bluuebunny/crossref_metadata_2025_split huggingface dataset:
Quality of life after vagal nerve stimulator insertion
Authors: Mikati Mohamad A. , Ataya Nour F. , El‐Ferezli Jessica C. , Baghdadi Tarafa S. , Turkmani Ali H. , Comair Youssef G. , Kansagra Sujay , Najjar Marwan W. 
Published: August 2019

ABSTRACTAimAssess quality‐of‐life after vagal nerve stimulation and determine patient characteristics associated with improvement in quality‐of‐life.MethodsSixteen patients (11 children, 5 adults) who had vagal nerve stimulation at our center were studied. Quality‐of‐life was assessed pre‐ and post‐vagal nerve stimulation using the Quality‐of‐Life in Childhood Epilepsy questionnaire for children and the Epilepsy Surgery Inventory‐55 for adults.ResultsSixteen patients who did not qualify for resective surgery were included; seven (43.75%) were males and 9 (56.25%) were females. Mean age at onset of seizures was 3.96 ± 4.00 years and at surgery was 15.78 ± 10.78. Follow‐up time was 1.26 ± 0.92 years. Fourteen patients (87.5%) were mentally retarded. Ten (62.5%) had cryptogenic etiology and 6 patients (37.5%) symptomatic etiology. Fifty percent had localization‐related epilepsy. Six of 7 patients with generalized cryptogenic etiology (85.71%) had Lennox‐Gastaut syndrome. Seizures dropped from 122.31 ± 159.49 to 67.84 ± 88.22 seizures/month. Seizure reduction (> 50%) correlated with improvement in total quality‐of‐life (p = 0.034). Post‐vagal nerve stimulation, the total group scored significantly higher in the social domain (p = 0.039). In patients with localization‐related epilepsy, significant improvements were detected in the social domain (p = 0.049) and in total quality‐of‐life (p = 0.042).ConclusionDespite a diverse and small population size, we observed significant improvements in the social domain 1.26 years post‐vagal nerve stimulation. In addition, there was an improvement in total quality‐of‐life amongst patients with partial seizures. Finally, seizure reduction was associated with quality‐of‐life improvement. Our results support previous studies from the West reporting improvement in quality‐of‐life following vagal nerve stimulation, contradict those studies that did not show such differences, and are the first coming from a developing country.
Anatomo‐electro‐clinical correlations: the Freiburg, Germany Case Report Case 02‐2009: Functional hemispherectomy in adults with intractable epilepsy syndromes: a report of 4 cases
Authors: Steinhoff Bernhard J. , Staack Anke Maren , Bilic Sofia , Kraus Uwe , Schulze‐Bonhage Andreas , Zentner Josef 
Published: August 2019

ABSTRACT[Case records of Epileptic Disorders. Anatomo‐electro‐clinical correlations. Case 02‐2009]BackgroundFunctional hemispherectomy (FH) is mainly performed in children with catastrophic epilepsies and preexisting severe hemiparesis. It is accepted that mainly patients at young ages with more or less complete loss of their hand function are suitable candidates for FH. However, we feel that FH should be considered also in some adults with devastating unilateral epilepsy syndromes.Patients and methodsWe report four cases of adults who underwent FH because of their intractable epilepsy syndromes. Three of the four patients had reduced but not complete loss of hand function. Careful weighing‐up of the risk‐benefit ratio still led to the decision to perform FH in order to achieve complete seizure relief.ResultsAll patients had a surgical outcome of Engel class I; one of them suffered from post‐operative psychogenic non‐epileptic seizures which were treated successfully by psychotherapy. No clinically relevant complications occurred.ConclusionWe wish to point out that FH is a possible surgical option in cases with catastrophic epilepsies in adulthood. Patients and relatives should strongly consider that the likelihood of at least partially, impaired hand function or other surgery‐related additional handicaps may be outweighed by the chance of freedom of seizures in a limited number of patients. In all of our cases, patients and relatives agreed that the surgical outcome led to a marked improvement of quality of life and that it had been the right decision to proceed with FH in spite of the expected negative impacts of the operation. Video examples of the pre‐ and post‐surgical hand function of one sample patient are enclosed. [Published with video sequences]
Paradoxical ictal EEG lateralization in children with unilateral encephaloclastic lesions
Authors: Garzon Eliana , Gupta Ajay , Bingaman William , Sakamoto Americo C. , Lüders Hans 
Published: August 2019

