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Article ; Online: Response Letter to the Editor: "Expanding eligibility for intracranial electroencephalography using dexmedetomidine hydrochloride in children with behavioral dyscontrol".

Johnstone, Thomas / Guinle, Maria Isabel Barros / Grant, Gerald A / Porter, Brenda E

2024 Volume 153, Page(s) 109657

MeSH term(s)	Child ; Humans ; Dexmedetomidine/therapeutic use ; Electrocorticography ; Hypnotics and Sedatives ; Electroencephalography
Chemical Substances	Dexmedetomidine (67VB76HONO) ; Hypnotics and Sedatives
Language	English
Publishing date	2024-02-17
Publishing country	United States
Document type	Letter
ZDB-ID	2010587-3
ISSN	1525-5069 ; 1525-5050
ISSN (online)	1525-5069
ISSN	1525-5050
DOI	10.1016/j.yebeh.2024.109657
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Article ; Online: Practical Advice on Surviving and Thriving as an Academic Physician-Neuroscientist.

Knowles, Juliet K / Porter, Brenda E

JAMA neurology

2021 Volume 78, Issue 12, Page(s) 1439–1440

MeSH term(s)	Academies and Institutes ; Career Choice ; Humans ; Neurosciences/education ; Physicians
Language	English
Publishing date	2021-10-24
Publishing country	United States
Document type	Journal Article
ZDB-ID	2702023-X
ISSN	2168-6157 ; 2168-6149
ISSN (online)	2168-6157
ISSN	2168-6149
DOI	10.1001/jamaneurol.2021.3889
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Article: Growth and Overall Health of Patients with

Brown, Tanya L / Nye, Kimberly L / Porter, Brenda E

Metabolites

2021 Volume 11, Issue 11

Abstract: We were interested in elucidating the non-neurologic health of patients with autosomal ... ...

Abstract	We were interested in elucidating the non-neurologic health of patients with autosomal recessive
Language	English
Publishing date	2021-10-29
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2662251-8
ISSN	2218-1989
ISSN	2218-1989
DOI	10.3390/metabo11110746
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Article: Characterizing a rare neurogenetic disease, SLC13A5 citrate transporter disorder, utilizing clinical data in a cloud-based medical record collection system.

Spelbrink, Emily M / Brown, Tanya L / Brimble, Elise / Blanco, Kirsten A / Nye, Kimberly L / Porter, Brenda E

Frontiers in genetics

2023 Volume 14, Page(s) 1109547

Abstract: Introduction: ...

Abstract	Introduction:
Language	English
Publishing date	2023-03-21
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2606823-0
ISSN	1664-8021
ISSN	1664-8021
DOI	10.3389/fgene.2023.1109547
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Article ; Online: Caregivers' impression of epilepsy surgery in patients with tuberous sclerosis complex.

Nguyen, Thuy / Porter, Brenda E

Epilepsy & behavior : E&B

2020 Volume 111, Page(s) 107331

Abstract: Epilepsy surgery is successful in the majority of patients with tuberous sclerosis complex (TSC), with high rates of postoperative seizure reduction and even seizure freedom. Epilepsy surgery is recommended after failing two appropriate antiseizure ... ...

Abstract	Epilepsy surgery is successful in the majority of patients with tuberous sclerosis complex (TSC), with high rates of postoperative seizure reduction and even seizure freedom. Epilepsy surgery is recommended after failing two appropriate antiseizure medication trials; however, this is rare in clinical practice. We hypothesized that following surgery, caregivers' perspectives on the path they took to epilepsy surgery would inform changes in clinical practice and future research to increase utilization and early use of surgery. A questionnaire was developed to explore caregivers' perspectives on their child's path to epilepsy surgery. All 46 caregivers that filled out the majority of the survey were glad that their child underwent epilepsy surgery. Fourteen of 34 caregivers that commented on surgery timing wished their child had undergone epilepsy surgery earlier. Epilepsy with a duration of 23.5 months [interquartile range (IQR), 11.1 to 32.2 months, N = 14] prior to surgery was associated with caregiver dissatisfaction and was twice as long compared with caregivers who were satisfied with the timing of surgery (10 months, IQR, 7 to 17.3 months, p = 0.03). Caregivers were willing to accept a lower likelihood of seizure freedom and improvement than what they felt was likely from the preoperative discussions with their physicians. Forty caregivers rated various neurology physician factors as very important in their decision to undergo surgery: neurologist's attitude toward epilepsy surgery, experience with epilepsy surgery, and discussions around the risks of having and not having epilepsy surgery. Optimizing the caregiver-physician relationship can help facilitate early surgery referral and caregiver perception of surgery, potentially preventing delays to surgery and improved caregiver satisfaction.
MeSH term(s)	Adolescent ; Caregivers/psychology ; Child ; Child, Preschool ; Emotions ; Epilepsy/complications ; Epilepsy/etiology ; Epilepsy/psychology ; Epilepsy/surgery ; Female ; Humans ; Infant ; Male ; Surveys and Questionnaires ; Treatment Outcome ; Tuberous Sclerosis/complications ; Tuberous Sclerosis/psychology ; Tuberous Sclerosis/surgery
Language	English
Publishing date	2020-07-29
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.2020.107331
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Article ; Online: GNAO1-related neurodevelopmental disorder: Literature review and caregiver survey.

