Efficacy of levetiracetam in the treatment of drug-resistant Rett syndrome

Summary 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...

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Veröffentlicht in:Epilepsy research 2010-02, Vol.88 (2), p.112-117
Hauptverfasser: Specchio, Nicola, Balestri, Martina, Striano, Pasquale, Cilio, Maria Roberta, Nardello, Rosaria, Patanè, Santina, Margiotta, Maria Luisa, D’Orsi, Giuseppe, Striano, Salvatore, Russo, Silvia, Specchio, Luigi Maria, Cusmai, Raffaella, Fusco, Lucia, Vigevano, Federico
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container_end_page 117
container_issue 2
container_start_page 112
container_title Epilepsy research
container_volume 88
creator Specchio, Nicola
Balestri, Martina
Striano, Pasquale
Cilio, Maria Roberta
Nardello, Rosaria
Patanè, Santina
Margiotta, Maria Luisa
D’Orsi, Giuseppe
Striano, Salvatore
Russo, Silvia
Specchio, Luigi Maria
Cusmai, Raffaella
Fusco, Lucia
Vigevano, Federico
description Summary 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.
doi_str_mv 10.1016/j.eplepsyres.2009.10.005
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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 &lt; 0.0001. 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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 &lt; 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.</abstract><cop>Kidlington</cop><pub>Elsevier B.V</pub><pmid>19914805</pmid><doi>10.1016/j.eplepsyres.2009.10.005</doi><tpages>6</tpages></addata></record>
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subjects Adolescent
Analysis of Variance
Anticonvulsants - therapeutic use
Anticonvulsants. Antiepileptics. Antiparkinson agents
Biological and medical sciences
Child
Drug Administration Schedule
Drug-resistance
EEG
Electroencephalography
Female
Focal seizures
Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy
Humans
Levetiracetam
Medical sciences
Methyl-CpG-Binding Protein 2 - genetics
Myoclonic seizures
Nervous system (semeiology, syndromes)
Neurology
Neuropharmacology
Patient Selection
Pharmacology. Drug treatments
Piracetam - analogs & derivatives
Piracetam - therapeutic use
Prospective Studies
Quality of Life
Rett syndrome
Rett Syndrome - drug therapy
Rett Syndrome - genetics
Seizures - drug therapy
Seizures - genetics
Treatment Outcome
Young Adult
title Efficacy of levetiracetam in the treatment of drug-resistant Rett syndrome
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