Late occurrence of additional ocular and intracranial pathologies in the linear naevus sebaceous (Feuerstein-Mims) syndrome.
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.
Brihaye
J
J
Department of Neurosurgery, Université Libre de Bruxelles, Belgium.
Brihaye-van Geertruyden
M
M
Retif
J
J
Mercier
A M
AM
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