Pediatric-Onset Gelastic Seizures: Clinical Data and Outcome
Gelastic seizures are an extremely rare form of epilepsy defined as automatic bouts of laughter without mirth commonly associated with a hypothalamic hamartoma. The objective was to survey all Israeli children found to develop recurrent gelastic seizures and report presenting symptoms, electroenceph...
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| Veröffentlicht in: | Pediatric neurology 2007-07, Vol.37 (1), p.29-34 |
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| creator | Shahar, Eli, MD Kramer, Uri, MD Mahajnah, Muhammad, MD, PhD Lerman-Sagie, Tallie, MD Goez, Rachel, MD Gross, Varda, MD Kutai, Miriam, MD Genizi, Jacob, MD |
| description | Gelastic seizures are an extremely rare form of epilepsy defined as automatic bouts of laughter without mirth commonly associated with a hypothalamic hamartoma. The objective was to survey all Israeli children found to develop recurrent gelastic seizures and report presenting symptoms, electroencephalographic and radiologic data, and response to either antiepileptic drugs or surgery. Ten children who developed gelastic seizures at the age of 1 week to 6.5 years (mean, 25 months) at a frequency from 3 bouts per week to >10 prolonged bouts per day were followed for a period of 1.3-12 years (mean, 6 years). Seven cases were defined as symptomatic: cortical magnetic resonance imaging revealed a hypothalamic hamartoma in four patients and cortical abnormalities in three others. Seizure control was achieved in four patients, including a neonate with status gelasticus and hypothalamic hamartoma, and partial control in one more. Five children remained resistant to polytherapy, including three with hypothalamic hamartoma even after two of them underwent hemartoma excision. Thus, children with gelastic seizures may respond relatively well to drug therapy. Four of the 10 patients became seizure free with drug therapy; in three intractable symptomatic cases, surgery was tried but failed in two of the three. |
| doi_str_mv | 10.1016/j.pediatrneurol.2007.03.003 |
| format | Article |
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The objective was to survey all Israeli children found to develop recurrent gelastic seizures and report presenting symptoms, electroencephalographic and radiologic data, and response to either antiepileptic drugs or surgery. Ten children who developed gelastic seizures at the age of 1 week to 6.5 years (mean, 25 months) at a frequency from 3 bouts per week to >10 prolonged bouts per day were followed for a period of 1.3-12 years (mean, 6 years). Seven cases were defined as symptomatic: cortical magnetic resonance imaging revealed a hypothalamic hamartoma in four patients and cortical abnormalities in three others. Seizure control was achieved in four patients, including a neonate with status gelasticus and hypothalamic hamartoma, and partial control in one more. Five children remained resistant to polytherapy, including three with hypothalamic hamartoma even after two of them underwent hemartoma excision. Thus, children with gelastic seizures may respond relatively well to drug therapy. Four of the 10 patients became seizure free with drug therapy; in three intractable symptomatic cases, surgery was tried but failed in two of the three.</description><identifier>ISSN: 0887-8994</identifier><identifier>EISSN: 1873-5150</identifier><identifier>DOI: 10.1016/j.pediatrneurol.2007.03.003</identifier><identifier>PMID: 17628219</identifier><language>eng</language><publisher>New York, NY: Elsevier Inc</publisher><subject>Age of Onset ; Anticonvulsants - therapeutic use ; Biological and medical sciences ; Brain Diseases - epidemiology ; Brain Diseases - pathology ; Brain Diseases - surgery ; Child ; Child, Preschool ; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases ; Electroencephalography ; Epilepsies, Partial - diagnosis ; Epilepsies, Partial - drug therapy ; Epilepsies, Partial - epidemiology ; Female ; Hamartoma - epidemiology ; Hamartoma - pathology ; Hamartoma - surgery ; Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy ; Humans ; Hypothalamus - pathology ; Hypothalamus - surgery ; Infant ; Infant, Newborn ; Israel - epidemiology ; Magnetic Resonance Imaging ; Male ; Medical sciences ; Nervous system (semeiology, syndromes) ; Neurology ; Neurosurgical Procedures ; Pediatrics ; Severity of Illness Index ; Treatment Outcome</subject><ispartof>Pediatric neurology, 2007-07, Vol.37 (1), p.29-34</ispartof><rights>Elsevier Inc.