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Linear nevus sebaceous syndrome associated with porencephaly and nonfunctioning major cerebral venous sinuses

Departments of Pediatrics, Radiology, Neurology and Neurosurgery (Neurology), Washington University School of Medicine, and Division of Pediatric Neurology, St. Louis Children's Hospital (Drs. Chalhub and Volpe), and the Neuroradiology Section, Mallinckrodt Institute of Radiology (Dr. Gado), Washington University School of Medicine, St. Louis.

An infant with the linear nevus sebaceous syndrome also had new findings of porencephaly and nonfunctioning major cerebral venous sinuses. This infant is the first described with the syndrome to undergo angiographic study. Similar nonfunctioning major cerebral venous sinuses are seen in the Sturge-Weber syndrome. A leptomeningeal angioma recently was reported in a newborn with the linear nevus sebaceous syndrome. The central nervous system disturbances in this syndrome might be secondary to developmental vascular anomalies. Therefore, the linear nevus sebaceous syndrome exhibits important similarities to the Sturge-Weber variety of neurocutaneous syndrome.

This study was supported in part by USPHS Grant NS-05633 and the Allen P. and Josephine B. Green Foundation, Mexico, Missouri.

Received for publication February 6, 1975.

Dr. Chalhub's address is Department of Pediatrics and Neurology, Washington University School of Medicine, St. Louis Children's Hospital, St. Louis, MO 63105.

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