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Late occurrence of additional ocular and intracranial pathologies in the linear naevus sebaceous (Feuerstein-Mims) syndrome

par Brihaye, Jean ;Van Geertruyden, Marthe ;Retif, Jean ;Mercier, A.M.
Référence Acta neurochirurgica, 92, 1-4, page (132-137)
Publication Publié, 1988

Article révisé par les pairs

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Résumé :

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 is 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.