Anti–tumor necrosis factor alpha therapy (adalimumab) in Rasmussen's encephalitis: An open pilot study
Summary Objective Rasmussen's encephalitis (RE) is a severe chronic inflammatory brain disease affecting one cerebral hemisphere and leading to drug‐resistant epilepsy, progressive neurologic deficit, and unilateral brain atrophy. Hemispherotomy remains the gold standard treatment but causes pe...
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| Veröffentlicht in: | Epilepsia (Copenhagen) 2016-06, Vol.57 (6), p.956-966 |
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| creator | Lagarde, Stanislas Villeneuve, Nathalie Trébuchon, Agnès Kaphan, Elsa Lepine, Anne McGonigal, Aileen Roubertie, Agathe Barthez, Marie‐Anne J. Trommsdorff, Valérie Lefranc, Jérémie Wehbi, Samer Portes, Vincent Laguitton, Virginie Quartier, Pierre Scavarda, Didier Giusiano, Bernard Milh, Mathieu Bulteau, Christine Bartolomei, Fabrice |
| description | Summary
Objective
Rasmussen's encephalitis (RE) is a severe chronic inflammatory brain disease affecting one cerebral hemisphere and leading to drug‐resistant epilepsy, progressive neurologic deficit, and unilateral brain atrophy. Hemispherotomy remains the gold standard treatment but causes permanent functional impairment. No standardized medical treatment protocol currently exists for patients prior to indication of hemispherotomy, although some immunotherapies have shown partial efficacy with functional preservation but poor antiseizure effect. Some studies suggest a role for tumor necrosis factor alpha (TNF‐α) in RE pathophysiology.
Methods
We report an open‐label study evaluating the efficacy and the safety of anti‐TNF‐α therapy (adalimumab) in 11 patients with RE. The primary outcome criterion was the decrease of seizure frequency. The secondary outcome criteria were neurologic and cognitive outcomes and existence of side effects.
Results
Adalimumab was introduced with a median delay of 31 months after seizure onset (range 1 month to 16 years), and follow‐up was for a median period of 18 months (range 9–54 months). There was a significant seizure frequency decrease after adalimumab administration (from a median of 360 to a median of 32 seizures per quarter, p ≤ 0.01). Statistical analysis showed that adalimumab had a significant intrinsic effect (p < 0.005) independent from disease fluctuations. Five patients (45%) were found to have sustained improvement over consecutive quarters in seizure frequency (decrease of 50%) on adalimumab. Three of these five patients also had no further neurocognitive deterioration. Adalimumab was well tolerated.
Significance
Our study reports efficacy of adalimumab in terms of seizure frequency control. In addition, stabilization of functional decline occurred in three patients. This efficacy might be particularly relevant for atypical slowly progressive forms of RE, in which hemispherotomy is not clearly indicated. Due to our study limitations, further studies are mandatory to confirm these preliminary results. |