ABSTRACTBackgroundDescribe an ictal EEG pattern of paradoxical lateralization in children with unilateral encephaloclastic hemispheric lesion acquired early in life.MethodsOf 68 children who underwent hemispherectomy during 2003‐2005, scalp video‐EEG and brain MRI of six children with an ictal scalp EEG pattern discordant to the clinical and imaging data were reanalyzed. Medical charts were reviewed for clinical findings and seizure outcome.ResultsAge of seizure onset was 1 day‐4 years. The destructive MRI lesion was an ischemic stroke in 2, a post‐infectious encephalomalacia in 2, and a perinatal trauma and hemiconvulsive‐hemiplegic syndrome in one patient each. Ictal EEG pattern was characterized by prominent ictal rhythms with either 3‐7 Hz spike and wave complexes or beta frequency sharp waves (paroxysmal fast) over the unaffected (contralesional) hemisphere. Scalp video‐EEG was discordant, however, other findings of motor deficits (hemiparesis; five severe, one mild), seizure semiology (4/6), interictal EEG abnormalities (3/6), and unilateral burden of MRI lesion guided the decision for hemispherectomy. After 12‐39 months of post‐surgery follow up, five of six patients were seizure free and one has brief staring spells.ConclusionWe describe a paradoxical lateralization of the EEG to the “good” hemisphere in children with unihemispheric encephaloclastic lesions. This EEG pattern is compatible with seizure free outcome after surgery, provided other clinical findings and tests are concordant with origin from the abnormal hemisphere.
Selection of ideal candidates for extratemporal resective epilepsy surgery in a country with limited resources
Authors: Chaudhry Neera , Radhakrishnan Ashalatha , Abraham Mathew , Kesavadas Chandrasekharan , Radhakrishnan Venkataraman V. , Sarma P. Sankara , Radhakrishnan Kurupath 
Published: August 2019

ABSTRACTAimTo investigate how to select ideal candidates for extratemporal resective epilepsy surgery, without compromising efficacy and safety, in countries with limited pre‐surgical diagnostic facilities.MethodFrom the prospective database maintained at an epilepsy surgery centre in southern India, we reviewed the attributes of consecutive patients who had completed at least two years of follow‐up after resections involving frontal, parietal and occipital lobes for medically refractory focal seizures.ResultsOf 386 patients diagnosed with extratemporal refractory epilepsies during the study period, 61 (15.8%) were selected based on the presence of magnetic resonance imaging (MRI)‐identified lesions (in all) and concordant scalp recorded electroencephalographic (EEG) data (in nearly two thirds). Seventeen (27.8%) required invasive investigations either to define the ictal onset zone, eloquent area, or both. During a median follow‐up period of five years, 63% of our patients were seizure‐free, excluding the presence of auras. Permanent disabling neurological sequelae occurred in three (4.9%) patients. According to univariate analysis, pre‐operative secondary generalised seizures and interictal epileptiform discharges (IEDs), during a one‐year post‐operative EEG monitoring period, portended unfavourable seizure outcome. In multivariate analysis, frontal lobe resections and IEDs in post‐operative EEGs were independent predictors of unfavourable outcome.ConclusionsExtratemporal resective epilepsy surgery can be undertaken in countries with limited resources with efficacy and safety, comparable to that in developed countries, when patients are selected based on the presence of MRI‐identified lesions and scalp EEG concordance. In such patients, invasive EEG examinations, when necessary, can be undertaken by limited coverage of cortical areas at an affordable cost.
The evolution of antiepileptic drug development and regulation
Authors: Arzimanoglou Alexis , Ben‐Menachem Elinor , Cramer Joyce , Glauser Tracy , Seeruthun Rav , Harrison Miranda 
Published: August 2019

