Linear naevus sebaceus
LINEAR NAEVUS SEBACEUS (Feuerstein's syndrome)
A sebaceous naevus with yellow nodules over the face or scalp was recognized in the late nineteenth century by Jadassohn. Later the triad of a linear midline sebaceous naevus with mental retardation and seizures was recognized by Feuerstein & Mims (1962). Further cases have since been reported of this combination, which seems to represent a distinct neurocutaneous syndrome affecting both sexes equally and without a familial incidence. Seizures, which may begin in the newborn period, usually under one year of age, are of various kinds, including grand mal, Jacksonian attacks and infantile spasms (Lovejoy & Boyle 1973). A child with the triad of naevus sebaceus, retardation and salaam seizures with the additional features of a staphyloma of the eye, an enlarged clitoris and a cardiac arrhythmia was reported by Tripp (1971), who suggested that this expanded syndrome should be called `the skin, eye, brain and heart syndrome'. Hemimacrocephaly was present at birth in an infant delivered by Caesarian section because of cephalopelvic disproportion who developed seizures on the fourth day of life and had an extensive midline naevus sebaceus (Bolthauser & Navratil 1978).
Two Polish cases were reported by Zaremba (1978), one of them with neuropathological details; gyri were enlarged, cortical stratification was abnormal, and some ectopic pyramidal cells were seen in the white matter, evidence of aberrant development. In a review of 37 cases (Zaremba et al 1978), the site of the naevus, in decreasing order of frequency, was: face; hairy scalp and cheek (equal); extremities; forehead and neck (equal); nose; trunk; chin. The naevus spared the face and head in only four cases.
