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.