JoJo Yang, Qian-Zhou / Porter, Brenda E / Axeen, Erika T

Epilepsy & behavior reports

2022 Volume 21, Page(s) 100582

Abstract: Background: GNAO1: Methods: An online survey was distributed to caregivers of individuals diagnosed with : Results: Fifteen respondents completed the survey with the median age of 39 months, including a novel variant p.Q52P. Nine had epilepsy - ... ...

Abstract	Background: GNAO1 Methods: An online survey was distributed to caregivers of individuals diagnosed with Results: Fifteen respondents completed the survey with the median age of 39 months, including a novel variant p.Q52P. Nine had epilepsy - six had onset in the first week of life, three in the first year of life - but two reported no ongoing seizures. Seizure types varied. Individuals were taking a median of 3 seizure medications without a single best treatment. Our cohort was compared to a literature review of epilepsy in Conclusions: While
Language	English
Publishing date	2022-12-31
Publishing country	United States
Document type	Case Reports
ISSN	2589-9864
ISSN (online)	2589-9864
DOI	10.1016/j.ebr.2022.100582
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Article ; Online: Expanding eligibility for intracranial electroencephalography using Dexmedetomidine Hydrochloride in children with behavioral dyscontrol.

Johnstone, Thomas / Isabel Barros Guinle, Maria / Grant, Gerald A / Porter, Brenda E

Epilepsy & behavior : E&B

2023 Volume 150, Page(s) 109541

Abstract: Introduction: Invasive intracranial electroencephalography (IEEG) is advantageous for identifying epileptogenic foci in pediatric patients with medically intractable epilepsy. Patients with behavioral challenges due to autism, intellectual disabilities, ...

Abstract	Introduction: Invasive intracranial electroencephalography (IEEG) is advantageous for identifying epileptogenic foci in pediatric patients with medically intractable epilepsy. Patients with behavioral challenges due to autism, intellectual disabilities, and hyperactivity have greater difficulty tolerating prolonged IEEG recording and risk injuring themselves or others. There is a need for therapies that increase the safety of IEEG but do not interfere with IEEG recording or prolong hospitalization. Dexmedetomidine Hydrochloride's (DH) use has been reported to improve safety in patients with behavioral challenges during routine surface EEG recording but has not been characterized during IEEG. Here we evaluated DH administration in pediatric patients undergoing IEEG to assess its safety and impact on the IEEG recordings. Methods: A retrospective review identified all pediatric patients undergoing IEEG between January 2016 and September 2022. Patient demographics, DH administration, DH dose, hospital duration, and IEEG seizure data were analyzed. The number of seizures recorded for each patient was divided by the days each patient was monitored with IEEG. The total number of seizures, as well as seizures per day, were compared between DH and non-DH patients via summary statistics, multivariable linear regression, and univariate analysis. Other data were compared across groups with univariate statistics. Results: Eighty-four pediatric patients met the inclusion criteria. Eighteen (21.4 %) received DH treatment during their IEEG recording. There were no statistical differences between the DH and non-DH groups' demographic data, length of hospital stays, or seizure burden. Non-DH patients had a median age of 12.0 years (interquartile range: 7.25-15.00), while DH-receiving patients had a median age of 8.0 years old (interquartile range: 3.00-13.50) (p = 0.07). The non-DH cohort was 57.6 % male, and the DH cohort was 50.0 % male (p = 0.76). The median length of IEEG recordings was 5.0 days (interquartile range: 4.00-6.25) for DH patients versus 6.0 days (interquartile range: 4.00-8.00) for non-DH patients (p = 0.25). Median total seizures recorded in the non-DH group was 8.0 (interquartile range: 5.00-13.25) versus 15.0 in the DH group (interquartile range: 5.00-22.25) (p = 0.33). Median total seizures per day of IEEG monitoring were comparable across groups: 1.50 (interquartile range: 0.65-3.17) for non-DH patients compared to 2.83 (interquartile range: 0.89-4.35) (p = 0.25) for those who received DH. Lastly, non-DH patients were hospitalized for a median of 8.0 days (interquartile range: 6.00-11.25), while DH patients had a median length of stay of 7.00 days (interquartile range: 5.00-8.25) (p = 0.27). No adverse events were reported because of DH administration. Conclusions: Administration of DH was not associated with adverse events. Additionally, the frequency of seizures captured on the IEEG, as well as the duration of hospitalization, were not significantly different between patients receiving and not receiving DH during IEEG. Incorporating DH into the management of patients with behavioral dyscontrol and intractable epilepsy may expand the use of IEEG to patients who previously could not tolerate it, improve safety, and preserve epileptic activity during the recording period.
MeSH term(s)	Humans ; Male ; Child ; Female ; Electrocorticography ; Dexmedetomidine/therapeutic use ; Electroencephalography ; Drug Resistant Epilepsy/diagnosis ; Drug Resistant Epilepsy/drug therapy ; Seizures
Chemical Substances	Dexmedetomidine (67VB76HONO)
Language	English
Publishing date	2023-11-29
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.2023.109541
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ab Jg. 2022: Lesesaal (EG)

