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Review Article: Holoprosencephaly and Related Midline Cerebral Anomalies: A Review

Neuropsychiatric Institute, Division of Neuropathology, the Department of Neurosciences, the University of North Dakota School of Medicine, Fargo, ND

Robert M. Shuman, MD

Department of Neurology, the University of Kentucky, Lexington, KY

We propose a simple pathogenetic mechanism that reduces a bewildering variety of central nervous system malformations to a manageable group sharing defects of midline prosencephalic growth. It is neither new nor innovative, but attempts to summarize many pathologic entities within a concept that accounts for known embryologic events and the sequence and timing of those events. We propose midline prosencephalic dysgenesis as a category of malformations including aprosencephaly, holoprosencephaly, septo-optic dysplasia, and agenesis of the corpus callosum. (J Child Neurol 1986; 1:3-18)

Journal of Child Neurology, Vol. 1, No. 1, 3-18 (1986)
DOI: 10.1177/088307388600100102


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