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Acta Neuropathologica

, Volume 61, Issue 1, pp 36–42 | Cite as

Alexander's disease: Further light-, and electron-microscopic observations

  • J. Towfighi
  • R. Young
  • J. Sassani
  • J. Ramer
  • D. S. Horoupian
Original Works

Summary

The neuropathologic and ophthalmopathologic findings in a 53/4-year-old boy with Alexander's disease are reported. Light- and electron-microscopic and immunohistochemical studies revealed that (1) the granular osmiophilic deposits (GOD) in Alexander's disease accumulate mainly in astrocytic processes to form Rosenthal fibers, (2) the Bergmann glia are different in this regard and accumulate the deposits primarily in their perikarya, (3) the Müller cells of retina (which closely resemble astrocytes) do not accumulate GOD, (4) the deposits are also not present in other glial cells and glial-like cells such as pituicytes and pineocytes, (5) the deposits are sparse in the retrobulbar optic nerves, and (6) the peroxidase-antiperoxidase and immunofluorescence studies did not demonstrate glial fibrillary acidic protein (GFAP), albumin, immunoglobulins, or fibrinogen in the astrocytic deposits.

The differential deposition of GOD in various cytoplasmic regions of astrocytes in different areas of central nervous system (CNS) suggests that astrocyte metabolism may not be uniform throughout the brain. Attention to this point may prove helpful in understanding the pathogenesis of the deposits in Alexander's disease. The absence of immunohistochemically demonstrable plasma proteins and GFAP in the astrocytic GOD indicates that the latter have an origin different from plasma proteins and glial filaments. Alternatively, the deposits may be derived from these proteins, but their antigenicity has since been altered.

Key words

Astrocytes Retina Optic nerve 

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References

  1. Alexander WS (1949) Progressive fibrinoid degeneration of fibrillary astrocytes associated with mental retardation in a hydrocephalic infant. Brain 72:373–381Google Scholar
  2. Cole G, DeVilliers F, Proctor NSF, Freiman I, Bill P (1979) Alexander's disease: Case report including histopathological and electron microscopic features. J Neurol Neurosurg Psychiatry 42:619–624Google Scholar
  3. Craft J, Albert DM, Reid TW (1975) Ultrastructural description of a “cylinder organelle” in the outer plexiform layer of human retinas. Invest Ophthalmol 14:923–927Google Scholar
  4. Crome L (1953) Megalencephaly associated with hyaline panneuropathy. Brain 76:215–228Google Scholar
  5. Escourolle R, de Baecque C, Gray F, Baumann N, Hauw J-J (1979) Etude en microscopie electronique et neurochimique d'un cas de maladie d'Alexander. Acta Neuropathol (Berl) 45:133–140Google Scholar
  6. Friede RL (1964) Alexander's disease. Arch Neurol 11:414–422Google Scholar
  7. Garret R, Ames RP (1974) Alexander disease. Arch Pathol 98: 379–385Google Scholar
  8. Herndon RM, Rubinstein LJ, Freeman JM, Mathieson G (1970) Light and electron microscopic observation on Rosenthal fibers in Alexander's disease and in multiple sclerosis. J Neuropathol Exp Neurol 29:524–551Google Scholar
  9. Hogan MI, Alvarado JA, Weddell JE (1971) Histology of the human eye. Saunders, PhiladelphiaGoogle Scholar
  10. Holland IM, Kendall BE (1980) Computed tomography in Alexander's disease. Neuroradiology 20:103–106Google Scholar
  11. Horoupian DS, Kress Y, Yen S-H, Gaskin F (1982) Nickel-induced changes and reappraisal of Rosenthal fibers in focal CNS lesions. J Neuropathol Exp Neurol 41:664–675Google Scholar
  12. Peiffer J (1968) Alexander's disease — Really a leukodystrophy? Pathol Eur 3:305–312Google Scholar
  13. Rankin WE, Hart MN, Weisenburger DD (1977) Thrombotic thrombocytopenic purpura in a child with Alexander's disease. Arch Pathol 101:655–657Google Scholar
  14. Russo LS, Aron A, Anderson PJ (1976) Alexander's disease: A report and reappraisal. Neurology (Minneap) 26:607–614Google Scholar
  15. Schochet SS Jr, Lampert PW, Earle KM (1968) Alexander's disease: A case report with electron microscopic observations. Neurology (Minneap) 18:543–549Google Scholar
  16. Seil FJ, Schochet SS, Jr, Earle KM (1968) Alexander's disease in an adult: Report of a case. Arch Neurol 19:494–502Google Scholar
  17. Soffer D, Horoupian DS (1979) Rosenthal fibers formation in the central nervous system, its relation to Alexander's disease. Acta Neuropathol (Berl) 47:81–84Google Scholar
  18. Sternberger LA (1979) Immunocytochemistry. Wiley, New YorkGoogle Scholar
  19. Stevenson LD, Vogel FS (1952) A case of macrocephaly associated with feeblemindedness and encephalopathy with peculiar deposits throughout the brain and spinal cord. Ciencia (Mex) 12:71–74Google Scholar
  20. Vogel FS, Hallervorden J (1962) Leukodystrophy with diffuse Rosenthal fiber formation. Acta Neuropathol (Berl) 2:126–143Google Scholar
  21. Wohlwill FJ, Bernstein J, Yakovlev PI (1959) Dysmyelinogenic leukodystrophy: Report of a case of a new presumably familial type of leukodystrophy with megalobarencephaly. J Neuropathol Exp Neurol 18:359–383Google Scholar

Copyright information

© Springer-Verlag 1983

Authors and Affiliations

  • J. Towfighi
    • 1
  • R. Young
    • 2
  • J. Sassani
    • 1
  • J. Ramer
    • 2
  • D. S. Horoupian
    • 3
  1. 1.Dept. of PathologyThe M.S. Hershey Medical College of the Pennsylvania State UniversityHersheyUSA
  2. 2.Dept of PediatricsThe M.S. Hershey Medical College of the Pennsylvania State UniversityHersheyUSA
  3. 3.Dept. of PathologyAlbert Einstein College of Medicine of Yeshiva UniversityBronxUSA

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