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Article ; Online: Connectivity increases during spikes and spike-free periods in self-limited epilepsy with centrotemporal spikes.

Goad, Beatrice S / Lee-Messer, Christopher / He, Zihuai / Porter, Brenda E / Baumer, Fiona M

Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology

2022 Volume 144, Page(s) 123–134

Abstract: Objective: To understand the impact of interictal spikes on brain connectivity in patients with Self-Limited Epilepsy with Centrotemporal Spikes (SeLECTS).: Methods: Electroencephalograms from 56 consecutive SeLECTS patients were segmented into ... ...

Abstract	Objective: To understand the impact of interictal spikes on brain connectivity in patients with Self-Limited Epilepsy with Centrotemporal Spikes (SeLECTS). Methods: Electroencephalograms from 56 consecutive SeLECTS patients were segmented into periods with and without spikes. Connectivity between electrodes was calculated using the weighted phase lag index. To determine if there are chronic alterations in connectivity in SeLECTS, we compared spike-free connectivity to connectivity in 65 matched controls. To understand the acute impact of spikes, we compared connectivity immediately before, during, and after spikes versus baseline, spike-free connectivity. We explored whether behavioral state, spike laterality, or antiseizure medications affected connectivity. Results: Children with SeLECTS had markedly higher connectivity than controls during sleep but not wakefulness, with greatest difference in the right hemisphere. During spikes, connectivity increased globally; before and after spikes, left frontal and bicentral connectivity increased. Right hemisphere connectivity increased more during right-sided than left-sided spikes; left hemisphere connectivity was equally affected by right and left spikes. Conclusions: SeLECTS patient have persistent increased connectivity during sleep; connectivity is further elevated during the spike and perispike periods. Significance: Testing whether increased connectivity impacts cognition or seizure susceptibility in SeLECTS and more severe epilepsies could help determine if spikes should be treated.
MeSH term(s)	Child ; Humans ; Epilepsy, Rolandic ; Electroencephalography ; Seizures ; Brain ; Functional Laterality/physiology
Language	English
Publishing date	2022-10-07
Publishing country	Netherlands
Document type	Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
ZDB-ID	1463630-x
ISSN	1872-8952 ; 0921-884X ; 1388-2457
ISSN (online)	1872-8952
ISSN	0921-884X ; 1388-2457
DOI	10.1016/j.clinph.2022.09.015
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Article ; Online: Clinical and electrographic features of sunflower syndrome.

Baumer, Fiona M / Porter, Brenda E

Epilepsy research

2018 Volume 142, Page(s) 58–63

Abstract: Background: Sunflower Syndrome describes reflex seizures - typically eyelid myoclonia with or without absence seizures - triggered when patients wave their hands in front of the sun. While valproate has been recognized as the best treatment for ... ...

