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  1. Article ; Online: Stiripentol for the treatment of seizures associated with Dravet syndrome in patients 6 months and older and taking clobazam.

    Vasquez, Alejandra / Wirrell, Elaine C / Youssef, Paul E

    Expert review of neurotherapeutics

    2023  Volume 23, Issue 4, Page(s) 297–309

    Abstract: ... neurotransmission and inhibits multiple hepatic isoenzymes (i.e. cytochrome P450 system) involved in the metabolism ...

    Abstract Introduction: Stiripentol (STP) is a structurally unique molecule with anticonvulsant and neuroprotective properties in animal and human studies. STP enhances gamma-aminobutyric acid (GABA)ergic neurotransmission and inhibits multiple hepatic isoenzymes (i.e. cytochrome P450 system) involved in the metabolism of other antiseizure medications (ASMs) potentiating their anticonvulsant effects and has proven to be a promising therapy in Dravet Syndrome (DS).
    Areas covered: The authors review randomized clinical trials and observational studies showing STP efficacy, safety, and tolerability when used as adjunctive therapy with VPA and clobazam in patients with DS. Moreover, they include recent evidence of its use in patients<2 years of age.
    Expert opinion: Evidence on STP demonstrates clinically meaningful efficacy in both short and long term in patients with DS. In addition to reducing convulsive seizure frequency, STP also markedly reduces the number of status epilepticus episodes and associated medical complications which are more common in younger children. STP adverse effects are generally not severe and often resolve following STP dose reduction or adjustments of concomitant ASMs. STP is approved by the FDA for children aged 6 months and older with DS who are also taking clobazam, making it the only DS-specific ASM for children under age 1 year.
    MeSH term(s) Child ; Child, Preschool ; Humans ; Anticonvulsants/pharmacology ; Clobazam/therapeutic use ; Epilepsies, Myoclonic/drug therapy ; Epilepsies, Myoclonic/complications ; Seizures/drug therapy ; Seizures/etiology ; Randomized Controlled Trials as Topic ; Observational Studies as Topic
    Chemical Substances Anticonvulsants ; Clobazam (2MRO291B4U) ; stiripentol (R02XOT8V8I)
    Language English
    Publishing date 2023-03-28
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 2112534-X
    ISSN 1744-8360 ; 1473-7175
    ISSN (online) 1744-8360
    ISSN 1473-7175
    DOI 10.1080/14737175.2023.2195550
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  2. Article ; Online: Pediatric psychogenic non-epileptic seizures: A retrospective observational cohort study at a quaternary center.

    Vasquez, Alejandra / Hilliker, Daniel R / Wirrell, Elaine C

    Epilepsy & behavior : E&B

    2023  Volume 146, Page(s) 109359

    Abstract: Background: Psychogenic non-epileptic seizures (PNES) represent a common functional disorder in the pediatric population. We aimed to characterize pediatric PNES by describing their clinical characteristics, PNES semiologies, and healthcare pathway ... ...

