Summary of the Papers on Neurocutaneous Syndromes

Periodontal Management of Sturge-Weber Syndrome

Case Reports in Dentistry ◽

10.1155/2013/517145 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Butchibabu Kalakonda ◽

Koppolu Pradeep ◽

Ashank Mishra ◽

Krishnanjaneya Reddy ◽

Tupili Muralikrishna ◽

...

Keyword(s):

Oral Manifestations ◽

Port Wine Stain ◽

Scaling And Root Planing ◽

Port Wine ◽

Ocular Abnormalities ◽

Parietal Lobes ◽

Gingival Enlargement ◽

Weber Syndrome ◽

Sturge Weber Syndrome ◽

Neurocutaneous Syndromes

Sturge-Weber syndrome (SWS) is a sporadic disorder and is frequent among the neurocutaneous syndromes specifically with vascular predominance. This syndrome consists of constellation of clinical features like facial nevus, seizures, hemiparesis, intracranial calcifications, and mental retardation. It is characterized by focal port-wine stain, ocular abnormalities (glaucoma), and choroidal hemangioma and leptomeningeal angioma most often involving occipital and parietal lobes. The present paper reports three cases of SWS with oral manifestations and periodontal management, which included thorough scaling and root planing followed by gingivectomy with scalpel and laser in cases 1 and 3 consecutively to treat the gingival enlargement. However, the treatment in case 2 was deferred as the patient was not a candidate for periodontal surgery.

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LINEAR NEVUS SEBACEOUS SYNDROME: REPORT OF TWO CASES AND A REVIEW OF THE LITERATURE

PEDIATRICS ◽

10.1542/peds.52.3.382 ◽

1973 ◽

Vol 52 (3) ◽

pp. 382-387

Author(s):

Frederick H. Lovejoy ◽

William E. Boyle

Keyword(s):

Cerebrospinal Fluid ◽

Neurologic Disease ◽

Review Of The Literature ◽

Cutaneous Lesions ◽

Neurocutaneous Syndromes ◽

Nevus Sebaceous ◽

Cerebrospinal Fluid Protein ◽

Syndrome Report

Two cases of linear nevus sebaceous syndrome are described and a review of the eleven cases now reported in the literature is undertaken. The first patient has retardation, seizures, and classic ectodermal lesions while the second patient manifests typical cutaneous lesions and only an elevated cerebrospinal fluid protein as evidence of neurologic disease. The rationale for defining the syndrome as an entity distinct from other neurocutaneous syndromes is discussed and a pleomorphic presentation of the syndrome is suggested.

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