers
  • Published:
Acta Neuropathologica Aims and scope Submit manuscript

Summary

A 75-year-old woman with parkinsonism plus was found at autopsy to have striatonigral degeneration (SND), olivopontocerebellar atrophy (OPCA) and intracytoplasmic neuronal inclusions, mostly confined to the hippocampus and pontine nuclei. These inclusions were intensely argyrophilic, ubiquitinated and expressed variable immunoreactivity for neurofilament but not for tau-1 and Alz 50 proteins. Ultrastructurally, they were formed of skeins of intermediate filaments averaging 11 nm in diameter. They were considered to represent Pick bodies. There was no cortical atrophy, gliosis or sponginess. To our knowledge, SND and OPCA in association with Pick's disease has not been previously reported. In addition, intracytoplasmic oligodendroglial inclusions were present in the deeper layers of the cortex, especially the pericentral gyri, the striatum and the white matter of certain regions of the cerebral hemispheres, as well as in the cerebellum. These inclusions which have been previously reported in multisystem atrophy, had to be distinguished from cortical Lewy bodies, Pick bodies, and the nonspecific ubiquitinated bodies in the white matter of the aged brain, mainly by their topographical distribution and immunostaining properties.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Adam RD, Salam-Adams M (1986) Striatonigral degeneration. Handbook Clin Neurol 49:205–212

    Google Scholar 

  2. Arima K (1989) Involvement of subcortical nuclei and brain stem in Pick's disease: a topographical study of Pick bodies. Neuropathology 9:105–115

    Google Scholar 

  3. Bannister R, Oppenheimer DR (1972) Degenerative diseases of the nervous system associated with autonomic failure. Brain 95:457–474

    Google Scholar 

  4. Binder LI, Frankfurter A, Rebhun LI (1985) Distribution of tau in the mammalian central nervous system. J Cell Biol 101:1371–1378

    Google Scholar 

  5. Borrett D, Becker LE (1985) Alexander's disease, a disease of astrocytes. Brain 108:367–385

    Google Scholar 

  6. Buonanno F, Nardelli E, Onnis L, Rizzuto N (1975) Striatonigral degeneration: report of a case with an unusually short course and multiple system degeneration. J Neurol Sci 26:545–553

    Google Scholar 

  7. Cambier J, Masson M, Dairon R, Henin D (1981) Etude anatomoclinique d'une forme parietale de maladie de Pick. Rev Neurol (Paris) 137:33–38

    Google Scholar 

  8. Clark AW, Manz HJ, White CL, Lehmann J, Miller D, Coyle JT (1986) Cortical degeneration with swollen chromatolytic neurons. Its relationship to Pick's disease. J Neuropathol Exp Neurol 45:268–284

    Google Scholar 

  9. Constantinidis J (1985) Pick dementia: anatomoclinical correlations and pathophysiological considerations. Interdiscip Top Gerontol 19:72–97

    Google Scholar 

  10. deLeon GA, Breningstall G, Zaeri N (1986) Congenital Pick cell encephalopathy: a distinct disorder characterized by diffuse formation of Pick cells in the cerebral cortex. Acta Neuropathol (Berl) 70:235–242

    Google Scholar 

  11. Dickson DW, Yen S-H, Horoupian DS (1986) Pick body-like inclusions in the dentate fascia of the hippocampus in Alzheimer's disease. Acta Neuropathol (Berl) 71:38–45

    Google Scholar 

  12. Dickson DW, Yen S-H, Suzuki KI, Davies P, Garcia JH, Hirano A (1986) Ballooned neurons in select neurodegenerative diseases contain phosphorylated neurofilament epitopes. Acta Neuropathol (Berl) 71:216–223

    Google Scholar 

  13. Dickson DW, Davies P, Mayeux R, Crystall H, Horoupian DS, Thompson A, Goldman JE (1987) Diffuse Lewy body disease: neuropathological and biochemical studies of six patients. Acta Neuropathol (Berl) 75:8–15

    Google Scholar 

  14. Dickson DW, Wertkin A, Kress Y, Ksiezak-Reding H, Yen S-H (1990) Ubiquitin immunoreactive structures in normal brains: distribution and developmental aspects. Lab Invest (in press)