    Abstract Background: Psychogenic non-epileptic seizures (PNES) represent a common functional disorder in the pediatric population. We aimed to characterize pediatric PNES by describing their clinical characteristics, PNES semiologies, and healthcare pathway towards and after diagnosis.
    Material and methods: This was a retrospective, observational chart review of pediatric patients aged 6 to 18 years admitted between December 2020 and December 2021 for spell classification or suspected PNES. Psychogenic non-epileptic seizure diagnosis was made by the capture of a typical event on video electroencephalogram (vEEG). We used descriptive statistics to summarize demographic and clinical characteristics.
    Results: We included 26 patients (18 females, 69.2%) with a mean age (SD) of 13.9 (2.5) years. Pre-morbid neurologic and psychiatric conditions included: epilepsy (23.1%), migraine (46.2%), mild traumatic brain injury (26.9%), anxiety (57.7%), ADHD (34.6%), and depression (30.8%). Six patients (23.1%) had a prior diagnosis of PNES. 14 patients (53.8%) presented with convulsive, and 6 (23.1%) each with non-convulsive and mixed PNES. Patients were seen by a range of providers prior to diagnosis including ED providers (50%), neurologists (53.8%), pediatricians (34.6%), and psychology/psychiatry (11.5%). Emergency department evaluation occurred for 13 patients (50%) on 15 occasions, and six (23.1%) were admitted to the hospital. The median (p25-p75) time from PNES onset to presentation and diagnosis at our institution was 3.5 (1.5-6.2) and 4.1 (3-7) months, respectively. A total of 33 events from the 26 patients were captured on vEEG. The most frequent semiologies in our cohort were rhythmic motor (27.3%) followed by equal frequency (18.2%) of complex motor and dialeptic. Eighteen patients (69.2%) were followed after the PNES diagnosis, for a median (p25-p75) of 17.3 months (6.3-21) with variable outcome.
    Conclusion: Pediatric PNES has female predominance and often presents with comorbid psychosocial stressors and psychiatric conditions. High clinical suspicion and early recognition are crucial to decrease healthcare utilization and establish timely diagnosis and treatment.
    MeSH term(s) Humans ; Child ; Female ; Adolescent ; Male ; Retrospective Studies ; Psychophysiologic Disorders/complications ; Psychophysiologic Disorders/diagnosis ; Psychophysiologic Disorders/epidemiology ; Seizures/diagnosis ; Seizures/epidemiology ; Seizures/drug therapy ; Epilepsy/psychology ; Comorbidity ; Electroencephalography
    Language English
    Publishing date 2023-07-25
    Publishing country United States
    Document type Observational Study ; Journal Article
    ZDB-ID 2010587-3
    ISSN 1525-5069 ; 1525-5050
    ISSN (online) 1525-5069
    ISSN 1525-5050
    DOI 10.1016/j.yebeh.2023.109359
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  3. Article ; Online: Drug resistance in epilepsy.

    Perucca, Emilio / Perucca, Piero / White, H Steve / Wirrell, Elaine C

    The Lancet. Neurology

    2023  Volume 22, Issue 8, Page(s) 723–734

    Abstract: Drug resistance is estimated to affect about a third of individuals with epilepsy, but its prevalence differs in relation to the epilepsy syndrome, the cause of epilepsy, and other factors such as age of seizure onset and presence of associated ... ...

    Abstract Drug resistance is estimated to affect about a third of individuals with epilepsy, but its prevalence differs in relation to the epilepsy syndrome, the cause of epilepsy, and other factors such as age of seizure onset and presence of associated neurological deficits. Although drug-resistant epilepsy is not synonymous with unresponsiveness to any drug treatment, the probability of achieving seizure freedom on a newly tried medication decreases with increasing number of previously failed treatments. After two appropriately used antiseizure medications have failed to control seizures, individuals should be referred whenever possible to a comprehensive epilepsy centre for diagnostic re-evaluation and targeted management. The feasibility of epilepsy surgery and other treatments, including those targeting the cause of epilepsy, should be considered early after diagnosis. Substantial evidence indicates that a delay in identifying an effective treatment can adversely affect ultimate outcome and carry an increased risk of cognitive disability, other comorbidities, and premature mortality. Research on mechanisms of drug resistance and novel therapeutics is progressing rapidly, and potentially improved treatments, including those targeting disease modification, are on the horizon.
    MeSH term(s) Humans ; Anticonvulsants/therapeutic use ; Epilepsy/drug therapy ; Epilepsy/diagnosis ; Seizures/drug therapy ; Treatment Outcome ; Drug Resistant Epilepsy/drug therapy ; Drug Resistance
    Chemical Substances Anticonvulsants
    Language English
    Publishing date 2023-06-20
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 2081241-3
    ISSN 1474-4465 ; 1474-4422
    ISSN (online) 1474-4465
    ISSN 1474-4422
    DOI 10.1016/S1474-4422(23)00151-5
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  4. Article ; Online: Impact of Antiseizure Medications on Appetite and Weight in Children.

    Buraniqi, Ersida / Dabaja, Hicham / Wirrell, Elaine C

    Paediatric drugs

    2022  Volume 24, Issue 4, Page(s) 335–363

    Abstract: There are numerous potential factors that may affect growth in children with epilepsy, and these must be evaluated in any child with appetite and weight concerns. Antiseizure medications (ASMs) have potential adverse effects, and many may affect appetite, ...

