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September 1968

Chronic Decerebrate State in Infancy: Neurologic Observations in Long Surviving Cases of Hydranencephaly

Author Affiliations

Birmingham, Ala; Columbus, Ohio; Chapel Hill, NC
From the Division of Neurology, Department of Medicine, and Department of Pediatrics, University of North Carolina School of Medicine, Chapel Hill, NC. Dr. Halsey is now at the University of Alabama Medical Center, Birmingham, Ala, and Dr. Allen is now at the Department of Medicine, Ohio State University, Columbus, Ohio.

Arch Neurol. 1968;19(3):339-346. doi:10.1001/archneur.1968.00480030117014

THE TERM hydranencephaly designates the condition in which large portions of the cerebral hemispheres have been reduced to membranous sacs within a cranium of relatively normal size. It may be suspected in the newborn by cranial transillumination, which generally indicates a cerebral thickness of less than 1 cm.1,2

Although this finding implies that such infants have varying levels of decerebration or decortication in a state sometimes permitting long survival, little attention has been paid to the clinical-physiologic features of hydranencephaly and related disorders with severe bilateral cerebral destruction or malformation. Despite many studies of the behavior of the normal newborn3-9 and also of the variable sequences of changes in common spastic diplegia10-12 little can be found concerning the neuroanatomic basis of such common neonatal activity as the Moro and the stepping reflexes. Moreover there are discrepancies between these studies and those in the experimental neurophysiologic literature on

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