Acta Neuropathologica

, Volume 48, Issue 1, pp 59–61 | Cite as

Apert's syndrome with central nervous system anomalies

  • J. A. Maksem
  • U. Roessmann
Short Original Communications


The pathologic anatomy of the central nervous system is described in a patient with Apert's syndrome. Multiple developmental anomalies of the brain were noted including disturbances of rhinencephalic organization. The association between maldevelopment of the rhinencephalon and the face is well known. Cranial vault malformations may also be associated with maldevelopment of the rhinencephalon.

Key words

Apert's syndrome Craniostenosis Craniosynostosis Rhinencephalon 


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  1. Brun, A.: Marginal glioneural heterotopias of the central nervous system. Acta Pathol. Microbiol. Scand.65, 221–233 (1965)Google Scholar
  2. Cohen, M. M.: An etiologic and nosologic overview of craniosynostosis syndromes. Birth Defects11, 137–189 (1975)Google Scholar
  3. De Meyer, W., Zeman, W., Palmer, C. G.: The face predicts the brain: Diagnostic significance of facial anomalies for holoprosencephaly (arhinencephaly). Pediatrics34, 256–263 (1964)Google Scholar
  4. Fishman, M. A., Hogan, G. R., Dodge, P. R.: The concurrence of hydrocephalus and craniosynostosis. J. Neurosurg.34, 621–629 (1971)Google Scholar
  5. Hogan, G. R., Baumann, M. L.: Hydrocephalus in Apert's syndrome. J Pediatr.79, 782–787 (1971)Google Scholar
  6. Laroche, J. D.: Developmental pathology of the neonate, p. 482. Amsterdam: Excerpta Medica 1977Google Scholar

Copyright information

© Springer-Verlag 1979

Authors and Affiliations

  • J. A. Maksem
    • 1
  • U. Roessmann
    • 1
  1. 1.Institute of PathologyCase Western Reserve UniversityClevelandUSA

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