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The ultrastructure of Sturge-Weber disease

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Summary

Five infantile and one adult case of Sturge-Weber disease were studied pathologically. The calcification occurring under the leptomeningeal angiomatosis increased with advancing age. Light and electron microscopy of two cases showed the smallest, and therefore possibly the earliest, calcifications occurred in perithelial cells. It is hypothesized the cause of calcification is anoxic injury to endothelial, perithelial and possibly glial mitochondria due to stasis and abnormal vessel permeability in the cerebral vessels composing the Sturge-Weber angioma.

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Authors and Affiliations

  1. Eleanor M. Paterson Department of Laboratory Medicine and Research, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada

    Margaret G. Norman

  2. Department of Pathology, University of Ottawa, Ottawa, Ontario, Canada

    Margaret G. Norman

  3. Department of Pathology, Peter Bent Brigham Hospital and Harvard Medical School, Boston, Massachusetts, USA

    William C. Schoene

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  1. Margaret G. Norman
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  2. William C. Schoene
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Norman, M.G., Schoene, W.C. The ultrastructure of Sturge-Weber disease. Acta Neuropathol 37, 199–205 (1977). https://doi.org/10.1007/BF00686879

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  • DOI: https://doi.org/10.1007/BF00686879

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