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Sturge Weber Syndrome. - radiologystar

Sturge Weber Syndrome.

Case study of sturge weber syndrome.

 

 

 

CT :- The axial CT soft tissue and bone window of head CT well defined unilateral right side cortical calcification and cerebral atrophy, subtle prominence of ipsilateral choroid plexus and subtle enlargement of calvary skull.

 

Diagnosis:- Sturge Weber syndrome

 

 What are the Sturge-Weber syndrome?

Sturge-Weber syndrome is a rare, neurological disorder present at birth and characterized by a port-wine stain birthmark on the forehead and upper eyelid on one side of the face. The birthmark can vary in color from light pink to deep purple and is caused by an overabundance of capillaries (small blood vessels) around the trigeminal nerve just beneath the surface of the face.

 

Sturge-Weber syndrome is also accompanied by abnormal blood vessels on the brain’s surface and the loss of tissue (atrophy) with deposits of calcium (calcification) in the cerebral cortex of the brain on the same side as the birthmark. Sturge-Weber syndrome rarely affects other body organs.

 

Neurological symptoms may include:

 

–Seizures that begin in infancy and may worsen with age. Convulsions usually happen on the side of the body opposite the birthmark and vary in severity.

 

— Intermittent or permanent muscle weakness.

 

— Developmental delays and cognitive impairment.

 

— Glaucoma (increased pressure within the eye) at birth or late.

 

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