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Corticonigral degeneration with neuronal achromasia and basal neurofibrillary tangles

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Summary

A man, aged 58 years, suffered from progressive dementia, parkinsonism, and gaze paralysis for 30 months. Autopsy revealed severe degeneration of the substantia nigra, numerous swollen chromatolytic neurons within the cerebral cortex, scattered basal neurofibrillary tangles, and gliosis of the cerebral white matter and basal ganglia. Unusual globular inclusions positive for tau protein were detected within neurons of the upper cortical layers. Although the pathological findings were comparable with corticonigral degeneration with neuronal achromasia, several clinical and pathological features characteristic for progressive supranuclear palsy, progressive subcortical gliosis, and Pick's disease in this and the nine previously reported cases hampered the unequivocal nosological placement.

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References

  1. Bancher C, Lassmann H, Budka H, Grundke-Iqbal I, Iqbal K, Wiche G, Seitelberger F, Wisniewski HM (1989) Neurofibrillary tangles in Alzheimer's disease and progressive supranuclear palsy: antigenic similarities and differences. Microtubule-associated protein tau antigenicity is prominent in all types of tangles. Acta Neuropathol (Berl) 74:39–46

    Google Scholar 

  2. Bancher C, Lassmann H, Budka H, Jellinger K, Grundke-Iqbal I, Iqbal K, Wiche G, Seitelberger F, Wisniewski HM (1989) An antigenic profile of Lewy bodies: immunocytochemical indication for protein phosphorylation and ubiquitination. J Neuropathol Exp Neurol 48:81–93

    Google Scholar 

  3. Case Records of the Massachusetts General Hospital (1985) Case 38-1985. N Engl J Med 313:739–748

    Google Scholar 

  4. Clark AW, Manz HJ, White III 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 

  5. Colmant HJ (1971) Progressive supranuclear palsy. Arch Psychiatr Nervenkr 214:324–330

    Google Scholar 

  6. Constantinidis J, Tissot R, de Ajuriaguerra J (1970) Dystonie oculo-faciocervicale ou paralysie progressive supranucleaire de Steele-Richardson-Olszewski. Rev Neurol (Paris) 122:249–262

    Google Scholar 

  7. Constantinidis J, Richard J, Tissot R (1974) Pick's disease. Histological and clinical correlations. Eur Neurol 11:208–217

    Google Scholar 

  8. Dickson DW, Yen SH, 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 

  9. Dickson DW, Crystal H, Mattiace LA, Kress Y, Schwagerl A, Ksiezak-Reding H, Davies P, Yen SHC (1989) Diffuse Lewy body disease: light and electron microscopic immunocytochemistry of senile plaques. Acta Neuropathol 78:572–584

    Google Scholar 

  10. Dubinsky RM, Jankovic J (1987) Progressive supranuclear palsy and a multi-infarct state. Neurology 37:570–576

    Google Scholar 

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

    Google Scholar 

  12. Giaccone G, Tagliavini F, Street JS, Ghetti B, Bugiani O (1988) Progressive supranuclear palsy with hypertrophy of the olives. An immunocytochemical study of the cytoskeleton of argyrophilic neurons. Acta Neuropathol 77:14–20

    Google Scholar 

  13. Gibb WRG, Lees AJ (1988) The relevance of the Lewy body to the pathogenesis of idiopathic Parkinson's disease. J Neurol Neurosurg Psychiatry 51:745–752

    Google Scholar 

  14. Gibb WRG, Luthert PJ, Marsden CD (1989) Corticobasal degeneration. Brain 112:1171–1192

    Google Scholar 

  15. Gomori AJ, Sima AAF (1984) An atypical case of progressive supranuclear palsy. Can J Neurol Sci 11:48–52

    Google Scholar 

  16. Ishino H, Sasaki T, Yamashita K, Seno H, Kodaka H, Yoshinaga J, Ideshita H, Yamanaka T, Hikiji A (1987) A case of progressive supranuclear palsy with fibrillar gliosis of the midbrain and pontine reticular formation. Clin Neuropathol 6:61–66

    Google Scholar 

  17. Jankovic J (1984) Progressive supranuclear palsy: clinical and pharmacologic update. Neurol Clin 2:473–486

    Google Scholar 

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

    Google Scholar 

  19. Jellinger K, Bancher C, Lassmann H (1989) Cytoskeletal pathology of Lewy bodies. In: Przuntek H, Riederer P (eds) Early diagnosis and preventive therapy in Parkinson's disease. Springer, Wien, pp 181–189

