Sturge Weber syndrome with intraoral manifestations. A case report.

A 15 Year old female patient with Sturge Weber Syndrome is presented. This neurocutaneous syndrome consists of angiomatosis of the skin and mucosa as well as the leptomeninges. This case report describes the classic presentation of the syndrome, emphasizing the oral manifestations. The radiographic and CT scan show the typical "tram line" intracranial calcifications. This case report presents a typical case of Sturge Weber Syndrome. It gives the radiological and CT scan findings and the important role played by them in the diagnosis of this syndrome. Emphasis is given to the differentiation of diphenylhydantoin induced gingival hyperplasia from the angiomatous enlargement of the gingiva before any treatment is planned.

Neuromuscular manifestations of diarrhea related hypokalemia.

Twenty-three children from 8-60 months (mean age 21.13 months) admitted with neuromuscular manifestations of diarrhea related hypokalemia were studied. Forty four per cent cases were suffering from diarrhea at the time of admission but in majority of cases (56%), the diarrheal episode had already terminated. Mild hypokalemia was seen in 17.4%, moderate in 43.5% and severe in 39.1%. Neck flop was the commonest (100%) neuromuscular manifestations followed by diminished bowel sounds (82.6%), truncal weakness (52.2%), weakness of limbs (52.2%), lethargy (43. 5%), abdominal distension (43.5%), respiratory involvement (4.3%) and phantom hernia (4.3%). Two cases (8.7%) had flaccid paralysis of both the lower limbs. Severe hypokalemia was more frequently observed in children below 24 months of age and those who had received i.v. fluids or salt sugar solution before reporting in the hospital. A significant correlation was noticed between severity of hypokalemia and frequency of stools (p < 0.05), degree of dehydration (p < 0.01), severity of nutrition (p < 0.01) and extent of neuromuscular involvement (p < 0.01). Our results highlights the importance of diarrhea related hypokalemia particularly in young malnourished children who are rehydrated with solutions inadequate in potassium. Early diagnosis and appropriate treatment can promptly reverse these manifestations within 48-72 hours.