ABSTRACTThe early years of antiepileptic drug development were characterised by observation and serendipity, rather than a rational, targeted approach to drug development. Control of seizures was seen as the primary aim of therapy, with much less focus on safety and tolerability. However, experience with thalidomide in the 1960s brought safety to the fore, resulting in an era of much tighter regulatory control that still persists today. A direct consequence of this was an increased emphasis on the importance of evidence from randomised controlled trials. Despite the continuing reliance on randomised controlled trials for regulatory approval and the formulation of evidence‐based guidelines, the modern era has seen an increasing acknowledgment of their limitations and the need for complementary sources of ‘real‐world’ evidence. Such sources include registries and studies that are designed to provide a much broader assessment of a drug's overall effectiveness; for example, by incorporating patient‐reported outcomes to assess the effects of treatment on quality of life or functional status. Such changes reflect a more patient‐centric approach to treatment, since it is now recognised that epilepsy can only be effectively managed if patients' individual real‐life needs are addressed, since a key to successful treatment is long‐term compliance. Alongside these changes in approach, the modern era has witnessed important advances in antiepileptic drugs themselves, either through development of novel molecules, or targeted, structural improvements of older agents. [Published with video sequences and a set of slides]
A seizure response dog: video recording of reacting behaviour during repetitive prolonged seizures
Authors: Di Vito Lidia , Naldi Ilaria , Mostacci Barbara , Licchetta Laura , Bisulli Francesca , Tinuper Paolo 
Published: August 2019

ABSTRACTSeizure response and alerting behaviour may spontaneously develop in dogs living with children or adults with epilepsy. Some dogs can also be reliably trained to respond and anticipate seizures. We describe the case of a dog, not previously trained for assistance work, showing complex seizure response behaviour. This is the first release of a home video recording of a dog reacting to its owner's seizure. [Published with video sequences]
Evolution and management of Lennox‐Gastaut syndrome through adolescence and into adulthood: are seizures always the primary issue?
Authors: Kerr Michael , Kluger Gerhard , Philip Sunny 
Published: August 2019

ABSTRACTAlthough Lennox‐Gastaut syndrome (LGS) typically begins during childhood, it frequently persists through adolescence and on into adulthood. It may also, rarely, have late onset during adolescence or adulthood. Longitudinal studies have highlighted that the “typical” features of LGS observed during childhood evolve and change over time, so that by adulthood it might be difficult to recognise LGS in previously undiagnosed patients. Approaches to treatment must therefore adapt to the changes in a patient's condition as they progress through life. Effective management of LGS requires a global approach to care that not only encompasses seizure control, but also the management of co‐morbidities associated with the condition, such as cognitive and behavioural problems, sleep disturbances and physical disability, together with the specific educational and psychosocial needs of the individual. This is particularly relevant during adolescence, when patients have to cope with a host of additional issues alongside those relating to their epilepsy. During all stages of life, management of LGS must carefully balance the need for treatment against its side effects, with the patient's overall quality of life always being the primary focus. The transition of care from paediatric to adult services presents important challenges for patients, their families and healthcare providers, and requires particular consideration to ensure that it is as smooth as possible. It also presents an important opportunity to review and reappraise a patient's condition, treatment and other longer‐term needs as they journey into adulthood.
Epilepsy in adult patients with Down syndrome: a clinical‐video EEG study
Authors: Vignoli Aglaia , Zambrelli Elena , Chiesa Valentina , Savini Miriam , Briola Francesca La , Gardella Elena , Canevini Maria Paola 
Published: August 2019

