levetiracetam and Tuberous-Sclerosis

Tuberous sclerosis or Tuberous Sclerosis Complex (TSC) is a genetic disorder characterized by the growth of numerous benign tumours in many parts of the body caused by mutations on either of two genes, TSC1 and TSC2. The case of a 31 year old female who fulfilled 8 major criteria of Tuberous Sclerosis Complex is reported. Multiple research work projects are being carried out on this disease, unfolding the realities regarding its etiology as well as treatment. TSC patients, besides receiving symptomatic treatment should also be offered special schooling and regular follow up by expert physician.

Topics: Adult; Anticonvulsants; Carbamazepine; Female; Humans; Levetiracetam; Mutation; Piracetam; Tuberous Sclerosis; Tuberous Sclerosis Complex 1 Protein; Tuberous Sclerosis Complex 2 Protein; Tumor Suppressor Proteins

We present the case of a boy with tuberous sclerosis who was referred for evaluation and treatment of his intractable epileptic seizures, having failed multiple anti-epileptic drug trials. He was subsequently treated with Levetiracetam that was gradually titrated to an effective dose, achieving full suppression of his seizures. Thereafter, his concomitant anti-epileptic drugs were gradually reduced and eventually discontinued. He remained on monotherapy with Levetiracetam, which continued to fully control his seizures. His EEG tracings before and after treatment are presented and compared, showing normalization of the latter.. Levetiracetam appears to be effective in treatment-resistant seizures which are symptomatic to tuberous sclerosis when used adjunctively as well as in monotherapy. This is the first report in the English literature regarding its use and efficacy in this condition.

Topics: Anticonvulsants; Child; Follow-Up Studies; Humans; Levetiracetam; Male; Piracetam; Seizures; Tuberous Sclerosis

Seizures are a common neurologic symptom of tuberous sclerosis complex. The use of levetiracetam as adjunctive antiepileptic therapy was assessed in 20 patients with tuberous sclerosis complex aged 2 to 19 years. In this retrospective evaluation, 40% of patients treated with levetiracetam achieved a seizure reduction of more than 50%. Levetiracetam was generally well tolerated, and adverse events were relatively uncommon in patients who responded to treatment. The most commonly reported adverse events were behavioral problems. Unstable gait, insomnia, poor appetite, and increased seizure frequency were also reported. Based on these results, the use of levetiracetam as adjunctive antiepileptic therapy can reduce seizure frequency in patients with tuberous sclerosis complex. (J Child Neurol 2006;21:53-57).

Topics: Adolescent; Adult; Anorexia; Anticonvulsants; Behavioral Symptoms; Child; Child, Preschool; Dose-Response Relationship, Drug; Drug Therapy, Combination; Female; Gait Disorders, Neurologic; Humans; Infant; Levetiracetam; Male; Piracetam; Retrospective Studies; Seizures; Sleep Initiation and Maintenance Disorders; Treatment Outcome; Tuberous Sclerosis