THE LINEAR NEVUS SEBACEUS SYNDROME

The linear naevus sebaceus syndrome (LNSS) is a phakomatosis, characte rized in general by a triad consisting of naevus sebaceus of Jadassohn , seizures, and mental retardation. In addition, a broad spectrum of n eurological, ophthalmological, skeletal, urogenital and cardiovascular symptoms may be encountered. According to our literature review, seiz ures and mental retardation were reported in 67 and 61% of cases, resp ectively. Because ophthalmological abnormalities (59%) and involvement of other organ systems (61%) occur frequently, we advise avoidance of adhering to the classical triad for recognizing or describing LNSS. G ross structural abnormality of the cerebrum or cranium was frequently observed (72%), consisting mainly of enlargement of one lateral ventri cle, hemimegalencephaly and hemimegacranium. We report a case of a mal e patient with the clinical features of LNSS, but without cerebral dev elopmental abnormalities at autopsy examination. (C) 1998 Elsevier Sci ence B.V. All rights reserved.