STURGE WEBER SYNDROME

a congenital neurocutaneous disease marked by a port-wine-colored capillary hemangioma over a sensory dermatome of a branch of the trigeminal nerve of the face.
Radiographic examination of the skull reveals intracranial calcification. The cerebral cortex may atrophy, and generalized or focal seizures, angioma of the choroid, secondary glaucoma, optic atrophy, and new cutaneous hemangiomas may develop. There is no known cure. Treatment is supportive and includes anticonvulsive medication.
Sturge-Weber Syndrome (SWS) or also called encephalotrigeminal angiomatosis is a congenital, non-familial disorder of unknown incidence and cause, which is characterized by a congenital port-wine nevus (facial birthmark) leptomenigeal angiomatosis, and glaucoma; it is commonly complicated by epilepsy and hemiparesis. Other symptoms associated with SWS can include eye and internal organ irregularities. Each case of SWS is unique and exhibits the characterizing findings to varying degrees.
The most apparent indication of SWS is a facial birthmark or “Port Wine Stain” (PWS) present at birth and typically involving at least one upper eyelid and the forehead. Much variation in the size of the stain has been reported and may be limited to one side of the face or may involve both sides. The stain, varying from light pink to deep purple, is due to an overabundance of capillaries just beneath the surface of the involved skin. In persons with dark pigmentation, the stain may be difficult to recognize. In rare instances, there is an absence of a PWS. Atypical presentations such as: intracranial venous anomalies, soft tissue hypertrophy, phakomatosis pigmentovascularis, overlapped Klippel-Trenaunay syndrome (cutaneous hemangiomas, venous varicosities and soft tissue or bone hypertrophy of the affected extremities), headache and epilepsy, and an acute life-threatening event have been reported to the medical literature^.
SWS is referred to as complete when both CNS and facial angiomas are present and incomplete when only one area is affected without the other.
The Roach Scale is used for classification, as follows:  
Type I: This is the most common presentation with facial and leptomeningeal angiomas. Glaucoma may be present. Ocular involvement is normally noted within the first year of life. The sclera may appear “bloodshot” as a result of the over-proliferation of blood vessels on the eye. In rare cases, the facial and brain involvement are bilateral (involving both sides of the head). Mental and physical development can be impaired to varying degrees, depending on the degree of vascular malformation throughout the brain and eye. 
Type 2: This type involves a facial angioma and the possibility of glaucoma, but no evidence of intracranial disease. There is no specific time-frame for the exhibition of symptoms beyond the initial recognition of the facial PWS. Throughout the life of the individual, interrelated symptoms may manifest in glaucoma, cerebral blood flow abnormalities, headaches, and various other complications. Additional research needs to be conducted on this type of SWS to determine the course of the syndrome over its natural progression. 
Type 3: This type of SWS is commonly noted to have a leptomeningeal angioma, with no facial involvement and usually no development of glaucoma. Commonly referred to as forme fruste, this type is identified with brain scans. It can also be confused with other diagnoses prior to a brain scan with contrasting agent.
While social stigma is lessened by the absence of PWS, the unknown natural course of the syndrome is still frustrating for parents and professionals treating the condition.