| doi_str_mv | 10.1111/epi.13387 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_hal_p</sourceid><recordid>TN_cdi_hal_primary_oai_HAL_hal_01431274v1</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>1795879358</sourcerecordid><originalsourceid>FETCH-LOGICAL-c4227-e2e5e07eef894b59c85dfe3afff1d6edb755bd3ec4e4ebc59ce1ec22b6ad53643</originalsourceid><addsrcrecordid>eNp10d1KwzAUB_AgipsfF76ABLzQXXQmTdN03g2ZThgootchTU9ZpE1r0yq78x18Q5_EzOoEwdyEhB8nJ-eP0BElY-rXOdRmTBlLxBYaUh4mAaWx2EZDQigLJjwhA7Tn3BMhRMSC7aJBKCiJkzgaouXUtubj7b3tyqrBFnRTOeNwrnTrz6qolwq3S2hUvcJnKlOFKbtSpSNsLL5XruycA3vqMFgN3hamNe4CTy2uarC4NkXVYtd22eoA7eSqcHD4ve-jx6vZw-U8WNxe31xOF4GOwlAEEAIHIgDyZBKlfKITnuXAVJ7nNIshSwXnacZARxBBqj0ACjoM01hlnMUR20ejvq5vRtaNKVWzkpUycj5dyPUdoRGjoYheqLdnva2b6rkD18rSOA1FoSxUnZNU-OGJCeOJpyd_6FPVNdb_5EvFlCSc_T6-HqNrIN90QIlcRyV9VPIrKm-Pvyt2aQnZRv5k48F5D15NAav_K8nZ3U1f8hNiYZ7z</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1795610853</pqid></control><display><type>article</type><title>Anti–tumor necrosis factor alpha therapy (adalimumab) in Rasmussen's encephalitis: An open pilot study</title><source>MEDLINE</source><source>Wiley Journals</source><source>Wiley Online Library Free Content</source><source>EZB-FREE-00999 freely available EZB journals</source><source>Alma/SFX Local Collection</source><creator>Lagarde, Stanislas ; Villeneuve, Nathalie ; Trébuchon, Agnès ; Kaphan, Elsa ; Lepine, Anne ; McGonigal, Aileen ; Roubertie, Agathe ; Barthez, Marie‐Anne J. ; Trommsdorff, Valérie ; Lefranc, Jérémie ; Wehbi, Samer ; Portes, Vincent ; Laguitton, Virginie ; Quartier, Pierre ; Scavarda, Didier ; Giusiano, Bernard ; Milh, Mathieu ; Bulteau, Christine ; Bartolomei, Fabrice</creator><creatorcontrib>Lagarde, Stanislas ; Villeneuve, Nathalie ; Trébuchon, Agnès ; Kaphan, Elsa ; Lepine, Anne ; McGonigal, Aileen ; Roubertie, Agathe ; Barthez, Marie‐Anne J. ; Trommsdorff, Valérie ; Lefranc, Jérémie ; Wehbi, Samer ; Portes, Vincent ; Laguitton, Virginie ; Quartier, Pierre ; Scavarda, Didier ; Giusiano, Bernard ; Milh, Mathieu ; Bulteau, Christine ; Bartolomei, Fabrice</creatorcontrib><description>Summary
Objective
Rasmussen's encephalitis (RE) is a severe chronic inflammatory brain disease affecting one cerebral hemisphere and leading to drug‐resistant epilepsy, progressive neurologic deficit, and unilateral brain atrophy. Hemispherotomy remains the gold standard treatment but causes permanent functional impairment. No standardized medical treatment protocol currently exists for patients prior to indication of hemispherotomy, although some immunotherapies have shown partial efficacy with functional preservation but poor antiseizure effect. Some studies suggest a role for tumor necrosis factor alpha (TNF‐α) in RE pathophysiology.
Methods
We report an open‐label study evaluating the efficacy and the safety of anti‐TNF‐α therapy (adalimumab) in 11 patients with RE. The primary outcome criterion was the decrease of seizure frequency. The secondary outcome criteria were neurologic and cognitive outcomes and existence of side effects.
Results
Adalimumab was introduced with a median delay of 31 months after seizure onset (range 1 month to 16 years), and follow‐up was for a median period of 18 months (range 9–54 months). There was a significant seizure frequency decrease after adalimumab administration (from a median of 360 to a median of 32 seizures per quarter, p ≤ 0.01). Statistical analysis showed that adalimumab had a significant intrinsic effect (p < 0.005) independent from disease fluctuations. Five patients (45%) were found to have sustained improvement over consecutive quarters in seizure frequency (decrease of 50%) on adalimumab. Three of these five patients also had no further neurocognitive deterioration. Adalimumab was well tolerated.
