Saturday, 3 September 2011

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

4 comments:

sonoman said...

nice case ....keep the good work

Njenga Mburu said...

Great updates everyday.

allabakash said...

very nice case sir

Anonymous said...

great update..keep it up..sandeep garg