    Abstract There are numerous potential factors that may affect growth in children with epilepsy, and these must be evaluated in any child with appetite and weight concerns. Antiseizure medications (ASMs) have potential adverse effects, and many may affect appetite, thus impacting normal growth and weight gain. The aim of this review is to focus on the impact of both epilepsy and ASMs on appetite and weight in children. We systematically reviewed studies using Medline assessing the impact of ASMs on appetite and weight in children. Eligible studies included randomized controlled trials and open-label studies (open-label extension and interventional) that targeted or included the pediatric population (0-18 years of age). Each study was classified using the American Academy of Neurology (AAN) Classification of Evidence for Therapeutic Studies, and the level of evidence for impact on appetite and weight in children was graded. ASMs associated with decreased appetite and/or weight loss include fenfluramine, topiramate, zonisamide, felbamate, rufinamide, stiripentol, cannabidiol, brivaracetam and ethosuximide; ASMs with minimal impact on weight and appetite in children include oxcarbazepine, eslicarbazepine, lamotrigine, levetiracetam, lacosamide, carbamazepine, vigabatrin and clobazam. The ASM most robustly associated with increased appetite and/or weight gain is valproic acid; however, both pregabalin and perampanel may also lead to modest weight gain or increased appetite in children. Certain ASMs may impact both appetite and weight, which may lead to increased morbidity of the underlying disease and impaired adherence to the treatment regimen.
    MeSH term(s) Anticonvulsants/adverse effects ; Appetite ; Child ; Epilepsy/drug therapy ; Humans ; Lamotrigine/therapeutic use ; Weight Gain
    Chemical Substances Anticonvulsants ; Lamotrigine (U3H27498KS)
    Language English
    Publishing date 2022-05-21
    Publishing country Switzerland
    Document type Journal Article ; Review ; Systematic Review
    ZDB-ID 1492748-2
    ISSN 1179-2019 ; 1174-5878
    ISSN (online) 1179-2019
    ISSN 1174-5878
    DOI 10.1007/s40272-022-00505-2
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  5. Article ; Online: Efficacy of felbamate in a cohort of patients with epilepsy with myoclonic atonic seizures (EMAtS).

    Reed, Laurel / Ciliberto, Michael / Fong, Susan L / Nickels, Katherine / Kossoff, Eric / Wirrell, Elaine / Joshi, Charuta

    Epilepsy research

    2024  Volume 201, Page(s) 107314

    Abstract: Epilepsy with myoclonic atonic seizures (EMAtS) is a rare childhood onset developmental and epileptic encephalopathy which is frequently refractory to medical therapy. The optimal antiseizure medication remains unknown. This study reports the efficacy of ...

    Abstract Epilepsy with myoclonic atonic seizures (EMAtS) is a rare childhood onset developmental and epileptic encephalopathy which is frequently refractory to medical therapy. The optimal antiseizure medication remains unknown. This study reports the efficacy of felbamate in children with EMAtS. Six large pediatric epilepsy centers performed a retrospective chart review on patients diagnosed with EMAtS at their institutions and collected data on felbamate usage and efficacy. Responders were classified as patients who had a 50% or greater reduction in seizures with a given therapy. Out of 259 patients, 37 (14%) were treated with felbamate. The efficacy of felbamate was 62%, which was greater than that of either levetiracetam or valproic acid (15%, p < 0.001% and 32%, p = 0.001 respectively) and similar to that of the ketogenic diet (69%, p = 0.8). Felbamate appears to be an effective treatment for EMAtS and should be strongly considered in the treatment course of this disease.
    MeSH term(s) Child ; Humans ; Felbamate/therapeutic use ; Retrospective Studies ; Electroencephalography ; Epilepsy/drug therapy ; Epilepsies, Myoclonic/drug therapy ; Seizures/drug therapy ; Anticonvulsants/therapeutic use
    Chemical Substances Felbamate (X72RBB02N8) ; Anticonvulsants
    Language English
    Publishing date 2024-02-02
    Publishing country Netherlands
    Document type Journal Article
    ZDB-ID 632939-1
    ISSN 1872-6844 ; 0920-1211
    ISSN (online) 1872-6844
    ISSN 0920-1211
    DOI 10.1016/j.eplepsyres.2024.107314
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  6. Article ; Online: New and emerging pharmacologic treatments for developmental and epileptic encephalopathies.

    Vasquez, Alejandra / Buraniqi, Ersida / Wirrell, Elaine C

    Current opinion in neurology

    2021  Volume 35, Issue 2, Page(s) 145–154

    Abstract: Purpose of review: Summarize evidence on Developmental and Epileptic Encephalopathies (DEEs) treatments focusing on new and emerging pharmacologic therapies (see Video, http://links.lww.com/CONR/A61, Supplementary Digital Content 1, which provides an ... ...