  15. Forno LS, Eng LF, Selkoe DJ (1989) Pic bodies in the locus ceruleus. Acta Neuropathol 79:10–17

    Google Scholar 

  16. Gambetti P, Schecket GF, Ghetti B, Hirano A, Dahl D (1983) Neurofibrillary changes in human brain: an immunocytochemical study with neurofilament antisera. J Neuropathol Exp Neurol 42:69–79

    Google Scholar 

  17. Gibb WRG, Esiri MM, Lees AJ (1985) Clinical and pathological features of diffuse cortical Lewy body disease (Lewy body dementia). Brain 110:1131–1153

    Google Scholar 

  18. Gosset A, Pelissier JF, Delpeuch F, Khalil R (1983) Degenerescence striato-nigrique associée à une atrophie olivo-pontocerebelleuse. Etude anatomoclinique de trois cas — Discussion nosologique. Rev Neurol (Paris) 139:125–139

    Google Scholar 

  19. Horoupian DS, Kress Y, Gaskin F, Yen S-H (1982) Nickel induced changes and reappraisal of Rosenthal fibers in focal CNS lesions. J Neuropathol Exp Neurol 41:664–675

    Google Scholar 

  20. Jakob H (1979) Die Picksche Krankheit: eine neuropathologisch-anatomisch-klinische Studie. Springer-Verlag, Berlin Heidelberg New York

    Google Scholar 

  21. Kato S, Nakamura H (1990) Cytoplasmic argyrophilic inclusions in neurons of pontine nuclei in patients with olivopontocerebellar atrophy: immunohistochemical and ultrastructural studies. Acta Neuropathol 79:584–594

    Google Scholar 

  22. Kato S, Nakamura H, Otomo E (1989) Reappraisal of neurofibrillary tangles. Immunohistochemical, ultrastructural and immunoelectron microscopic studies. Acta Neuropathol 77:258–266

    Google Scholar 

  23. Kosaka K, Iizuka R, Mizutani Y, Kondo T, Nagatsu T (1981) Striato-nigral degeneration combined with Alzheimer's disease. Acta Neuropathol (Berl) 54:253–256

    Google Scholar 

  24. Kosaka K, Yoshimura M, Ikeda K, Budka H (1984) Diffuse type of Lewy body disease: progressive dementia and abundant cortical Lewy bodies and senile changes of varying degree — A new disease? Clin Neuropathol 3:185–192

    Google Scholar 

  25. Ksiezak-Reding H, Davies P, Yen S-H (1988) Alz 50, a monoclonal antibody to Alzheimer's disease antigen, cross-reacts with tau proteins from bovine and normal human brain. J Biol Chem 263:7943–7947

    Google Scholar 

  26. Lee S, Park YD, Yen S-H, Ksiezak-Reding H, Goldman JE, Dickson DW (1989) A study of infantile motor neuron disease with neurofilament and ubiquitin immunocytochemistry. Neuropediatrics 20:107–111

    Google Scholar 

  27. Lennox G, Lowe J, Morrell K, Landon M, Mayer RJ (1989) Anti-ubiquitin immunocytochemistry is more sensitive than conventional techniques in the detection of diffuse Lewy body disease. J Neurol Neurosurg Psychiatry 52:67–71

    Google Scholar 

  28. Love S, Saitoh T, Quijada S, Cole GM, Terry RD (1988) Alz 50, ubiquitin and Tau immunoreactivity of neurofibrillary tangles, Pick bodies and Lewy bodies. J Neuropathol Exp Neurol 47:393–405

    Google Scholar 

  29. Mikol J, Brion S, Guicharnaud L, Waks O (1980) A new case of Pick's disease. Anatomical and ultrastructural studies. Acta Neuropathol (Berl) 49:57–61

    Google Scholar 

  30. Munoz-Garcia D, Ludwin SK (1984) Classic and generalized variants of Pick's disease: a clinicopathological, ultrastructural and immunocytochemical comparative study. Ann Neurol 16:467–480

    Google Scholar 

  31. Murayama S, Mori H, Ihara Y, Tomonaga M (1990) Immunocytochemical and ultrastructural studies of Pick's disease. Ann Neurol 27:394–405

