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Neurocutaneous Disorders

Sturge-Weber Syndrome
»	Name the two essential components of the Sturge-Weber syndrome.
»	What is the inheritance of the Sturge-Weber syndrome?
»	Where does the port wine stain most commonly occur in Sturge-Weber syndrome?
»	What are the complications of leptomeningeal angiomatosis?
»	When does epilepsy usually begin in the Sturge-Weber syndrome?
»	Can the extent of neurologic involvement be predicted from the size of the facial port wine stain?
»	What investigations confirm leptomeningeal angiomatosis?
»	Do ocular complications occur in the Sturge-Weber syndrome?
»	How can the facial port wine stain be treated?
»	In what other neurocutaneous disorders do facial or occipitocervical port wine stains occur?
»	What is the inheritance of ataxia-telangiectasia?
»	What is the earliest clinical sign of ataxia-telangiectasia?
»	Name the typical skin sign of ataxia-telangiectasia.
»	What are the two most common causes of death in ataxia-telangiectasia?

How can the facial port wine stain be treated?

The pulsed dye laser (wavelength 5 585/595 nm) is the most effective treatment modality. Regular laser treatments may result in significant fading and may also help prevent the soft tissue hypertrophy that gradually develops with many port wine stains.