Neuropediatrics 2006; 37 - THP60
DOI: 10.1055/s-2006-945883

INFANTILE SPASMS IN TUBEROUS SCLEROSIS

F Buruiana 1, D Plesca 1, R Teleanu 1, AM Davitoiu 1, M Moiceanu 1, L Cretu 1, M Stefanescu 1, I Stancea 1, D Dragomir 1
  • 1“Dr. Victor Gomoiu” Children's Hospital, Bucharest, Romania

Objectives: Determination of incidence of infantile spasms in tuberous sclerosis; evaluation of therapeutic response and its correlation with neurodevelopment.

Material and Methods: The authors present the results of a clinical trial which included 17 patients with tuberous sclerosis, hospitalized betweeen 1995–2004. The main including criteria was the diagnisis of tuberous sclerosis.

Results: All the included patients had epilepsy. Among these, 14 (83%) had IS and 3 (17%) had other forms of epilepsy. The median age of onset of IS was 4 month. All the patients received anticonvulsant therapy: hormonal therapy (ACTH, prednisone), vigabatrin, sodium valproate, benzodiazepines (clonazepam, nitrazepam). The hormonal therapy was administered in 10 patients (71.43%); in 6 of them (42.85%) the epilepsy was controlled. In 4 patients (28.57%) the IS reappeared after cessation of steroids. Partial control of seizures was obtained after sodium valproate or benzodiazepines administration. Treatment with vigabatrin was initiated in 4 patients (28.57%); in 3 of them IS were cessed. Evaluation of therapeutical response and neurodevelopment was done at 3, 6, 12 and 24 month.

Conclusion: The incidence of IS in TS among the studied patients was greater than the data found in litterature (83%, in comparison to 69%). Early onset (around the age of 2 month) of IS was correlated with a less favorable response to tratment and with a more severe alteration of neurodevelopment. Patients with favorable response to vigabatrin had subsequently a more satisfactory development.