Distribution of Leptomeningeal Glioneuronal Heterotopia in Alobar Holoprosencephaly

Abstract
Objective: To clarify the pathogenetic significance of the topographic distribution of leptomeningeal glioneuronal heterotopia, a common finding in holoprosencephaly. Design: Gross, histological, and immunohistochemical observations of alobar holoprosencephaly in brain specimens taken at autopsy. Setting: Referral center. Materials: Brains removed at autopsy from five consecutive patients with alobar holoprosencephaly. Main Outcome Measures: Immunoperoxidase staining for glial fibrillary acidic protein. Results: In all brains, leptomeningeal glioneuronal heterotopia showed an identical distribution, ranging from the basal prosencephalon to the pons, with the thickest distribution occurring in the basal prosencephalon. Conclusion: The constant localization implicates leptomeningeal glioneuronal heterotopia in severe dysgenesis of midline prosencephalon, the basic pathogenesis of the anomaly.

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