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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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 < 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.</description><identifier>ISSN: 0920-1211</identifier><identifier>EISSN: 1872-6844</identifier><identifier>DOI: 10.1016/j.eplepsyres.2009.10.005</identifier><identifier>PMID: 19914805</identifier><identifier>CODEN: EPIRE8</identifier><language>eng</language><publisher>Kidlington: Elsevier B.V</publisher><subject>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</subject><ispartof>Epilepsy research, 2010-02, Vol.88 (2), p.112-117</ispartof><rights>Elsevier B.V.</rights><rights>2009 Elsevier B.V.</rights><rights>2015 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c490t-b0af6849bf28bd754565b536b6087973aeaba89f41d15c2661852e5624c57a173</citedby><cites>FETCH-LOGICAL-c490t-b0af6849bf28bd754565b536b6087973aeaba89f41d15c2661852e5624c57a173</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S0920121109002939$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,776,780,3537,27901,27902,65534</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=22576089$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/19914805$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Specchio, Nicola</creatorcontrib><creatorcontrib>Balestri, Martina</creatorcontrib><creatorcontrib>Striano, Pasquale</creatorcontrib><creatorcontrib>Cilio, Maria Roberta</creatorcontrib><creatorcontrib>Nardello, Rosaria</creatorcontrib><creatorcontrib>Patanè, Santina</creatorcontrib><creatorcontrib>Margiotta, Maria Luisa</creatorcontrib><creatorcontrib>D’Orsi, Giuseppe</creatorcontrib><creatorcontrib>Striano, Salvatore</creatorcontrib><creatorcontrib>Russo, Silvia</creatorcontrib><creatorcontrib>Specchio, Luigi Maria</creatorcontrib><creatorcontrib>Cusmai, Raffaella</creatorcontrib><creatorcontrib>Fusco, Lucia</creatorcontrib><creatorcontrib>Vigevano, Federico</creatorcontrib><title>Efficacy of levetiracetam in the treatment of drug-resistant Rett syndrome</title><title>Epilepsy research</title><addtitle>Epilepsy Res</addtitle><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.</description><subject>Adolescent</subject><subject>Analysis of Variance</subject><subject>Anticonvulsants - therapeutic use</subject><subject>Anticonvulsants. Antiepileptics. Antiparkinson agents</subject><subject>Biological and medical sciences</subject><subject>Child</subject><subject>Drug Administration Schedule</subject><subject>Drug-resistance</subject><subject>EEG</subject><subject>Electroencephalography</subject><subject>Female</subject><subject>Focal seizures</subject><subject>Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy</subject><subject>Humans</subject><subject>Levetiracetam</subject><subject>Medical sciences</subject><subject>Methyl-CpG-Binding Protein 2 - genetics</subject><subject>Myoclonic seizures</subject><subject>Nervous system (semeiology, syndromes)</subject><subject>Neurology</subject><subject>Neuropharmacology</subject><subject>Patient Selection</subject><subject>Pharmacology. Drug treatments</subject><subject>Piracetam - analogs & derivatives</subject><subject>Piracetam - therapeutic use</subject><subject>Prospective Studies</subject><subject>Quality of Life</subject><subject>Rett syndrome</subject><subject>Rett Syndrome - drug therapy</subject><subject>Rett Syndrome - genetics</subject><subject>Seizures - drug therapy</subject><subject>Seizures - genetics</subject><subject>Treatment Outcome</subject><subject>Young Adult</subject><issn>0920-1211</issn><issn>1872-6844</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2010</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkkmLFDEUgIMoTjv6F6Qu4qnal1SWykXQYcaFAcHlHFKpF01bS5ukBurfm6IbB7zoKfDyvSXfCyEVhT0FKl8d9ngc8JjWiGnPAHQJ7wHEA7KjrWK1bDl_SHagGdSUUXpBnqR0AAAFnD8mF1RrylsQO_Lx2vvgrFur2VcD3mEO0TrMdqzCVOUfWOWINo845Y3o4_K9Lk1DyrZEPmPOVVqnPs4jPiWPvB0SPjufl-TbzfXXq_f17ad3H67e3NaOa8h1B9aX-XTnWdv1SnAhRSca2UlolVaNRdvZVntOeyock5K2gqGQjDuhLFXNJXl5qnuM868FUzZjSA6HwU44L8koLhSjmul_k02jGyUpL2R7Il2cU4rozTGG0cbVUDCbcnMw98rNpny7KcpL6vNzk6Ubsb9PPDsuwIszYJOzg492ciH94RgTqjx9m_bticMi7y5gNMkFnBz2IaLLpp_D_0zz-q8ibghT2fDwE1dMh3mJU1mOoSYxA-bL9kW2HwIaoBjTzW9Csbis</recordid><startdate>20100201</startdate><enddate>20100201</enddate><creator>Specchio, Nicola</creator><creator>Balestri, Martina</creator><creator>Striano, Pasquale</creator><creator>Cilio, Maria