Significance
Our study reports efficacy of adalimumab in terms of seizure frequency control. In addition, stabilization of functional decline occurred in three patients. This efficacy might be particularly relevant for atypical slowly progressive forms of RE, in which hemispherotomy is not clearly indicated. Due to our study limitations, further studies are mandatory to confirm these preliminary results.</description><identifier>ISSN: 0013-9580</identifier><identifier>EISSN: 1528-1167</identifier><identifier>EISSN: 1528-1157</identifier><identifier>DOI: 10.1111/epi.13387</identifier><identifier>PMID: 27106864</identifier><identifier>CODEN: EPILAK</identifier><language>eng</language><publisher>United States: Wiley Subscription Services, Inc</publisher><subject>Adalimumab ; Adalimumab - therapeutic use ; Adolescent ; Adult ; Anti-Inflammatory Agents - therapeutic use ; Anti–tumor necrosis factor alpha ; Child ; Child, Preschool ; Cognitive science ; Cohort Studies ; Electroencephalography ; Encephalitis ; Encephalitis - drug therapy ; Epilepsy ; Female ; Gangrene ; Humans ; Infant ; Inflammation ; Male ; Neuropsychological Tests ; Neuroscience ; Pilot Projects ; Rasmussen encephalitis ; Statistics, Nonparametric ; Treatment Outcome ; Tumor Necrosis Factor-alpha - immunology ; Tumor necrosis factor-TNF ; Young Adult</subject><ispartof>Epilepsia (Copenhagen), 2016-06, Vol.57 (6), p.956-966</ispartof><rights>Wiley Periodicals, Inc. © 2016 International League Against Epilepsy</rights><rights>Wiley Periodicals, Inc. © 2016 International League Against Epilepsy.</rights><rights>Copyright © 2016 International League Against Epilepsy</rights><rights>Distributed under a Creative Commons Attribution 4.0 International License</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4227-e2e5e07eef894b59c85dfe3afff1d6edb755bd3ec4e4ebc59ce1ec22b6ad53643</citedby><cites>FETCH-LOGICAL-c4227-e2e5e07eef894b59c85dfe3afff1d6edb755bd3ec4e4ebc59ce1ec22b6ad53643</cites><orcidid>0000-0002-0155-8597 ; 0000-0003-2916-1302 ; 0000-0002-8180-4857 ; 0000-0002-1678-0297 ; 0000-0001-6775-5318 ; 0000-0001-9344-8071 ; 0000-0002-1769-549X ; 0000-0002-5300-7738</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fepi.13387$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fepi.13387$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>230,314,780,784,885,1417,1433,27924,27925,45574,45575,46409,46833</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/27106864$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink><backlink>$$Uhttps://hal.science/hal-01431274$$DView record in HAL$$Hfree_for_read</backlink></links><search><creatorcontrib>Lagarde, Stanislas</creatorcontrib><creatorcontrib>Villeneuve, Nathalie</creatorcontrib><creatorcontrib>Trébuchon, Agnès</creatorcontrib><creatorcontrib>Kaphan, Elsa</creatorcontrib><creatorcontrib>Lepine, Anne</creatorcontrib><creatorcontrib>McGonigal, Aileen</creatorcontrib><creatorcontrib>Roubertie, Agathe</creatorcontrib><creatorcontrib>Barthez, Marie‐Anne J.</creatorcontrib><creatorcontrib>Trommsdorff, Valérie</creatorcontrib><creatorcontrib>Lefranc, Jérémie</creatorcontrib><creatorcontrib>Wehbi, Samer</creatorcontrib><creatorcontrib>Portes, Vincent</creatorcontrib><creatorcontrib>Laguitton, Virginie</creatorcontrib><creatorcontrib>Quartier, Pierre</creatorcontrib><creatorcontrib>Scavarda, Didier</creatorcontrib><creatorcontrib>Giusiano, Bernard</creatorcontrib><creatorcontrib>Milh, Mathieu</creatorcontrib><creatorcontrib>Bulteau, Christine</creatorcontrib><creatorcontrib>Bartolomei, Fabrice</creatorcontrib><title>Anti–tumor necrosis factor alpha therapy (adalimumab) in Rasmussen's encephalitis: An open pilot study</title><title>Epilepsia (Copenhagen)</title><addtitle>Epilepsia</addtitle><description>Summary
Objective
Rasmussen's encephalitis (RE) is a severe chronic inflammatory brain disease affecting one cerebral hemisphere and leading to drug‐resistant epilepsy, progressive neurologic deficit, and unilateral brain atrophy. Hemispherotomy remains the gold standard treatment but causes permanent functional impairment. No standardized medical treatment protocol currently exists for patients prior to indication of hemispherotomy, although some immunotherapies have shown partial efficacy with functional preservation but poor antiseizure effect. Some studies suggest a role for tumor necrosis factor alpha (TNF‐α) in RE pathophysiology.
Methods
We report an open‐label study evaluating the efficacy and the safety of anti‐TNF‐α therapy (adalimumab) in 11 patients with RE. The primary outcome criterion was the decrease of seizure frequency. The secondary outcome criteria were neurologic and cognitive outcomes and existence of side effects.
