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Background. Encephalotrigeminal angiomatosis also called Sturge Weber Syndrome (SWS) is neurocutaneous abnormality with angioma affecting the leptomeninges and the face skin, commonly in the eyes and maxillary distribution of trigeminal nerve. The characteristic of SWS is dilated face skin veins, and referred to nevus flammeus and port wine stain. Case reports. We report a case of Sturge Weber Syndrome in a 41 years old male with uncontrolled hypertension and smoking, left-sided weakness, and left-sided facial port wine stain. Conclusions. Neurological examination was suggestive of SWS and further radiological examination confirmed the diagnosis.

Background. Encephalotrigeminal angiomatosis also called Sturge Weber Syndrome (SWS) is neurocutaneous abnormality with angioma affecting the leptomeninges and the face skin, commonly in the eyes and maxillary distribution of trigeminal nerve. The characteristic of SWS is dilated face skin veins, and referred to nevus flammeus and port wine stain. Case reports. We report a case of Sturge Weber Syndrome in a 41 years old male with uncontrolled hypertension and smoking, left-sided weakness, and left-sided facial port wine stain. Conclusions. Neurological examination was suggestive of SWS and further radiological examination confirmed the diagnosis. [ABSTRACT FROM AUTHOR]

Encephalotrigeminal Angiomatosis, also known as Sturge Weber Syndrome (SWS), Dimitri disease, Sturge Kalischer Weber Syndrome is specifically non familial, congenital rare disorder consisting of hamartomatous malformations that may affect eye, skin, and CNS. Failure of proper vascular development is believed to be the most likely cause of the condition. The malformed blood vessels may lead to port wine stains, epilepsy, and glaucoma depending on its location. We are presenting here a triad of case reports on Sturge Weber Syndrome representing individuals belonging to varied age groups.

Encephalotrigeminal angiomatosis of Sturge-Weber-Krabbe-Dimitri is a rare hereditary sporadic phacomatosis, characterized by the presence of angiomatosis of brain lining vessels, face and eye capillaries. We present the case of a 25-year-old girl with a birth mark, a facial angioma localized in the territory of the right ophthalmic nerve, also since she had 4 months, she presents generalized tonic-clonic seizures, for which she is currently under treatment with Levetiracetam 1000 mg/day, Lamotrigine 100 mg/day, Valproic Acid 1500 mg/day and Clonazepam 2 mg/day. She was hospitalized because of the higher incidence of the seizures, despite the treatment that she performs. Physical examination revealed her facial angioma, a facial dysmorphism, gingival hypertrophy with sharp teeth, and she has an upper limb asymmetrical development. The neurological exam reveals central facial paresis, a rough motor deficit on her left side, increased tendon reflexes on the left side, left limb ataxia, and a minor cognitive disorder. The cerebral CT identified cortical asymmetry with right frontal atrophy, gyriform calcifications and frontal hyperostosis. The brain MRI showed hemihypotrophy of the right cerebral hemisphere, especially regarding the frontal lobe, gyriform calcifications on the right side, hypertrophy of the right frontal sinus. We increased the dosage of antiepileptic drugs, to 1500 mg/day of Levetiracetam and 200 mg/day of Lamotrigine. The outcome was favorable, the patient was seizures-free. The particularity of the case is that being a rare disease, it is easily overlooked, although the patient had classic symptoms and imagistic findings for Sturge-Weber-Krabbe disease.

Aim: Sturge-Weber Syndrome, also known as encephalotrigeminal angiomatosis, is an uncommon, nonhereditary developmental anomaly. Methodology: A 48-year-old woman presented for routine dental treatment. She had a history of seizures and had a port wine stain on the right side of her face, which followed the distribution of the trigeminal nerve. Skull radiographs revealed "tram-track" calcifications. Conclusions: The early diagnosis of Sturge-Weber Syndrome requires a multidisciplinary approach. Oral health care professionals need adequate knowledge and understanding of the disease process to help diagnose and treat these patients. [ABSTRACT FROM AUTHOR]