    Abstract Purpose of review: Summarize evidence on Developmental and Epileptic Encephalopathies (DEEs) treatments focusing on new and emerging pharmacologic therapies (see Video, http://links.lww.com/CONR/A61, Supplementary Digital Content 1, which provides an overview of the review).
    Recent findings: Advances in the fields of molecular genetics and neurobiology have led to the recognition of underlying pathophysiologic mechanisms involved in an increasing number of DEEs that could be targeted with precision therapies or repurposed drugs, some of which are currently being evaluated in clinical trials. Prompt, optimal therapy is critical, and promising therapies approved or in clinical trials for tuberous sclerosis complex, Dravet and Lennox-Gastaut Syndromes including mammalian target of rapamycin inhibitors, selective membrane channel and antisense oligonucleotide modulation, and repurposed drugs such as fenfluramine, stiripentol and cannabidiol, among others, may improve seizure burden and neurological outcomes. There is an urgent need for collaborative efforts to evaluate the efficacy and safety of emerging DEEs therapies.
    Summary: Development of new therapies promise to address unmet needs for patients with DEEs, including improvement of neurocognitive function and quality of life.
    MeSH term(s) Anticonvulsants/therapeutic use ; Epilepsies, Myoclonic/chemically induced ; Epilepsies, Myoclonic/drug therapy ; Fenfluramine/pharmacology ; Fenfluramine/therapeutic use ; Humans ; Lennox Gastaut Syndrome/drug therapy ; Quality of Life
    Chemical Substances Anticonvulsants ; Fenfluramine (2DS058H2CF)
    Language English
    Publishing date 2021-11-24
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 1182686-1
    ISSN 1473-6551 ; 1350-7540
    ISSN (online) 1473-6551
    ISSN 1350-7540
    DOI 10.1097/WCO.0000000000001029
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  7. Article ; Online: International consensus on diagnosis and management of Dravet syndrome.

    Wirrell, Elaine C / Hood, Veronica / Knupp, Kelly G / Meskis, Mary Anne / Nabbout, Rima / Scheffer, Ingrid E / Wilmshurst, Jo / Sullivan, Joseph

    Epilepsia

    2022  Volume 63, Issue 7, Page(s) 1761–1777

    Abstract: Objective: This study was undertaken to gain consensus from experienced physicians and caregivers regarding optimal diagnosis and management of Dravet syndrome (DS), in the context of recently approved, DS-specific therapies and emerging disease- ... ...

    Abstract Objective: This study was undertaken to gain consensus from experienced physicians and caregivers regarding optimal diagnosis and management of Dravet syndrome (DS), in the context of recently approved, DS-specific therapies and emerging disease-modifying treatments.
    Methods: A core working group was convened consisting of six physicians with recognized expertise in DS and two representatives of the Dravet Syndrome Foundation. This core group summarized the current literature (focused on clinical presentation, comorbidities, maintenance and rescue therapies, and evolving disease-modifying therapies) and nominated the 31-member expert panel (ensuring international representation), which participated in two rounds of a Delphi process to gain consensus on diagnosis and management of DS.
    Results: There was strong consensus that infants 2-15 months old, presenting with either a first prolonged hemiclonic seizure or first convulsive status epilepticus with fever or following vaccination, in the absence of another cause, should undergo genetic testing for DS. Panelists agreed on evolution of specific comorbidities with time, but less agreement was achieved on optimal management. There was also agreement on appropriate first- to third-line maintenance therapies, which included the newly approved agents. Whereas there was agreement for recommendation of disease-modifying therapies, if they are proven safe and efficacious for seizures and/or reduction of comorbidities, there was less consensus for when these should be started, with caregivers being more conservative than physicians.
    Significance: This International DS Consensus, informed by both experienced global caregiver and physician voices, provides a strong overview of the impact of DS, therapeutic goals and optimal management strategies incorporating the recent therapeutic advances in DS, and evolving disease-modifying therapies.
    MeSH term(s) Consensus ; Epilepsies, Myoclonic/diagnosis ; Epilepsies, Myoclonic/genetics ; Epilepsies, Myoclonic/therapy ; Epileptic Syndromes ; Humans ; Infant ; Seizures/drug therapy ; Spasms, Infantile
    Language English
    Publishing date 2022-05-12
    Publishing country United States
    Document type Journal Article
    ZDB-ID 216382-2
    ISSN 1528-1167 ; 0013-9580
    ISSN (online) 1528-1167
    ISSN 0013-9580
    DOI 10.1111/epi.17274
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  8. Article ; Online: Adaptive functioning and neurodevelopment in patients with Dravet syndrome: 12-month interim analysis of the BUTTERFLY observational study.