Results
Adalimumab was introduced with a median delay of 31 months after seizure onset (range 1 month to 16 years), and follow‐up was for a median period of 18 months (range 9–54 months). There was a significant seizure frequency decrease after adalimumab administration (from a median of 360 to a median of 32 seizures per quarter, p ≤ 0.01). Statistical analysis showed that adalimumab had a significant intrinsic effect (p < 0.005) independent from disease fluctuations. Five patients (45%) were found to have sustained improvement over consecutive quarters in seizure frequency (decrease of 50%) on adalimumab. Three of these five patients also had no further neurocognitive deterioration. Adalimumab was well tolerated.
Significance
Our study reports efficacy of adalimumab in terms of seizure frequency control. In addition, stabilization of functional decline occurred in three patients. This efficacy might be particularly relevant for atypical slowly progressive forms of RE, in which hemispherotomy is not clearly indicated. Due to our study limitations, further studies are mandatory to confirm these preliminary results.</description><subject>Adalimumab</subject><subject>Adalimumab - therapeutic use</subject><subject>Adolescent</subject><subject>Adult</subject><subject>Anti-Inflammatory Agents - therapeutic use</subject><subject>Anti–tumor necrosis factor alpha</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Cognitive science</subject><subject>Cohort Studies</subject><subject>Electroencephalography</subject><subject>Encephalitis</subject><subject>Encephalitis - drug therapy</subject><subject>Epilepsy</subject><subject>Female</subject><subject>Gangrene</subject><subject>Humans</subject><subject>Infant</subject><subject>Inflammation</subject><subject>Male</subject><subject>Neuropsychological Tests</subject><subject>Neuroscience</subject><subject>Pilot Projects</subject><subject>Rasmussen encephalitis</subject><subject>Statistics, Nonparametric</subject><subject>Treatment Outcome</subject><subject>Tumor Necrosis Factor-alpha - immunology</subject><subject>Tumor necrosis factor-TNF</subject><subject>Young Adult</subject><issn>0013-9580</issn><issn>1528-1167</issn><issn>1528-1157</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2016</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp10d1KwzAUB_AgipsfF76ABLzQXXQmTdN03g2ZThgootchTU9ZpE1r0yq78x18Q5_EzOoEwdyEhB8nJ-eP0BElY-rXOdRmTBlLxBYaUh4mAaWx2EZDQigLJjwhA7Tn3BMhRMSC7aJBKCiJkzgaouXUtubj7b3tyqrBFnRTOeNwrnTrz6qolwq3S2hUvcJnKlOFKbtSpSNsLL5XruycA3vqMFgN3hamNe4CTy2uarC4NkXVYtd22eoA7eSqcHD4ve-jx6vZw-U8WNxe31xOF4GOwlAEEAIHIgDyZBKlfKITnuXAVJ7nNIshSwXnacZARxBBqj0ACjoM01hlnMUR20ejvq5vRtaNKVWzkpUycj5dyPUdoRGjoYheqLdnva2b6rkD18rSOA1FoSxUnZNU-OGJCeOJpyd_6FPVNdb_5EvFlCSc_T6-HqNrIN90QIlcRyV9VPIrKm-Pvyt2aQnZRv5k48F5D15NAav_K8nZ3U1f8hNiYZ7z</recordid><startdate>201606</startdate><enddate>201606</enddate><creator>Lagarde, Stanislas</creator><creator>Villeneuve, Nathalie</creator><creator>Trébuchon, Agnès</creator><creator>Kaphan, Elsa</creator><creator>Lepine, Anne</creator><creator>McGonigal, Aileen</creator><creator>Roubertie, Agathe</creator><creator>Barthez, Marie‐Anne J.</creator><creator>Trommsdorff, Valérie</creator><creator>Lefranc, Jérémie</creator><creator>Wehbi, Samer</creator><creator>Portes, Vincent</creator><creator>Laguitton, Virginie</creator><creator>Quartier, Pierre</creator><creator>Scavarda, Didier</creator><creator>Giusiano, Bernard</creator><creator>Milh, Mathieu</creator><creator>Bulteau, Christine</creator><creator>Bartolomei, Fabrice</creator><general>Wiley Subscription Services, Inc</general><general>Wiley</general><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>7TK</scope><scope>7X8</scope><scope>1XC</scope><orcidid>https://orcid.org/0000-0002-0155-8597</orcidid><orcidid>https://orcid.org/0000-0003-2916-1302</orcidid><orcidid>https://orcid.org/0000-0002-8180-4857</orcidid><orcidid>https://orcid.org/0000-0002-1678-0297</orcidid><orcidid>https://orcid.org/0000-0001-6775-5318</orcidid><orcidid>https://orcid.org/0000-0001-9344-8071</orcidid><orcidid>https://orcid.org/0000-0002-1769-549X</orcidid><orcidid>https://orcid.org/0000-0002-5300-7738</orcidid></search><sort><creationdate>201606</creationdate><title>Anti–tumor necrosis factor alpha therapy (adalimumab) in Rasmussen's