    Google Scholar 

  32. Oppenheimer RD (1984) Diseases of the basal ganglia, cerebellum and motor neurons. In: Adams JH, Corsellis JAN, Duchen LW (eds) Greenfield's Neuropathology 4th edn. Edward Arnold, London, pp 699–747

    Google Scholar 

  33. Papolla MA, Omar R, Saran B (1989) The “Normal” brain. “Abnormal” ubiquitinilated deposits highlight an age-related protein change. Am J Pathol 135:585–591

    Google Scholar 

  34. Papp MI, Kahn JE, Lantos PL (1989) Glial cytoplasmic inclusions in the CNS of patients with multiple system atrophy (striatonigral degeneration, olivopontocerebellar atrophy and Shy-Drager syndrome). J Neurol Sci 94:79–100

    Google Scholar 

  35. Probst A, Anderton BH, Ulrich J, Kohler R, Kahn J, Heitz PU (1983) Pick's disease: an immunocytochemical study of neuronal changes. Acta Neuropathol (Berl) 60:175–182

    Google Scholar 

  36. Rasool CG, Selkoe DJ (1985) Sharing of specific antigens by degenerating neurons in Pick's disease and Alzheimer's disease. N Engl J Med 312:700–705

    Google Scholar 

  37. Rebeiz JJ, Kolodny EH, Richardson EP (1968) Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 18:20–33

    Google Scholar 

  38. Seitelberger F (1975) Achromatic neuronal dystrophy. VIIth International Congress on Neuropathology, vol 1. Akademiai Kiado, Budapest, pp 299–305

    Google Scholar 

  39. Seitelberger F, Gross H, Pilz P (1983) Pick's disease: a neuropathologic study. In: Hirano A, Miyoshi K (eds) Neuropsychiatric Disorders in the Elderly. Proceedings of the International Conference III. Igaku-Shoin, Tokyo New York, pp 87–117

    Google Scholar 

  40. Takauchi S, Hosomi M, Marasigan S, Sato M, Hayashi S, Miyoshi K (1984) An ultrastructural study of Pick bodies. Acta Neuropathol (Berl) 64:344–348

    Google Scholar 

  41. Takei Y, Mirra SS (1973) Striatonigral degeneration — A form of multiple system atrophy. Prog Neuropathol 2:217–251

    Google Scholar 

  42. Tiller-Borcich JK, Forno LS (1988) Parkinson's disease and dementia with neuronal inclusions in the cortex: Lewy bodies or Pick bodies. J Neuropathol Exp Neurol 47:526–535

    Google Scholar 

  43. Towfighi J (1972) Early Pick's disease. A light and ultrastructural study. Acta Neuropathol (Berl) 21:224–231

    Google Scholar 

  44. Trotter JL (1973) Striatonigral degeneration, Alzheimer's disease and inflammatory changes. Neurology 23:1211–1216

    Google Scholar 

  45. Wisniewski HM, Coblenth JM, Terry RD (1972) Pick's disease: a clinical and ultrastructural study. Arch Neurol 26:97–108

    Google Scholar 

  46. Wolozin BL, Pruchnicki A, Dickson DW, Davies P (1986) A neuronal antigen in the brains of Alzheimer patients. Science 232:648–650

    Google Scholar 

  47. Yoshimura N (1989) Topography of Pick body distribution in Pick's disease: contribution to understanding the relationship between Pick's and Alzheimer's diseases. Clin Neuropathol 8:1–6

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pathology/Neuropathology, Stanford University School of Medicine, 94305-5324, Stanford, CA, USA

    D. S. Horoupian

  2. Neuropathology, Albert Einstein College of Medicine, 1300 Morris Park Avenue, 10461, Bronx, NY, USA

    D. W. Dickson

Authors
  1. D. S. Horoupian
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. D. W. Dickson
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Horoupian, D.S., Dickson, D.W. Striatonigral degeneration, olivopontocerebellar atrophy and „atypical” Pick disease. Acta Neuropathol 81, 287–295 (1991). https://doi.org/10.1007/BF00305870

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00305870

Key words

Search

Navigation