Efficacy of levetiracetam in the treatment of drug-resistant Rett Syndrome.

Rosaria Nardello, Martina Balestri, Maria Luisa Margiotta, Santina Patanè, Giuseppe D'Orsi, Nicola Specchio, Maria Roberta Cilio, Pasquale Striano, Luigi Maria Specchio, Raffaella Cusmai, Federico Vigevano, Lucia Fusco, Salvatore Striano, Silvia Russo

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Abstract

Rett syndrome (RTT) is a progressive neurological disorder characterized by a wide spectrum of phenotypes. Epilepsy is reported to occur in 50–90% of patients with RTT; some develop medically refractory epilepsy. The aim of this study is to investigate the efficacy of levetiracetam (LEV) in drug-resistant patients with RTT.This prospective, pragmatic, open-label study consisted of an 8-week baseline period and a 6-month evaluation period. Efficacy variable was the mean frequency of monthly seizures before, and after 3 and 6 months of treatment with LEV.Eight female patients, aged 7.5–19 years (M12.8 ± 5) entered the study. Mean age at epilepsy onset was 25.8 ± 14.1 months. All patients showed MeCP2 mutation. Patients had been treated with a mean of 3.4 AEDs (2–7) before LEV. The mean LEV dose was 44.84 ± 18.02 mg/kg/day. The mean monthly seizure frequency for all types of seizures during the baseline period was 21.3 ± 8.1 (range 10–35); after 3 months it was 3.3 ± 4.1 (range 0–9) and after 6 months of LEV treatment it was 1.5 ± 2 (range 0–4), p < 0.0001. The mean follow-up period was 20.2 ± 13 months. Mild sleepiness occurred in two patients, one reported intermittent agitation.Levetiracetam appeared effective in our series of drug-resistant RTT patients. All reported a reduction in seizure frequency and consequently a better quality of life.
Lingua originaleEnglish
pagine (da-a)112-117
Numero di pagine5
RivistaEpilepsy Research
Volume88
Stato di pubblicazionePublished - 2010

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All Science Journal Classification (ASJC) codes

  • Neurology
  • Clinical Neurology

Cita questo

Nardello, R., Balestri, M., Margiotta, M. L., Patanè, S., D'Orsi, G., Specchio, N., Cilio, M. R., Striano, P., Specchio, L. M., Cusmai, R., Vigevano, F., Fusco, L., Striano, S., & Russo, S. (2010). Efficacy of levetiracetam in the treatment of drug-resistant Rett Syndrome. Epilepsy Research, 88, 112-117.