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.
