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Biomedical subjects

C Guardalà

Publications and source records attributed to C Guardalà.

4 recordsLinked to original sources
PubMed1984

Holoprosencephalic disorders. Case report of a semilobar type.

A child with microcephaly, facial dysmorphisms, seizures, and congenital cardiopathy is presented. On the basis of skull x-rays, electroencephalogram, transillumination of the head, and computed tomography (CT) scan, the diagnosis of semilobar holoprosencephaly was made. The heterogeneous etiology of the disorder is discussed in order to evaluate the recurrence risk. The usefulness of CT scan for the classification in the different types of holoprosencephaly is stressed.

Brain↗
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[New drugs in the treatment of childhood epilepsy: vigabatrin (study of 61 subjects)].

This study was conducted to determine the efficacy of vigabatrin in children with epilepsy. Sixty-one children with various types of severe epilepsy were studied. In 12 children vigabatrin was introduced as monotherapy, while in 49 it was added to other antiepileptic drugs. Following the introduction of vigabatrin, 17 patients became seizure free, and 19 responded with a greater than 50% reduction in seizure frequency. The following types of epilepsy responded favorably to treatment (in order of decreasing efficacy): West syndrome, especially if secondary to Tuberous Sclerosis; cryptogenic and symptomatic partial epilepsy; Lennox-Gastaut syndrome, and other symptomatic generalized epilepsy. Optimal responses were found with vigabatrin doses of 30 to 50 mg/kg/day. Forty-one responders continued on vigabatrin, with generally good efficacy. Vigabatrin tolerability was good; in only 6 children was treatment discontinued because of side-effects such as somnolence, irritability, weight gain, and cutaneous rash. This study shows that vigabatrin is effective in the treatment of refractory epilepsy, especially in West syndrome secondary to Tuberous Sclerosis, partial epilepsy, and Lennox-Gastaut syndrome. Further studies are needed to analyze the increased frequency of seizures and the appearance of new seizure types associated with vigabatrin treatment.

4-Aminobutyrate Transaminase↗