Roberta</creator><creator>Nardello, Rosaria</creator><creator>Patanè, Santina</creator><creator>Margiotta, Maria Luisa</creator><creator>D’Orsi, Giuseppe</creator><creator>Striano, Salvatore</creator><creator>Russo, Silvia</creator><creator>Specchio, Luigi Maria</creator><creator>Cusmai, Raffaella</creator><creator>Fusco, Lucia</creator><creator>Vigevano, Federico</creator><general>Elsevier B.V</general><general>Elsevier</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>7TK</scope></search><sort><creationdate>20100201</creationdate><title>Efficacy of levetiracetam in the treatment of drug-resistant Rett syndrome</title><author>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</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c490t-b0af6849bf28bd754565b536b6087973aeaba89f41d15c2661852e5624c57a173</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2010</creationdate><topic>Adolescent</topic><topic>Analysis of Variance</topic><topic>Anticonvulsants - therapeutic use</topic><topic>Anticonvulsants. Antiepileptics. Antiparkinson agents</topic><topic>Biological and medical sciences</topic><topic>Child</topic><topic>Drug Administration Schedule</topic><topic>Drug-resistance</topic><topic>EEG</topic><topic>Electroencephalography</topic><topic>Female</topic><topic>Focal seizures</topic><topic>Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy</topic><topic>Humans</topic><topic>Levetiracetam</topic><topic>Medical sciences</topic><topic>Methyl-CpG-Binding Protein 2 - genetics</topic><topic>Myoclonic seizures</topic><topic>Nervous system (semeiology, syndromes)</topic><topic>Neurology</topic><topic>Neuropharmacology</topic><topic>Patient Selection</topic><topic>Pharmacology. Drug treatments</topic><topic>Piracetam - analogs & derivatives</topic><topic>Piracetam - therapeutic use</topic><topic>Prospective Studies</topic><topic>Quality of Life</topic><topic>Rett syndrome</topic><topic>Rett Syndrome - drug therapy</topic><topic>Rett Syndrome - genetics</topic><topic>Seizures - drug therapy</topic><topic>Seizures - genetics</topic><topic>Treatment Outcome</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Specchio, Nicola</creatorcontrib><creatorcontrib>Balestri, Martina</creatorcontrib><creatorcontrib>Striano, Pasquale</creatorcontrib><creatorcontrib>Cilio, Maria Roberta</creatorcontrib><creatorcontrib>Nardello, Rosaria</creatorcontrib><creatorcontrib>Patanè, Santina</creatorcontrib><creatorcontrib>Margiotta, Maria Luisa</creatorcontrib><creatorcontrib>D’Orsi, Giuseppe</creatorcontrib><creatorcontrib>Striano, Salvatore</creatorcontrib><creatorcontrib>Russo, Silvia</creatorcontrib><creatorcontrib>Specchio, Luigi Maria</creatorcontrib><creatorcontrib>Cusmai, Raffaella</creatorcontrib><creatorcontrib>Fusco, Lucia</creatorcontrib><creatorcontrib>Vigevano, Federico</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>Neurosciences Abstracts</collection><jtitle>Epilepsy research</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Specchio, Nicola</au><au>Balestri, Martina</au><au>Striano, Pasquale</au><au>Cilio, Maria Roberta</au><au>Nardello, Rosaria</au><au>Patanè, Santina</au><au>Margiotta, Maria Luisa</au><au>D’Orsi, Giuseppe</au><au>Striano, Salvatore</au><au>Russo, Silvia</au><au>Specchio, Luigi Maria</au><au>Cusmai, Raffaella</au><au>Fusco, Lucia</au><au>Vigevano, Federico</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Efficacy of levetiracetam in the treatment of drug-resistant Rett syndrome</atitle><jtitle>Epilepsy research</jtitle><addtitle>Epilepsy Res</addtitle><date>2010-02-01</date><risdate>2010</risdate><volume>88</volume><issue>2</issue><spage>112</spage><epage>117</epage><pages>112-117</pages><issn>0920-1211</issn><eissn>1872-6844</eissn><coden>EPIRE8</coden><abstract>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.</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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