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Neuropediatrics 1973; 4(3): 338-343
DOI: 10.1055/s-0028-1091750
Case report

© 1973 by Thieme Medical Publishers, Inc.

CNS Developmental Anomaly in the Basal Cell Nevus Syndrome: Another Congenital Neurocutaneous Syndrome?

Stephen D. Codish, Jon Kraszeski, Kenneth Pratt
  • New York State Kidney Disease Institute and Department of Neurology, the Albany Medical College, Albany, New York
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Publication History

1973

1973

Publication Date:
18 November 2008 (online)

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Abstract

Cerebellar atrophy was discovered in a patient with multiple ectodermal anomalies characteristic of the basal cell nevus syndrome (BCNS). This underscores the neurocutaneous of the syndrome, the hallmark of which is multiple basal cell carcinomas. It also suggets that the frequency of structural underdevelopment in the CNS of patients afflicted with the BCNS may be higher than has been reported previously.

Keyword

Phacomatoses - basal cell nevus syndrome - congenital malformation - immunoglobulin D cerebellum - autosomal dominant