    Sullivan, Joseph / Wirrell, Elaine / Knupp, Kelly G / Chen, Dillon / Flamini, Robert / Zafar, Muhammad / Ventola, Pam / Avendaño, Javier / Wang, Fei / Parkerson, Kimberly A / Ticho, Barry

    Epilepsy & behavior : E&B

    2024  Volume 151, Page(s) 109604

    Abstract: ... Global Impression of Change scales (CGI-C or CaGI-C) respectively.: Results: Overall, 36 patients were ... in the CaGI-C scores for the all-patient group.: Significance: This BUTTERFLY interim analysis shows small ...

    Abstract Objective: The BUTTERFLY observational study aims to elucidate the natural trajectory of Dravet syndrome (DS) and associated comorbidities in order to establish a baseline for clinical therapies. We present the 12-month interim analysis of the study.
    Materials and methods: Patients with a genetically confirmed diagnosis of DS were enrolled in the study. Adaptive functioning and neurodevelopmental status were measured using the Vineland Adaptive Behavior Scale, Third Edition (Vineland-III), Bayley Scales of Infant Development, Third Edition (BSID-III), and Wechsler Preschool and Primary Scale of Intelligence, Fourth Edition (WPPSI-IV). Executive function, ambulatory function and locomotor activities, and overall clinical status were measured using the Behavior Rating Inventory of Executive Function - Preschool Version (BRIEF-P) scale, Gillette Functional Assessment Questionnaire (Gillette FAQ), and Clinician or Caregiver Global Impression of Change scales (CGI-C or CaGI-C) respectively.
    Results: Overall, 36 patients were enrolled across three age groups, with 35 patients completing at least part or all of one post-baseline visit through Month 12. Significant improvements in receptive communication, as assessed by Vineland-III and BSID-III raw scores, and in verbal comprehension subtests, as assessed by WPPSI-IV raw scores, were observed in BUTTERFLY patients for the all-patient group. Many patients performed on the impaired end of the BRIEF-P Global Executive Composite scale at baseline suggesting difficulties in executive function, and no significant change was observed in BRIEF-P scores for the all-patient group. Most patients performed in the dynamic range of the Gillette FAQ at baseline, and no significant change was observed in Gillette FAQ scores for the all-patient group. Lastly, there was significant improvement observed in the CaGI-C scores for the all-patient group.
    Significance: This BUTTERFLY interim analysis shows small improvements in communication skills along with stability in other developmental abilities across patients with DS enrolled in the study from baseline to Month 12.
    MeSH term(s) Child, Preschool ; Humans ; Infant ; Communication ; Epilepsies, Myoclonic ; Executive Function ; Wechsler Scales ; Observational Studies as Topic
    Language English
    Publishing date 2024-01-13
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2010587-3
    ISSN 1525-5069 ; 1525-5050
    ISSN (online) 1525-5069
    ISSN 1525-5050
    DOI 10.1016/j.yebeh.2023.109604
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  9. Article ; Online: Stereo-EEG localization of midline onset seizures on scalp EEG.

    Smith, Kelsey M / Starnes, Donnie K / Brinkmann, Benjamin H / So, Elson / Cox, Benjamin C / Marsh, W Richard / Van Gompel, Jamie J / Wirrell, Elaine / Britton, Jeffrey W / Burkholder, David B / Wong-Kisiel, Lily C

    Epilepsy research

    2023  Volume 193, Page(s) 107162

    Abstract: Purpose: The objective of this study was to describe the sEEG-defined seizure onset zone (SOZ), seizure semiology, presurgical evaluations, surgical intervention and outcome in patients with midline onset noninvasive phase I monitoring.: Methods: A ... ...

