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Porencephaly, Hydranencephaly, Multicystic Encephalopathy

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Developmental Neuropathology

Abstract

This chapter concerns a group of cavitary lesions of the full thickness of the hemispheric wall representing the endstages of destruction of cerebral tissue during early development. There is inconsistency in the naming of these lesions. In neuroradiology, it is customary to use the term porencephaly indiscriminately for all large cavities in the brains of infants. Neuropathologic features allow a more detailed classification: hydranencephaly, basket brains and porencephaly are described here as equivalent lesions differing only in extent. Schizencephaly (Yakovlev and Wadsworth 1946a, b) is not considered a separate entity, nor are the defects of septum pellucidum associated with porencephaly (Aicardi and Goutières 1981). All of these lesions typically occur in the fetus. If massive necrotizing lesions develop perinatally or postnatally, their features are somewhat different, as described later in this chapter in the section on global hemispheric necrosis. Multicystic encephalopathy can also be differentiated from porencephaly. This chapter does not include the cavitary residual defects of periventricular infarcts or periventricular hemorrhages in newborns, which are described in Chaps. 4 and 6.

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  1. Zentrum Neurologische Medizin, Abteilung Neuropathologie, Georg-August-Universität Göttingen, Robert-Koch-Straße 40, 3400, Göttingen, Germany

    Reinhard L. Friede

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© 1989 Springer-Verlag Berlin Heidelberg

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Friede, R.L. (1989). Porencephaly, Hydranencephaly, Multicystic Encephalopathy. In: Developmental Neuropathology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-73697-1_3

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  • DOI: https://doi.org/10.1007/978-3-642-73697-1_3

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