Skip to main content

Advertisement

Log in

A case of Pick's disease with unusual neuronal inclusions

  • Case Report
  • Published:
Acta Neuropathologica Aims and scope Submit manuscript

Abstract

An autopsy case of unusual Pick's diesease in a 61-year-old male is described. Findings included severe atrophy of the frontal and temporal lobes, pyramidal tracts and basal ganglia accompanied by numerous intraneuronal argyrophilic hyaline inclusions. His neurological symptoms were constantly progressive during the 12-year course, characterized by akinesia and emotional incontinence. The inclusions were round, well-demarcated, slightly eosinophilic and intensely argyrophilic bodies in the perikarya, and distributed mainly in the subiculum and Sommer's sector of the hippocampus, amygdala and affected gyri. Immunocytochemically, they contain antigenic determinants of both phosphorylated and nonphosphorylated neurofilaments, but were negative for ubiquitin. Ultrastructurally, they were composed primarily of skeins of neurofilaments intermingled with cell organelles. Tubular profiles studded with granular substances, previously reported as a feature of the generalized variant of Pick's disease, and Hirano body-like lattice structures were occasionally observed in the inclusions. This case represents a slowly progressive neurodegenerative disorder characterized by fronto-temporal lobar atrophy and might by categorized as a variant of Pick's disease. However, some unusual properties of neuronal inclusions may suggest a different pathogenesis from that in classical Pick's disease.

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

Access this article

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

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

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Brion S, Mikol J, Psimaras A (1973) Recent findings in Pick's disease. In: zimmerman HM (ed) Progress in neuropathology, vol 2. Grune and Stratton, New York, pp 421–452

    Google Scholar 

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

    Google Scholar 

  3. Guesdon JL, Ternynck T, Avrameas S (1979) The use of avidin-biotin interaction in immunoenzymatic techniques. J Histochem Cytochem 27: 1131–1139

    Google Scholar 

  4. Ihara Y (1988) Massive somatodendritic sprouting of cortical neurons in Alzheimer's disease. Brain Res 459: 138–144

    Google Scholar 

  5. Ihara Y, Carmela A, Selkoe DJ (1983) Antibodies to paired helical filaments in Alzheimer's disease do not recognize normal brain proteins. Nature 304: 727–730

    Google Scholar 

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

    Google Scholar 

  7. Kato S, Nakamura H (1990) Presence of two different fibril subtypes in the Pick body: an immunoelectron microscopic study. Acta Neuropathol 81: 125–129

    Google Scholar 

  8. Leigh PN, Probst A, Dale GE, Power DP, Brion JP, Dodson A, Anderton BH (1989) New aspects of the pathology of neurodegenerative disorders as revealed by ubiquitin antibodies. Acta Neuropathol 79: 61–72

    Google Scholar 

  9. 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 

  10. Mochizuki A, Mizusawa H, Ohkoshi N, Yoshizawa K, Komatsuzaki Y, Inoue K, Kanazawa I (1992) Argyrophilic intracytoplasmic inclusions in multiple system atrophy. J Neurol 239: 311–316

    Google Scholar 

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

    Google Scholar 

  12. Murayama S, Nukina N, Ihara Y, Nakazato Y, Ishida Y (1985) Immunocytochemistry of Pick's argyrophilic bodies: evidence for the involvement of high molecular weight microtubule-associated proteins (HMWP) before the appearance of tubulin. Clin Neurol (Tokyo) 25: 80–87 (in Japanese)

    Google Scholar 

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

    Google Scholar 

  14. Nakazato Y, Sasaki A, Hirato J, Ishida Y (1984) Immunohistochemical localization of neurofilament protein in neuronal degenerations. Acta Neuropathol (Berl) 64: 30–36

    Google Scholar 

  15. Nakazato Y, Ishida Y, Hoshi S, Amano Y (1985) Juvenile dementia with several types of neuronal inclusions. An autopsy case of atypical Pick's disease. Neuropathology 6: 19–32 (in Japanese)

    Google Scholar 

  16. Nakazato Y, Sasaki A, Hirato J, Ishida Y (1987) Monoclonal antibodies which recognize phosphorylated and nonphosphorylated epitopes of neurofilament protein. Biomed Res 8: 369–376

    Google Scholar 

  17. Oda M, Akagawa N, Tabuchi Y, Tanabe H (1978) A sporadic juvenile case of the amyotrophic lateral sclerosis with neuronal intracytoplasmic inclusions. Acta Neuropathol (Berl) 44: 211–216

    Google Scholar 

  18. Oyanagi S (1974) An electron microscopic observation on senile dementia, with special references to transformation of neurofilaments to twisted tubules and a structural connection of Pick bodies to Alzheimer's neurofibrillary changes. Shinkei Kenkyu No Shinpo 18: 77–88 (in Japanese)

    Google Scholar 

  19. Oyanagi S, Tanaka M, Omori T, Matsushita M, Ishii T (1979) Regular rearrangements of tubular structures in Pick bodies formed in an autopsied case of Pick's disease. Shinkei Kenkyu No Shinpo 23: 441–451 (in Japanese)

    Google Scholar 

  20. Peña CE, Katoh A (1989) Intracytoplasmic eosinophilic inclusions in the neurons of the central nervous system. Acta Neuropathol 79: 73–77

    Google Scholar 

  21. Probst A, Anderton H, Ulich 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 

  22. Rewcastle NB, Ball MJ (1968) Electron microscopic structure of the “inclusion bodies” in Pick's disease. Neurology 18: 1205–1213

    Google Scholar 

  23. Schochet SS, Lampert PW, Lindenberg R (1968) Fine structure of the Pick and Hirano bodies in a case of Pick's disease. Acta Neuropathol (Berl) 11: 330–337

    Google Scholar 

  24. Shibayama H, Kitoh J, Marui Y, Kobayashi H, Iwase S, Kayukawa Y (1983) An unusual case of Pick's disease. Acta Neuropathol (Berl) 59: 79–87

    Google Scholar 

  25. Sternberger LA, Hardy PH, Cuculis JJ, Meyer HG (1970) The unlabeled antibody enzyme method of immunohistochemistry. J Histochem Cytochem 18: 315–333

    Google Scholar 

  26. 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 

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

    Google Scholar 

  28. Ulich J, Haugh M, Anderton H, Probst A, Lautenschlager C, His B (1987) Alzheimer dementia and Pick's disease: neurofibrillary tangles and Pick bodies are associated with identical phosphorylated neurofilament epitopes. Acta Neuropathol (Berl) 73: 240–246

    Google Scholar 

  29. Wisniewski HM, Coblents JM, Terry RD (1972) Pick's disease. A clinical and ultrastructural study. Arch Neurol 25: 97–108

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Yokoo, H., Oyama, T., Hirato, J. et al. A case of Pick's disease with unusual neuronal inclusions. Acta Neuropathol 88, 267–272 (1994). https://doi.org/10.1007/BF00293404

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00293404

Key words

Navigation