Infantile spasms and pigmentary mosaicism

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Infantile spasms.

Infantile spasms constitute both a distinctive seizure type and an age-specific epilepsy syndrome that have been extensively described for over a century. Standardization of the classification of infantile spasms has evolved, culminating in recent recommendations for separately recognizing and distinguishing the seizure type (spasms or epileptic spasms) and the epilepsy syndrome of infantile sp...

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Infantile spasms.

Overview Infantile spasms, also known as West syndrome, is a catastrophic childhood epilepsy with seizures that are difficult to control; it is associated with mental retardation. It usually has an onset during the first year of life, and typically between 4 and 8 months. Early recognition, careful diagnostic evaluation, and proper treatment may allow some children to attain seizure control and...

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Trisomy 8 mosaicism and favorable outcome after treatment of infantile spasms: case report.

Constitutional mosaic trisomy 8 syndrome occurs in approximately 1 of 35 000 live births. Clinically, it has a variable presentation. Some patients are asymptomatic, while others have multisystemic involvement. The overall incidence of neurological abnormalities has not been reported, but seizures are among the neurological symptoms associated with this condition. Previous reports describe asta...

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Infantile spasms Richard

Infantile spasms represent a seizure disorder with unique clinical and electroencephalographic (hypsarrhythmia) features and a poor prognosis including chronic intractable epilepsy and psychomotor retardation. The association of spasms and hypsarrhythmia, with or without mental retardation, defines West's syndrome. West's syndrome is not uncommon; the incidence is considered to be 0 16-0-42 per...

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Infantile spasms and HLA antigens.

21 new cases of infantile spasms were reported in 1976 from paediatric departments in Denmark. The connection between infantile spasms and the Lennox-Gastaut syndrome is mentioned, because of reports of a significantly higher incidence of HLA-B7 in children with Lennox-Gastaut syndrome. The HLA antigen distribution in 19 of the 21 children was compared with that of 1967 healthy adults. No diffe...

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ژورنال

عنوان ژورنال: Epilepsia

سال: 2009

ISSN: 0013-9580,1528-1167

DOI: 10.1111/j.1528-1167.2009.02475.x