</rights><rights>2007 Elsevier Inc.</rights><rights>2007 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c466t-aab52d730e9589867d6996808ecde7ffb6a09cbb2aae1eb46f7594800a1726053</citedby><cites>FETCH-LOGICAL-c466t-aab52d730e9589867d6996808ecde7ffb6a09cbb2aae1eb46f7594800a1726053</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.pediatrneurol.2007.03.003$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=18951722$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/17628219$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Shahar, Eli, MD</creatorcontrib><creatorcontrib>Kramer, Uri, MD</creatorcontrib><creatorcontrib>Mahajnah, Muhammad, MD, PhD</creatorcontrib><creatorcontrib>Lerman-Sagie, Tallie, MD</creatorcontrib><creatorcontrib>Goez, Rachel, MD</creatorcontrib><creatorcontrib>Gross, Varda, MD</creatorcontrib><creatorcontrib>Kutai, Miriam, MD</creatorcontrib><creatorcontrib>Genizi, Jacob, MD</creatorcontrib><title>Pediatric-Onset Gelastic Seizures: Clinical Data and Outcome</title><title>Pediatric neurology</title><addtitle>Pediatr Neurol</addtitle><description>Gelastic seizures are an extremely rare form of epilepsy defined as automatic bouts of laughter without mirth commonly associated with a hypothalamic hamartoma. The objective was to survey all Israeli children found to develop recurrent gelastic seizures and report presenting symptoms, electroencephalographic and radiologic data, and response to either antiepileptic drugs or surgery. Ten children who developed gelastic seizures at the age of 1 week to 6.5 years (mean, 25 months) at a frequency from 3 bouts per week to >10 prolonged bouts per day were followed for a period of 1.3-12 years (mean, 6 years). Seven cases were defined as symptomatic: cortical magnetic resonance imaging revealed a hypothalamic hamartoma in four patients and cortical abnormalities in three others. Seizure control was achieved in four patients, including a neonate with status gelasticus and hypothalamic hamartoma, and partial control in one more. Five children remained resistant to polytherapy, including three with hypothalamic hamartoma even after two of them underwent hemartoma excision. Thus, children with gelastic seizures may respond relatively well to drug therapy. Four of the 10 patients became seizure free with drug therapy; in three intractable symptomatic cases, surgery was tried but failed in two of the three.</description><subject>Age of Onset</subject><subject>Anticonvulsants - therapeutic use</subject><subject>Biological and medical sciences</subject><subject>Brain Diseases - epidemiology</subject><subject>Brain Diseases - pathology</subject><subject>Brain Diseases - surgery</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases</subject><subject>Electroencephalography</subject><subject>Epilepsies, Partial - diagnosis</subject><subject>Epilepsies, Partial - drug therapy</subject><subject>Epilepsies, Partial - epidemiology</subject><subject>Female</subject><subject>Hamartoma - epidemiology</subject><subject>Hamartoma - pathology</subject><subject>Hamartoma - surgery</subject><subject>Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy</subject><subject>Humans</subject><subject>Hypothalamus - pathology</subject><subject>Hypothalamus - surgery</subject><subject>Infant</subject><subject>Infant, Newborn</subject><subject>Israel - epidemiology</subject><subject>Magnetic Resonance Imaging</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Nervous system (semeiology, syndromes)</subject><subject>Neurology</subject><subject>Neurosurgical Procedures</subject><subject>Pediatrics</subject><subject>Severity of Illness Index</subject><subject>Treatment