Abstract
Sturge-Weber-Crabbe syndrome (SWCS, encephalo-trigeminal angiomatosis) is a rare neurodermal disease (phacomatosis), characterized by vascular skin, eyes and brain abnormalities. The incidence is 1 for 50,000 and it is equal in women and men. All races and ethnic groups are affected without gender differences. The history of the first descriptions of SWCS, its detailing, elements of etiopathogenesis and clinical description, aspects of the medical imaging technologies application in SWCS identifying are presented. As part of a clinical observation the brain CT and MRI results of the 9-year patient with SWCS are described and illustrated in details. It is noted that patients with SWCS should be under the supervision of a multidisciplinary team (medical geneticist, dermatologist, neurologist, epileptologist, neurosurgeon, neuropsychologist, ophthalmologist, ophthalmosurgeon, vascular surgeon, neuroradiologist) with training in the diagnostics and treatment of hereditary neurodermal syndromes . Keywords: Sturge-Weber-Crabbe syndrome, encephalo-trigeminal angiomatosis, phacomatosis.
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