Observations on the Sturge-Weber syndrome

Abstract

In this Thesis some original observations concerning the Sturge Weber syndrome will be placed on record, and new deductions therefrom and from the literature will be submitted.

A survey of the literature reveals that many cases described as examples of the Sturge Weber syndrome can hardly be accepted as such; some are cases of intracerebral racemose angioma, some of the patients have a facial naevus but scanty neurological symptoms or deficits are recorded; a few lack the facial cutaneous stigma. A rather rigid definition of the Sturge Weber syndrome seems therefore justifiable and desirable.

The Sturge Weber syndrome is characterised by cutaneous angiomatosis (capillary naevus, naevus flammeus) affecting the face, epilepsy, gyriform calcifications within the cerebral cortex in relation to an angiomatosis at leptomeningeal level, and in many cases, buphthalmos or glaucoma. The intracerebral calcifications are not visible radiologically at birth. When the lower part of the face is included in the naevoid area the soft tissues of the face, notably the lips and the buccal and alveolar mucosa, are often hypertrophied.