Progressive Sclerosing Cortical Atrophy: Spongy Glio-Neuronal Dystrophy

  • Reinhard L. Friede


The etiologic definition of the disease, or group of diseases, described in this chapter is controversial. Pertinent cases are reported under the names of progressive sclerosing cortical atrophy, progressive cerebral poliodystrophy, “Alpers’ disease”, or spongy glioneuronal dystrophy, or as hemiatrophy if there is lateralization of the lesions. No satisfactory subclassification can be arrived at on the basis of morphologic examination alone, and there is no generally accepted definition of clear-cut nosologic entities. Much of the pathogenetic discussion has centered on the significance of convulsions: The cerebral lesions may be considered postictal damage, or the convulsions may be a nonobligatory symptom of an acquired or an inborn progressive destructive disease process which affects mainly the cerebral gray matter. A combined description of the histopathology of these lesions is given in the present chapter, including the processes known as hemiatrophy.


Status Epilepticus Cortical Atrophy Unilateral Lesion Glial Tissue Convulsive Disorder 
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Copyright information

© Springer-Verlag Wien 1975

Authors and Affiliations

  • Reinhard L. Friede
    • 1
    • 2
  1. 1.Case Western Reserve UniversityClevelandUSA
  2. 2.University of ZurichSwitzerland

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