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A Neurocutaneous Syndrome Associated With Infantile Spasms

Bernadette A. Herbst, MD; Michael E. Cohen, MD

Arch Neurol. 1971;24(4):317-322.

Abstract
A fourth case of a new neurocutaneous syndrome occurred in a 51/2-month-old child who presented with skin lesions in a characteristic midline distribution. Histologically, findings were consistent with the diagnosis of nervus sebaceus of Jadassohn. There was also sudden and almost simultaneous onset of psychomotor deterioration with onset of seizures three weeks prior to admission. The electroencephalogram revealed a typical hypsarrythmic pattern, a feature not previously reported. Relation of the skin lesion along the midline of the nose to underlying defects of the brain, as well as to the time in fetal development when certain and as yet unknown factors exert their influence on the developing ectoderm, are discussed.(24:317-322,1971)

Key Words.—
Neurocutaneous syndrome; infantile spasms; linea nevus sebaceus; retardation.

Author Affiliations
Buffalo

From the Department of Pediatric Neurology, Children's Hospital of Buffalo, Buffalo.

Footnotes
Accepted for publication Sept 2,1970.

Reprint requests to Department of Pediatric Neurology, Children's Hospital of Buffalo, 219 Bryant St, Buffalo 14222 (Dr. Cohen).

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