Outcome</subject><issn>0887-8994</issn><issn>1873-5150</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2007</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkU1r3DAQhkVpaLZJ_0IxlOZmZyRbX00phG2SFgIbSHsWsjwGbb32RrID6a-vzBpCc8ppLs-8MzwvIZ8oFBSoON8We2y8HUOPUxi6ggHIAsoCoHxDVlTJMueUw1uyAqVkrrSujsn7GLcAwDWr3pFjKgVTjOoV-Xp3yPIu3_QRx-wGOxtH77J79H-ngPFLtu58753tsu92tJntm2wzjW7Y4Sk5am0X8cMyT8jv66tf6x_57ebm5_ryNneVEGNubc1ZI0tAzZVWQjZCa6FAoWtQtm0tLGhX18xapFhXopVcVwrAUskE8PKEnB1y92F4mDCOZuejw66zPQ5TNBJSILAZvDiALgwxBmzNPvidDU-Ggpnlma35T56Z5RkoTZKXtj8uZ6Z6h83z7mIrAZ8XwMbkow22dz4-c0rz9DFL3NWBwyTl0WMw0XnsXboc0I2mGfwrH_r2IsctVfzBJ4zbYQp98m6oicyAuZ_7nusGCUBZxct_SjypXA</recordid><startdate>20070701</startdate><enddate>20070701</enddate><creator>Shahar, Eli, MD</creator><creator>Kramer, Uri, MD</creator><creator>Mahajnah, Muhammad, MD, PhD</creator><creator>Lerman-Sagie, Tallie, MD</creator><creator>Goez, Rachel, MD</creator><creator>Gross, Varda, MD</creator><creator>Kutai, Miriam, MD</creator><creator>Genizi, Jacob, MD</creator><general>Elsevier Inc</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></search><sort><creationdate>20070701</creationdate><title>Pediatric-Onset Gelastic Seizures: Clinical Data and Outcome</title><author>Shahar, Eli, MD ; Kramer, Uri, MD ; Mahajnah, Muhammad, MD, PhD ; Lerman-Sagie, Tallie, MD ; Goez, Rachel, MD ; Gross, Varda, MD ; Kutai, Miriam, MD ; Genizi, Jacob, MD</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c466t-aab52d730e9589867d6996808ecde7ffb6a09cbb2aae1eb46f7594800a1726053</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2007</creationdate><topic>Age of Onset</topic><topic>Anticonvulsants - therapeutic use</topic><topic>Biological and medical sciences</topic><topic>Brain Diseases - epidemiology</topic><topic>Brain Diseases - pathology</topic><topic>Brain Diseases - surgery</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases</topic><topic>Electroencephalography</topic><topic>Epilepsies, Partial - diagnosis</topic><topic>Epilepsies, Partial - drug therapy</topic><topic>Epilepsies, Partial - epidemiology</topic><topic>Female</topic><topic>Hamartoma - epidemiology</topic><topic>Hamartoma - pathology</topic><topic>Hamartoma - surgery</topic><topic>Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. 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The objective was to survey all Israeli children found to develop recurrent gelastic seizures and report presenting symptoms, electroencephalographic and radiologic data, and response to either antiepileptic drugs or surgery. Ten children who developed gelastic seizures at the age of 1 week to 6.5 years (mean, 25 months) at a frequency from 3 bouts per week to >10 prolonged bouts per day were followed for a period of 1.3-12 years (mean, 6 years). Seven cases were defined as symptomatic: cortical magnetic resonance imaging revealed a hypothalamic hamartoma in four patients and cortical abnormalities in three others. Seizure control was achieved in four patients, including a neonate with status gelasticus and hypothalamic hamartoma, and partial control in one more. Five children remained resistant to polytherapy, including three with hypothalamic hamartoma even after two of them underwent hemartoma excision. Thus, children with gelastic seizures may respond relatively well to drug therapy. Four of the 10 patients became seizure free with drug therapy; in three intractable symptomatic cases, surgery was tried but failed in two of the three.</abstract><cop>New York, NY</cop><pub>Elsevier Inc</pub><pmid>17628219</pmid><doi>10.1016/j.pediatrneurol.2007.03.003</doi><tpages>6</tpages></addata></record> |
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| subjects | Age of Onset Anticonvulsants - therapeutic use Biological and medical sciences Brain Diseases - epidemiology Brain Diseases - pathology Brain Diseases - surgery Child Child, Preschool Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Electroencephalography Epilepsies, Partial - diagnosis Epilepsies, Partial - drug therapy Epilepsies, Partial - epidemiology Female Hamartoma - epidemiology Hamartoma - pathology Hamartoma - surgery Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy Humans Hypothalamus - pathology Hypothalamus - surgery Infant Infant, Newborn Israel - epidemiology Magnetic Resonance Imaging Male Medical sciences Nervous system (semeiology, syndromes) Neurology Neurosurgical Procedures Pediatrics Severity of Illness Index Treatment Outcome |
| title | Pediatric-Onset Gelastic Seizures: Clinical Data and Outcome |
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