Sturge Weber Syndrome

A 15 yo male for epilepsy screening.

Imaging finding on CT brain are very typical of Sturge Weber syndrome.

Unilateral involvement,

Left side cortical atrophy,

Dense gyriform calcification with,

Ipsilateral  mild choroid plexus enlargement,

Ipsilateral bony calvarial thickening, enlargement of para nasal sinuses including left side frontal, sphenoid and mastoid air cells. 

SWS is an encephalo trigeminal angiomatosis, a rare congenital neurological and skin disorder, one of the phakomatoses. Unlike other neurocutaneous disorders (phakomatoses),  Sturge-Weber occurs sporadically without hereditary aetiology.

Characterised by port-wine stains of the face and ipsilateral lepto meningeal angioma.

Port-wine stain as birthmark on the forehead light pink to deep purple in colour, caused by abundance of capillaries around the ophthalmic branch of the trigeminal nerve.

Symptomatic with seizures from birth, mental retardation, glaucoma.

Related posts:

Sturge-weber-syndrome Case 1

Sturge-weber-syndrome Case 2

Mimics-of-sturge-weber-syndrome