Acta Neuropathologica

, Volume 33, Issue 1, pp 1–8 | Cite as

Symmetrical infantile thalamic degeneration with focal cytoplasmic calcification

  • Mary Ambler
  • William O'Neil
Original Investigations


Infantile thalamic degeneration is a rare clinico-pathological entity. Restricted location of the lesion and peculiar cytopathological changes serve to distinguish this disorder from other common encephalopathies.

Optical and ultrastructural studies demonstrate cytoplasmic calcopherules in previously viable cells.

According to current concepts of acute cellular reactions to injury and mechanism of intracellular calcification, the cytological changes cannot be attributed to either hypoxic ischemic cell change or dystrophic calcification.

By analogy to other human and pathological material, the most likely basis for nondystrophic calcopherule formation is toxic or infectious injury with local synthesis, or autophagic or phagolysosomal degradation of cellular debris of specific chemical composition favoring calcium deposition.

Key words

Thalamus Cytoplasmic calcospherules Non-dystrophic Electron microscopy 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Bonucci, E., Sadun, R.: An electron microscope study on experimental calcification of skeletal muscle. Clin. Orthop.88, 197–217 (1972)Google Scholar
  2. Bonucci, E., Sadun, R.: Experimental calcification of the myocardium. Amer. J. Path.71, 167–192 (1973)Google Scholar
  3. Brown, A. W., Brierley, J. B.: Anoxic-ischemic cell change in rat brain: Light microscopic and fine structural observations. J. neurol. Sci.23, 9–22 (1972)Google Scholar
  4. Chou, S. M., Fukuhara, N.: EM studies on calcospherites induced in cerebellar granular layers of rats by chronic methyl mercury poisoning. J. Neuropath. exp. Neurol.32, 175–176 (1973)Google Scholar
  5. DeGirolami, U., Hass, M. L., Richardson, E. P.: Subacute diencephalic angioencephalopathy. J. neurol. Sci.22, 197–210 (1974)Google Scholar
  6. Field, E. J., Raine, C. S., Joyce, G.: Scrapie in the rat: An electron microscope study. Acta neuropath. (Berl.)8, 47–56 (1967)Google Scholar
  7. Ghatak, N. R., Zimmerman, H. R.: Fine structure of toxoplasma in the human brain. Arch. Path.95, 276–283 (1973)Google Scholar
  8. Gilles, F. H., Deuel, R. K.: Neuronal cytoplasmic globules in the brain in Morquio's syndrome. Arch. Neurol. (Chic.)25, 393–403 (1971)Google Scholar
  9. Gritzka, T. L., Trump, B. F.: Renal tubular lesions caused by mercuric chloride. Amer. J. Path.52, 1225–1277 (1968)Google Scholar
  10. Iizuka, R., Spalhe, G.: Giant cell pseudocalcium necrosis in the early stages of cortical necrosis due to arteriosclerotic inclusion. Acta neuropath. (Berl.)23, 313–319 (1973)Google Scholar
  11. Lemieux, L. H.: The thalamic pathology of amaurotic family idiocy. J. Neuropath. exp. Neurol.13, 343–352 (1954)Google Scholar
  12. Lou, T. Y., Teplitz, C.: Malakoplakia: Pathogenesis and ultrastructural morphogenesis. Human Path.5, 191–207 (1974)Google Scholar
  13. Mergner, W. J., Garbus, J., Dees, J. H., Valigorsky, J. M., Trump, B. F.: Structural and functional changes in human kidney and liver mitochondria acute cell injury after shock and trauma. Amer. J. Path.66, 36a (1972)Google Scholar
  14. Richardson, E. P.: In discussion of symmetrical thamalic degeneration in infants. J. Neuropath. exp. Neurol.21, 300–301 (1962)Google Scholar
  15. Rosales, R. K., Riggs, H. A.: Symmetrical thalamic degeneration in infants. J. Neuropath. exp. Neurol.21, 371–376 (1962)Google Scholar
  16. Schulman, S.: Bilateral symmetrical degeneration of the thalamus. J. Neuropath. exp. Neurol.16, 446 (1957)Google Scholar
  17. Stern, K.: Severe dementia associated with bilateral symmetrical degeneration of the thalamus. Brain62, 157–171 (1939)Google Scholar
  18. Trump, B. F., Valigorsky, J. M., Dees, J. H., Mergner, W. J., Kim, K. M., Jones, R. T.: Pendergrass, R. E., Garbus, J., Cowley, R. A.: Cellular changes in human disease. Human Path.4, 89–109 (1973)Google Scholar

Copyright information

© Springer-Verlag 1975

Authors and Affiliations

  • Mary Ambler
    • 1
  • William O'Neil
    • 1
  1. 1.Department of Pathology (Neuropathology) and PediatricsRhode Island HospitalProvidenceUSA

Personalised recommendations