    Google Scholar 

  20. Kosaka K, Tsuchiya K, Yoshimura M (1988) Lewy body disease with and without dementia: a clinicopathologic study of 35 cases. Clin Neuropathol 7:299–305

    Google Scholar 

  21. Kuzuhara S, Mori H, Izumiyama N, Yoshimura M, Ihara Y (1988) Lewy bodies are ubiquitinated. A light and electron microscopic immunocytochemical study. Acta Neuropathol (Berl) 75:345–353

    Google Scholar 

  22. Lee S, Park YD, Yen SHC, 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 

  23. Lippa CF, Smith TW, Fontneau N (1990) Corticonigral degeneration: a clinicopathological study of two cases. J Neurol Sci (in press)

  24. Mizusawa H, Matsumoto S, Yen SH, Hirano A, Rojas-Corona RR, Donnenfeld H (1989) Focal accumulation of phosphorylated neurofilaments within anterior horn cell in familial amyotrophic lateral sclerosis. Acta Neuropathol 79:37–43

    Google Scholar 

  25. Moossy J, Martinez AJ, Hanin I, Rao G, Yonas H, Boller F (1987) Thalamic and subcortical gliosis with dementia. Arch Neurol 44:510–513

    Google Scholar 

  26. Mori H, Yoshimura M, Tomonaga M, Yamanouchi H (1986) Progressive supranuclear palsy with Lewy bodies. Acta Neuropathol (Berl) 71:344–346

    Google Scholar 

  27. Neumann MA (1949) Pick's disease. J Neuropathol Exp Neurol 8:255–282

    Google Scholar 

  28. Neumann MA, Cohn R (1967) Progressive subcortical gliosis. A rare form of presenile dementia. Brain 40:405–418

    Google Scholar 

  29. Probst A, Anderton BH, Ulrich J, Kohler R, Kahn J, Heitz PU (1983) Pick's disease: an immunocytochemical study of neuronal changes. Monoclonal antibodies show that Pick bodies share antigenic determinants with neurofibrillary tangles and neurofilaments. Acta Neuropathol (Berl) 60:175–182

    Google Scholar 

  30. Rebeiz JJ, Kolodny EH, Richardson EP (1967) Corticodentatonigral degeneration with neuronal achromasia. Trans Am Neurol Assoc 92:23–26

    Google Scholar 

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

    Google Scholar 

  32. Seitelberger F (1968) Praesenile gliale Dystrophie. Acta Neuropathol [Suppl] (Berl) IV:109–118

    Google Scholar 

  33. Seitelberger F (1974) Achromatic neuronal dystrophy. VIIth International Congress of Neuropathology, Budapest, 1974. Akademiai Kiado, Budapest, p 276

    Google Scholar 

  34. Steele JC, Richardson JC, Olszeski J (1964) Progressive supranuclear palsy: a heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 10:333–359

    Google Scholar 

  35. Uchihara T, Tsuchiya K, Kosaka K (1990) Selective loss of nigral neurons in Pick's disease. A morphometric study. Acta Neuropathol (in press)

  36. Verity MA, Wechsler AF (1987) Progressive subcortical gliosis of Neumann: a clinicopathologic study of two cases with review. Arch Gerontol Geriatr 6:245–261

    Google Scholar 

  37. Watts RL, Williams RS, Growdon JD, Young RR, Haley EC, Beal MF (1985) Corticobasal ganglionic degeneration. Neurology 35 [Suppl 1]:178

    Google Scholar 

  38. Weiler R, Lassmann H, Fischer P, Jellinger K, Winkler H (1990) A high ratio of chromogranin A to synaptophysin is a common feature of brains in Alzheimer and Pick disease. FEBS Lett (in press)

  39. Will RG, Lees AJ, Gibb W, Barnard RO (1988) A case of progressive subcortical gliosis presenting clinically as Steele-Richardson-Olszewski syndrome. J Neurol Neurosurg Psychiatry 51:1224–1227

    Google Scholar 

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Authors and Affiliations

  1. Ludwig-Boltzmann-Institut für Klinische Neurobiologie, Wien, Austria

    W. Paulus

  2. Neurologisches Krankenhaus Marie-Theresien-Schloessel, Wien, Austria

    M. Selim

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  1. W. Paulus
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  2. M. Selim
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Paulus, W., Selim, M. Corticonigral degeneration with neuronal achromasia and basal neurofibrillary tangles. Acta Neuropathol 81, 89–94 (1990). https://doi.org/10.1007/BF00662643

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  • DOI: https://doi.org/10.1007/BF00662643

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