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

Sturge Weber Syndrome CT Brain

Clinical details: A 14 y o male with mental retardation, seizures and a pink patch on left side fore head.

This non contrast CT brain shows:
Left hemiatrophy.
Dense left temporo parietal cortical gyriform calcification.
Poor development of ipsilateral hemicranium, thickening of bony calvarium.
Enlargement of ipsilateral choroid plexus.
Hyperpneumatisation of left frontal sinus.

Clinical and imaging findings are very typical of Sturge Weber syndrome.

Related posts:
Sturge-weber-syndrome Case 1
Sturge-weber-syndrome Case 3
Mimics-of-sturge-weber-syndrome

Sturge Weber syndrome Typical Imaging Findings

A 35 y o female brought unconscious with history of seizures since birth and mental retardation.

Clinically port wine stain on forehead, imaging finding on CT and MRI study of brain are very typical of Sturge Weber syndrome.

Non contrast CT Brain

MRI T2*GRE and T2w images

Typical imaging findings of SWS
Unilateral involvement.

Hemi cortical atrophy.

Dense tram track line gyriform calcification with leptomeningeal enhancement.

Ipsilateral choroid plexus enlargement.

Ipsilateral bony calvarial thickening, enlargement of para nasal sinuses and mastoid air cells seen in adults. 

Sturge Weber syndrome

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:
Mimics-of-sturge-weber-syndrome
Sturge-weber-syndrome Case 2
Sturge-weber-syndrome Case 3