    Abstract Purpose: The objective of this study was to describe the sEEG-defined seizure onset zone (SOZ), seizure semiology, presurgical evaluations, surgical intervention and outcome in patients with midline onset noninvasive phase I monitoring.
    Methods: A single center sEEG database was reviewed to identify patients with seizures onset predominantly involving midline electrodes (FZ, CZ, PZ, OZ) on scalp EEG. Data abstracted included clinical factors, seizure semiology graded into lobar segmentation, imaging and electrographic findings, sEEG plan, interventions, and outcome.
    Results: Twelve patients were identified (8 males, median age of sEEG 28 years) out of 100 cases of sEEG performed from January 2015-September 2019. "Frontal lobe" seizure semiology was the most common. sEEG-defined SOZ were frontal (5), diffuse (1), multifocal (1), frontal and insular (1), frontal and cingulate (1), insular (1), cingulate (1), and mesial temporal (1). CZ and/or FZ scalp EEG changes were present for all patients with SOZ involving the frontal, cingulate, and insular regions. PZ/OZ scalp involvement was present in one patient with mesial temporal SOZ. Four patients underwent a definitive resective or ablative surgery, and the remaining patients underwent a palliative intervention. Of those with follow-up information available, 8/11 had seizure reduction by ≥ 50%, including 4 with an Engel I outcome. No clinical factors were associated with outcome.
    Conclusions: SOZ for midline onset seizures from noninvasive phase I monitoring was most commonly in the frontal, cingulate, and insular regions. A complex cortical network between these regions may explain overlap in semiology and scalp EEG findings. While the number rendered seizure-free was limited, a significant proportion experienced a reasonably favorable outcome justifying use of sEEG to identify surgical options in these patients.
    MeSH term(s) Male ; Humans ; Adult ; Scalp ; Drug Resistant Epilepsy/surgery ; Electroencephalography/methods ; Seizures/diagnostic imaging ; Seizures/surgery ; Electrodes, Implanted ; Magnetic Resonance Imaging
    Language English
    Publishing date 2023-05-05
    Publishing country Netherlands
    Document type Journal Article
    ZDB-ID 632939-1
    ISSN 1872-6844 ; 0920-1211
    ISSN (online) 1872-6844
    ISSN 0920-1211
    DOI 10.1016/j.eplepsyres.2023.107162
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  10. Article ; Online: Results of an international Delphi consensus in epilepsy with myoclonic atonic seizures/ Doose syndrome.

    Joshi, Charuta / Nickels, Katherine / Demarest, Scott / Eltze, Christin / Cross, J Helen / Wirrell, Elaine

    Seizure

    2020  Volume 85, Page(s) 12–18

    Abstract: Objective: To establish a standard framework for early phenotypic diagnosis, investigations, expected findings from investigations, evolution, effective therapies and prognosis in the syndrome of Epilepsy with myoclonic atonic seizures (EMAS) / Doose ... ...

    Abstract Objective: To establish a standard framework for early phenotypic diagnosis, investigations, expected findings from investigations, evolution, effective therapies and prognosis in the syndrome of Epilepsy with myoclonic atonic seizures (EMAS) / Doose syndrome.
    Methods: A core study group (CSG) interested in EMAS was convened. CSG then identified and nominated 15 experts in the field of EMAS. This expert panel (EP) from English speaking nations was invited to participate in anonymous questionnaires. A literature review was provided to them (supplement 1). Three rounds of questionnaires were sent to identify areas of consensus, strength of consensus and areas of contention.
    Results: Strong consensus was obtained regarding the clinical phenotype of EMAS: myoclonic atonic seizure was identified among others as a mandatory seizure type with typical onset of afebrile seizures between one and six years. A new term "stormy phase" (SP) was designated to delineate a characteristic phenotypic evolution in EMAS patients associated with seizure worsening. Strong consensus regarding the existence and time of onset of the SP, mandatory investigations to be performed early and later in the clinical course of EMAS, first and second tier treatment and prognostic factors for poor outcome were identified. Areas of lack of consensus included some seizure types that are necessary to diagnose EMAS, interictal EEG findings that prognosticate the course of EMAS, overall duration of SP, time to complete remission, and best approach to treat drug resistant EMAS.
    Significance: Expert consensus on core diagnostic criteria of EMAS necessary for natural history studies, phenotype-genotype correlations, and clinical trials including comparative studies was demonstrated. Areas of disagreements (especially prognostic features; treatment options) need further research.
    Language English
    Publishing date 2020-12-28
    Publishing country England
    Document type Journal Article
    ZDB-ID 1137610-7
    ISSN 1532-2688 ; 1059-1311
    ISSN (online) 1532-2688
    ISSN 1059-1311
    DOI 10.1016/j.seizure.2020.11.017
    Database MEDical Literature Analysis and Retrieval System OnLINE

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