Sturge Weber Syndrome (SWS) or Encephalotrigeminal Angiomatosis is specifically a rare non hereditary congenital sporadic disorder of elusive etiology. It belongs to a group of disorders collectively known as the phakomatoses (motherspot diseases). It has a vast continuum of cutaneous, neurologic, ophthalmic and oral manifestations. It consists of congenital hamartomatous malformations that may affect the eye, skin, and central nervous system (CNS) at different times, characterized by the combination of venous angiomas of leptomeninges, face, jaws and oral soft tissues. The classic pathognomonic features of disease include angioma of the leptomeninges extending to cerebral cortex with ipsilateral angiomatous lesions, unilateral facial nevus after one division of trigeminal nerve and epileptic convulsions. The most characteristic oral manifestation is represented by gingival hemangiomatous lesion usually restricted to ipsilateral maxilla or mandible. We report a case of Sturge Weber Syndrome with its characteristic oral manifestations and review of relevant prevailing literature. Update Dent. Coll. j: 2015; 5 (2): 47-51

Sturge.Weber syndrome (SWS), also known as encephalotrigeminal angiomatosis, is a rare vascular neurocutaneous alteration. It is characterized by facial nevus . usually unilateral, seizures, hemiparesis, intracranial calcification, mental retardation and ocular involvement. Nevertheless each case of SWS is unique and exhibits the characteristic features to a varying degree. We report here a case of SWS who presented with features suggestive of this syndrome, and having bilateral facial nevus as well. [ABSTRACT FROM AUTHOR]

Sturge-Weber syndrome (SWS), sometimes referred to as encephalotrigeminalangiomatosis, is a rare congenital neurological and skin disorder of unknown etiology. The classic pathognomonic features of disease include angioma of the leptomeninges extending to cerebral cortex with ipsilateral angiomatous lesions, unilateral facial nevus affecting the division of trigeminal nerve and epileptic convulsions. The most characteristic oral manifestation is represented by gingival hemangiomatous lesion usually restricted to ipsilateral maxilla or mandible. A classic case of Sturge-Weber syndrome with extended involvement of other parts of the body is reported here. [ABSTRACT FROM AUTHOR]

Sturge-Weber syndrome (SWS) is a sporadic, congenital disorder that impairs the development of the skin, eye and brain. Owing to its multisystem clinical manifestations, a multidisciplinary approach from dermatologists, neurologists and ophthalmologists is required. This article reviews data on pathogenesis, diagnosis and treatment of SWS with a focus on neurologic and dermatologic abnormalities. In terms of diagnosis, MRI, PET and electroencephalogram are discussed. Treatment of seizures includes prophylactic anti-epileptic medications, focal resection and hemispherectomy. Various treatment options for port-wine stains are also discussed, including pulsed-dye laser and new techniques, such as photodynamic therapy and the concurrent use of diagnostic imaging with pulsed-dye laser. Data supporting different theories of pathogenesis are also reviewed. [ABSTRACT FROM AUTHOR]

Background: Sturge-Weber syndrome (encephalotrigeminal angiomatosis) is a rather uncommon congenital condition that is characterized by a combination of venous angioma of leptomeninges over the cerebral cortex and ipsilateral angiomatous lesions of the face and sometimes of the skull, jaws, and oral soft tissues. It is commonly referred to as Sturge-Weber syndrome after Sturge and Weber who first described this affliction in 1879. This article presents a case of Sturge-Weber syndrome associated with severe gingival enlargement, its management, and follow-up results. Methods: A 15-year-old male patient was referred to the Department of Periodontics, Government Dental College and Hospital, for severe gingival enlargement. A detailed dental and medical history, clinical examination, and investigations confirmed the diagnosis of Sturge-Weber syndrome. This report reveals a classic presentation of the syndrome with emphasis on its oral manifestations. Periodontal management included thorough scaling and root planing followed by periodontal flap surgery to treat the gingival enlargement. Histopathologic examination of the excisional biopsy specimen revealed features suggestive of fibrous gingival enlargement. Results: Reevaluation of the patient after 2 years showed remarkable (90%) reduction of the gingival enlargement in the maxillary arch and complete diminution (100%) in the mandibular arch. However, a slight recurrence was noted in the maxillary right quadrant. Conclusions: Sturge-Weber syndrome is clinically important to the periodontist because of its associated gingival vascular features and their complicating manifestations. Periodic systemic and oral examinations are recommended to identify and prevent any complications from the cranial and oral lesions. [ABSTRACT FROM AUTHOR]

Abstract: A 14-year-old patient of Sturge–Weber syndrome who presented with history of intractable seizure for last 10 years and mental retardation without any cerebellar sign or symptoms was evaluated by cranial computed tomography (CT), which showed severe right cerebral and mild ipsilateral cerebellar atrophy with extensive gyral and folial calcification. The involvement of the posterior fossa is extremely rare in cases of Sturge–Weber syndrome and may be found on imaging without cerebeller signs and symptoms. [Copyright &y& Elsevier]