Distribution of Leptomeningeal Glioneuronal Heterotopia in Alobar Holoprosencephaly
- 1 September 1994
- journal article
- research article
- Published by American Medical Association (AMA) in Archives of Neurology
- Vol. 51 (9) , 951-954
- https://doi.org/10.1001/archneur.1994.00540210125022
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.Keywords
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