encephalitis: An open pilot study</title><author>Lagarde, Stanislas ; Villeneuve, Nathalie ; Trébuchon, Agnès ; Kaphan, Elsa ; Lepine, Anne ; McGonigal, Aileen ; Roubertie, Agathe ; Barthez, Marie‐Anne J. ; Trommsdorff, Valérie ; Lefranc, Jérémie ; Wehbi, Samer ; Portes, Vincent ; Laguitton, Virginie ; Quartier, Pierre ; Scavarda, Didier ; Giusiano, Bernard ; Milh, Mathieu ; Bulteau, Christine ; Bartolomei, Fabrice</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4227-e2e5e07eef894b59c85dfe3afff1d6edb755bd3ec4e4ebc59ce1ec22b6ad53643</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2016</creationdate><topic>Adalimumab</topic><topic>Adalimumab - therapeutic use</topic><topic>Adolescent</topic><topic>Adult</topic><topic>Anti-Inflammatory Agents - therapeutic use</topic><topic>Anti–tumor necrosis factor alpha</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Cognitive science</topic><topic>Cohort Studies</topic><topic>Electroencephalography</topic><topic>Encephalitis</topic><topic>Encephalitis - drug therapy</topic><topic>Epilepsy</topic><topic>Female</topic><topic>Gangrene</topic><topic>Humans</topic><topic>Infant</topic><topic>Inflammation</topic><topic>Male</topic><topic>Neuropsychological Tests</topic><topic>Neuroscience</topic><topic>Pilot Projects</topic><topic>Rasmussen encephalitis</topic><topic>Statistics, Nonparametric</topic><topic>Treatment Outcome</topic><topic>Tumor Necrosis Factor-alpha - immunology</topic><topic>Tumor necrosis factor-TNF</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Lagarde, Stanislas</creatorcontrib><creatorcontrib>Villeneuve, Nathalie</creatorcontrib><creatorcontrib>Trébuchon, Agnès</creatorcontrib><creatorcontrib>Kaphan, Elsa</creatorcontrib><creatorcontrib>Lepine, Anne</creatorcontrib><creatorcontrib>McGonigal, Aileen</creatorcontrib><creatorcontrib>Roubertie, Agathe</creatorcontrib><creatorcontrib>Barthez, Marie‐Anne J.</creatorcontrib><creatorcontrib>Trommsdorff, Valérie</creatorcontrib><creatorcontrib>Lefranc, Jérémie</creatorcontrib><creatorcontrib>Wehbi, Samer</creatorcontrib><creatorcontrib>Portes, Vincent</creatorcontrib><creatorcontrib>Laguitton, Virginie</creatorcontrib><creatorcontrib>Quartier, Pierre</creatorcontrib><creatorcontrib>Scavarda, Didier</creatorcontrib><creatorcontrib>Giusiano, Bernard</creatorcontrib><creatorcontrib>Milh, Mathieu</creatorcontrib><creatorcontrib>Bulteau, Christine</creatorcontrib><creatorcontrib>Bartolomei, Fabrice</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Neurosciences Abstracts</collection><collection>MEDLINE - Academic</collection><collection>Hyper Article en Ligne (HAL)</collection><jtitle>Epilepsia (Copenhagen)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Lagarde, Stanislas</au><au>Villeneuve, Nathalie</au><au>Trébuchon, Agnès</au><au>Kaphan, Elsa</au><au>Lepine, Anne</au><au>McGonigal, Aileen</au><au>Roubertie, Agathe</au><au>Barthez, Marie‐Anne J.</au><au>Trommsdorff, Valérie</au><au>Lefranc, Jérémie</au><au>Wehbi, Samer</au><au>Portes, Vincent</au><au>Laguitton, Virginie</au><au>Quartier, Pierre</au><au>Scavarda, Didier</au><au>Giusiano, Bernard</au><au>Milh, Mathieu</au><au>Bulteau, Christine</au><au>Bartolomei, Fabrice</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Anti–tumor necrosis factor alpha therapy (adalimumab) in Rasmussen's encephalitis: An open pilot study</atitle><jtitle>Epilepsia (Copenhagen)</jtitle><addtitle>Epilepsia</addtitle><date>2016-06</date><risdate>2016</risdate><volume>57</volume><issue>6</issue><spage>956</spage><epage>966</epage><pages>956-966</pages><issn>0013-9580</issn><eissn>1528-1167</eissn><eissn>1528-1157</eissn><coden>EPILAK</coden><abstract>Summary
Objective
Rasmussen's encephalitis (RE) is a severe chronic inflammatory brain disease affecting one cerebral hemisphere and leading to drug‐resistant epilepsy, progressive neurologic deficit, and unilateral brain atrophy. Hemispherotomy remains the gold standard treatment but causes permanent functional impairment. No standardized medical treatment protocol currently exists for patients prior to indication of hemispherotomy, although some immunotherapies have shown partial efficacy with functional preservation but poor antiseizure effect. Some studies suggest a role for tumor necrosis factor alpha (TNF‐α) in RE pathophysiology.
