The Sturge‐Weber Syndrome

Abstract

The Sturge-Weber syndrome is characterized by angiomas of the leptomeninges overlying the cerebral cortex in association with a facial nevus flammeus. Although frequently included with other neurocutaneous genodermatoses, the syndrome is almost always sporadic in occurrence. The most common associated neurologic abnormality is seizures, which are controlled in more than 50% of patients by the administration of anticonvulsants. Laser therapy is the most promising therapeutic option for cosmetic management of the facial nevus flammeus.

Management of Patient with Sturge-Weber Syndrome: a Case Report

“…Sturge-Weber syndrome is a rare disease in the group of phakomatoses that cause physical, psycho-logical, and social disorders. This syndrome occurs with equal frequency in both sexes, with seizures typically developing in the first year of life 5,6 . This is a case report of a young patient who has type one of Sturge-Weber Syndrome according to the Roach Scale classification.…”

Sturge Weber Syndrome- A Rare Congenital Neuro-cutaneous Disease

“…Macroglossia and maxillary bone hypertrophy found in some patients can cause malocclusion and facial asymmetry. The gingival hyperplasia in these patients could be secondary to anticonvulsant therapy further complicated by poor oral hygiene secondary to mental retardation 2,3,14 .…”

Sturge-Weber syndrome diagnosed in a 45-year-old man

“…4 Only port-wine stains in the V1 distribution have been shown to be associated with leptomeningeal angiomatosis, which defines Sturge-Weber syndrome. 1 Enjolras and associates 5 reviewed 42 cases of portwine stains in the V1 distribution and found that patients were at increased risk when the entire V1 distribution was covered, with 11 (44%) of the 25 such patients having SturgeWeber syndrome. Seventeen patients had part of the V1 distribution covered by a port-wine nevus; only 1 was found to have the syndrome.…”

“…Glaucoma occurs in 30% of patients with Sturge-Weber syndrome, and it develops before 2 years of age in 60% of these patients. 1 People of any age with port-wine stains in the V1 distribution may have Sturge-Weber syndrome and be at risk of seizures and neurological complications. They require yearly eye examinations and measurements of intraocular pressures, regardless of whether they have had symptoms in childhood.…”

“…1 Seizures are controlled with antiepileptic medications in 40% of affected patients. 3 Cortical resection of the affected brain areas is sometimes advocated in cases of seizures uncontrollable by medication, but some favour surgery before permanent neurological deficits can develop.…”