title: Late occurrence of additional ocular and intracranial pathologies in the linear naevus sebaceous (Feuerstein-Mims) syndrome.
abstract: The linear naevus sebaceous syndrome of Feuerstein-Mims belongs to the phacomatoses and classically is composed of a linear cutaneous lesion associated with mental retardation and epilepsy, sometimes also with ocular dystrophies. During an unusually long follow-up of 32 years of such a case, which is reported here, the late occurrence of additional ocular and intracranial pathology are demonstrated: conjunctival and bulbar tumours with progressive deterioration of visual acuity, bilateral intracranial arachnoidal cysts, dilatation and tortuosity of the middle cerebral artery. As a consequence of these observations the prognosis in cases with this syndrome has to be given with special caution.
authors.affiliation: Department of Neurosurgery, Université Libre de Bruxelles, Belgium.
authors.forename: J
authors.initials: J
authors.lastname: Brihaye
authors.forename: M
authors.initials: M
authors.lastname: Brihaye-van Geertruyden
authors.forename: J
authors.initials: J
authors.lastname: Retif
authors.forename: A M
authors.initials: AM
authors.lastname: Mercier
mesh_terms: D000328:Adult; D004416:Dysplastic Nevus Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D005128:Eye Diseases / Q000000981:diagnostic imaging / Q000209:etiology*; D006801:Humans; D014057:Tomography, X-Ray Computed