Methods
We report an open‐label study evaluating the efficacy and the safety of anti‐TNF‐α therapy (adalimumab) in 11 patients with RE. The primary outcome criterion was the decrease of seizure frequency. The secondary outcome criteria were neurologic and cognitive outcomes and existence of side effects.
Results
Adalimumab was introduced with a median delay of 31 months after seizure onset (range 1 month to 16 years), and follow‐up was for a median period of 18 months (range 9–54 months). There was a significant seizure frequency decrease after adalimumab administration (from a median of 360 to a median of 32 seizures per quarter, p ≤ 0.01). Statistical analysis showed that adalimumab had a significant intrinsic effect (p < 0.005) independent from disease fluctuations. Five patients (45%) were found to have sustained improvement over consecutive quarters in seizure frequency (decrease of 50%) on adalimumab. Three of these five patients also had no further neurocognitive deterioration. Adalimumab was well tolerated.
Significance
Our study reports efficacy of adalimumab in terms of seizure frequency control. In addition, stabilization of functional decline occurred in three patients. This efficacy might be particularly relevant for atypical slowly progressive forms of RE, in which hemispherotomy is not clearly indicated. Due to our study limitations, further studies are mandatory to confirm these preliminary results.</abstract><cop>United States</cop><pub>Wiley Subscription Services, Inc</pub><pmid>27106864</pmid><doi>10.1111/epi.13387</doi><tpages>11</tpages><orcidid>https://orcid.org/0000-0002-0155-8597</orcidid><orcidid>https://orcid.org/0000-0003-2916-1302</orcidid><orcidid>https://orcid.org/0000-0002-8180-4857</orcidid><orcidid>https://orcid.org/0000-0002-1678-0297</orcidid><orcidid>https://orcid.org/0000-0001-6775-5318</orcidid><orcidid>https://orcid.org/0000-0001-9344-8071</orcidid><orcidid>https://orcid.org/0000-0002-1769-549X</orcidid><orcidid>https://orcid.org/0000-0002-5300-7738</orcidid><oa>free_for_read</oa></addata></record> |
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| ispartof | Epilepsia (Copenhagen), 2016-06, Vol.57 (6), p.956-966 |
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| source | MEDLINE; Wiley Journals; Wiley Online Library Free Content; EZB-FREE-00999 freely available EZB journals; Alma/SFX Local Collection |
| subjects | Adalimumab Adalimumab - therapeutic use Adolescent Adult Anti-Inflammatory Agents - therapeutic use Anti–tumor necrosis factor alpha Child Child, Preschool Cognitive science Cohort Studies Electroencephalography Encephalitis Encephalitis - drug therapy Epilepsy Female Gangrene Humans Infant Inflammation Male Neuropsychological Tests Neuroscience Pilot Projects Rasmussen encephalitis Statistics, Nonparametric Treatment Outcome Tumor Necrosis Factor-alpha - immunology Tumor necrosis factor-TNF Young Adult |
| title | Anti–tumor necrosis factor alpha therapy (adalimumab) in Rasmussen's encephalitis: An open pilot study |
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