Sturge-Weber syndrome presenting with intractable seizures

Roka, Yam Bahadur; Puri, PR; Ahmad, WS; Bista, BK

doi:10.3126/joim.v32i1.4002

Cited by 1 publication

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“…5,6 It is a nonhereditary developmental condition characterized by a congenital capillary malformation and hamartomatous vascular proliferation involving the face (portwine stain or facial birthmark), skull and the tissues of brain, oral soft tissue as well as alveolar bone hypertrophy and rarely other body organs. [1][2][3]7 Congenital glaucoma, and cortical calcification (tram-tracks) may also be seen in this syndrome. [8][9][10] It is complicated when associated with other clinical features like neurologic deficits, including seizures, contralateral hemiparesis, hemiatrophy, headache, transient stroke and behavioral problems.…”

Section: Introduction Hementioning

confidence: 99%

Periodontal Manifestations and Unusual Radiographic Features in a Patient with Sturge-Weber Syndrome: A Case Report

Chitsazi

Shirmohammadi

Rahmanpour

et al. 2018

J Periodontol Implant Dent

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The Sturge-Weber syndrome or encephalotrigeminal angiomatosis is a rare neurological and congenital disorder with a frequency of 1 in 50,000 births. This syndrome is a nonhereditary developmental condition and is characterized by the presence of congenital capillary malformation and a hamartomatous vascular proliferation involving the face (port-wine stain or facial birthmark), sometimes skull and the tissues of brain, jaws, oral soft and hard tissues and rarely other body organs. Seizures, mental retardation, and cortical calcification (tram-tracks) and congenital glaucoma may be seen in this syndrome. We report here a 40-year-old female with Sturge-Weber syndrome associated with bilateral cutaneous capillary malformation on her face, neck, hands and feet and also gingival enlargement.

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