Abstract	Background: Sunflower Syndrome describes reflex seizures - typically eyelid myoclonia with or without absence seizures - triggered when patients wave their hands in front of the sun. While valproate has been recognized as the best treatment for photosensitive epilepsy, many clinicians now initially treat with newer medications; the efficacy of these medications in Sunflower Syndrome has not been investigated. We reviewed all cases of Sunflower Syndrome seen at our institution over 15 years to describe the clinical course, electroencephalogram (EEG), and treatment response in these patients. Methods: Search of the electronic medical record and EEG database, as well as survey of epilepsy providers at our institution, yielded 13 cases of Sunflower Syndrome between 2002 and 2017. We reviewed the records and EEG tracings. Results: Patients were mostly young females, with an average age of onset of 5.5 years. Seven had intellectual, attentional or academic problems. Self-induced seizures were predominantly eyelid myoclonia ± absences and 6 subjects also had spontaneous seizures. EEG demonstrated a normal background with 3-4 Hz spike waves ± polyspike waves as well as a photoparoxysmal response. Based on both clinical and EEG response, valproate was the most effective treatment for reducing or eliminating seizures and improving the EEG; 9 patients tried valproate and 66% had significant improvement or resolution of seizures. None of the nine patients on levetiracetam or seven patients on lamotrigine monotherapy achieved seizure control, though three patients had improvement with polypharmacy. Conclusions: Valproate monotherapy continues to be the most effective treatment for Sunflower Syndrome and should be considered early. For patients who cannot tolerate valproate, higher doses of lamotrigine or polypharmacy should be considered. Levetiracetam monotherapy, even at high doses, is unlikely to be effective.
MeSH term(s)	Adolescent ; Anticonvulsants/therapeutic use ; Child ; Cost of Illness ; Electroencephalography/methods ; Electroencephalography/statistics & numerical data ; Electronic Health Records/statistics & numerical data ; Epilepsy, Reflex/physiopathology ; Epilepsy, Reflex/therapy ; Female ; Humans ; Male ; Retrospective Studies ; Treatment Outcome
Chemical Substances	Anticonvulsants
Language	English
Publishing date	2018-03-03
Publishing country	Netherlands
Document type	Journal Article ; Research Support, N.I.H., Extramural
ZDB-ID	632939-1
ISSN	1872-6844 ; 0920-1211
ISSN (online)	1872-6844
ISSN	0920-1211
DOI	10.1016/j.eplepsyres.2018.03.002
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Article ; Online: Antiseizure medication use and medical resource utilization after resective epilepsy surgery in children in the United States: A contemporary nationwide cross-sectional cohort analysis.

Parker, Jonathon J / Zhang, Yi / Fatemi, Parastou / Halpern, Casey H / Porter, Brenda E / Grant, Gerald A

Epilepsia

2022 Volume 63, Issue 4, Page(s) 824–835

Abstract: Objective: Antiseizure drug (ASD) therapy can significantly impact quality of life for pediatric patients whose epilepsy remains refractory to medications and who experience neuropsychological side effects manifested by impaired cognitive and social ... ...

Abstract	Objective: Antiseizure drug (ASD) therapy can significantly impact quality of life for pediatric patients whose epilepsy remains refractory to medications and who experience neuropsychological side effects manifested by impaired cognitive and social development. Contemporary patterns of ASD reduction after pediatric epilepsy surgery across practice settings in the United States are sparsely reported outside of small series. We assessed timing and durability of ASD reduction after pediatric epilepsy surgery and associated effects on health care utilization. Methods: We performed a retrospective analysis of 376 pediatric patients who underwent resective epilepsy surgery between 2007 and 2016 in the United States using the Truven MarketScan database. Filled ASD prescriptions during the pre- and postoperative periods were compared. Univariate and multivariate analyses identified factors associated with achieving a stable discontinuation of or reduction in number of ASDs. Health care utilization and costs were systematically compared. Results: One hundred seventy-one patients (45.5%) achieved a >90-day ASD-free period after surgery, and 84 (22.3%) additional patients achieved a stable reduction in number of ASDs. Achieving ASD freedom was more common in patients undergoing total hemispherectomy (n = 21, p = .002), and less common in patients with tuberous sclerosis (p = .003). A higher number of preoperative ASDs was associated with a greater likelihood of achieving ASD reduction postoperatively (hazard ratio [HR]: 1.85, 95% confidence interval [CI]: 1.50-2.28), but was not associated with a significant difference in the likelihood of achieving ASD freedom (0.83, 95% CI: 0.49-1.39). Achieving an ASD-free period was associated with fewer hospital readmissions within the first year after surgery. Significance: Patterns of ASD use and discontinuation after pediatric epilepsy surgery provide an unbiased surgical outcome endpoint extractable from administrative databases, where changes in seizure frequency are not captured. This quantitative measure can augment traditional surgical outcome scales, incorporating a significant clinical parameter associated with improved quality of life.
MeSH term(s)	Child ; Cohort Studies ; Cross-Sectional Studies ; Epilepsy/drug therapy ; Epilepsy/surgery ; Humans ; Quality of Life ; Retrospective Studies ; Treatment Outcome ; United States
Language	English
Publishing date	2022-02-25
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.17180
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