Usefulness of diagnostic criteria of tuberous sclerosis complex in pediatric patients.

The Tuberous Sclerosis Complex 1998 Consensus Conference clinical criteria represent an important advance in the diagnosis of tuberous sclerosis complex. Since many findings regarded as highly specific for tuberous sclerosis complex are not apparent until late childhood or adulthood, refinements by age may prove of value. We have stratified 106 children into five age groups (0 to 2 years of age, above 2 to 5 years, above 5 to 9 years, above 9 to 14 years, and above 14 to 18 years). Physicians should be alerted as to the frequency of the criteria in different stages of children.

Intractable epilepsy in children who develop epilepsy in the first decade of life--a prospective study.

Prospective studies on 347 children under treatment due to seizures which appeared in the first 10 years of life helped to evaluate and define the incidence of epilepsy resistant to treatment. With regard to each patient the following aspects were analysed: the kind of seizures, their etiology, accompanying neurological disorders and a type of epileptic syndrome, kind of treatment applied before admission to the clinical department as well as socioeconomic conditions of the families. Patients under study were divided into four age groups to evaluate the results. Epilepsy, which was completely resistant to treatment, was observed in 10% of the patients, partially resistant in 20%. Resistance is the outcome of the following factors: onset of epilepsy in early childhood, symptomatic etiology coexisting symptoms of CNS damage, occurrence of unfavourable epileptic syndromes such as Lennox-Gastaut, wrong selection and low dosage of drugs, inappropriate polytherapy and adverse social conditions. On the basis of the results obtained, the authors suggested point evaluation as a kind of screening in prediction of failures in treatment.