ABSTRACTPatients with Down syndrome are now living longer and the overall prevalence of epilepsy is increasing, however, full characterisation of epilepsy in adult age is still incomplete. We describe the electroclinical characteristics of epilepsy in 22 adult patients with Down syndrome (11 males, 11 females), with a mean age of 46 years (range: 28‐64 years), followed at the Epilepsy Centre, San Paolo Hospital in Milan. Mean age at epilepsy onset was 36.8 years (range: 6‐60 years). Nine out of 22 patients had focal epilepsy, while nine had late‐onset myoclonic epilepsy. In four patients, epilepsy was unclassified. The EEG pattern of our patients was characterised by a progressive slowing of the background activity with sharp‐and‐slow waves with frontal predominance. In the patients diagnosed with late‐onset myoclonic epilepsy, the EEGs showed generalised polyspike waves. Three subjects had an episode of myoclonic status epilepticus at the beginning or in the course of the disorder. After the first descriptions of late‐onset myoclonic epilepsy by Genton and Paglia (1994), this is one of the largest patient cohorts reported. Our data confirm that epilepsy in adult patients with Down syndrome presents peculiar electroclinical characteristics which should be recognized early as prompt, effective treatment may be beneficial. [Published with video sequences]
Side effects of phenobarbital in epilepsy: a systematic review
Authors: Zhang Ling‐Li , Zeng Li‐Nan , Li You‐Ping 
Published: August 2019

ABSTRACTAimIn recent years, phenobarbital, as an antiepileptic drug, has become less popular based on adverse events, especially cognitive and behavioural side effects. Despite the development of better tolerated new generation AEDs, phenobarbital is still widely used particularly in developing countries because of its low cost. The purpose of this review was to: (i) investigate whether phenobarbital can be safely used as an antiepileptic drug and (ii) determine the questions which need to be addressed in order to comprehensively and adequately evaluate the safety of phenobarbital for the treatment of epilepsy.MethodsThe literature was searched using the Cochrane Central Register of randomised controlled trials (1800‐2009), Medline (1966‐2009), Embase (1966‐2009) and three Chinese databases.ResultsTwenty studies were finally included in this systematic review. The determination of adverse effects of combined antiepileptic drugs (AEDs) from different studies was complicated by numerous factors including study design, different descriptions of adverse events and a lack of standardised data collection. These factors may also have been responsible for the heterogeneity present in the meta‐analysis. The data did not demonstrate any evidence of association between phenobarbital and a higher risk of adverse events. However, phenobarbital appeared to be associated with a higher rate of adverse drug reaction related withdrawal (ADR‐related withdraw), compared to carbamazepine, valproic acid and phenytoin. This may have been due to a concern for possible adverse effects of phenobarbital.ConclusionsPhenobarbital was associated with a higher rate of drug withdrawal although there was no evidence to suggest that phenobarbital caused more adverse events compared to carbamazepine, valproic acid or phenytoin. However, in the case of pregnant women, it is important for clinicians to evaluate the benefits and risks of phenobarbital administration before making a final recommendation. Furthermore, unified scales for the assessment of cognitive function should be applied for future studies particularly in children.
Confusion and SIRPIDs regress with parenteral lorazepam
Authors: Kaplan Peter W. , Duckworth Josh 
Published: August 2019

ABSTRACTStimulus‐induced rhythmic, periodic or ictal discharges (SIRPIDs) are EEG epileptiform periodic discharges (PD) induced by arousal. SIRPIDs lie along an ictal‐interictal continuum with debate regarding urgency of treatment. In a patient with SIRPIDs, aphasia and confusion, i.v. lorazepam during EEG resulted in improved level of consciousness, return of verbal interaction and regression of SIRPIDs. This suggests that some forms of SIRPIDs may be associated with an ictal confusional state.
Montreal Cognitive Assessment in cryptogenic epilepsy patients with normal Mini‐Mental State Examination scores
Authors: Phabphal Kanitpong , Kanjanasatien Janyapon 
Published: August 2019

ABSTRACTThis cross‐sectional study examined the Montreal Cognitive Assessment (MoCA) performance in cryptogenic epileptic patients aged more than 15 years with normal global cognition according to the Mini‐Mental State Examination (MMSE) score. We tested our hypothesis that the prevalence of mild cognitive impairment and associated patient correlation factors might be increased (score < 26) according to the MoCA, in spite of a normal MMSE score, and that cognitive impairment might occur in a range of domains of the MoCA. Eighty‐five patients participated in this study. The mean MoCA score was 22.44 (± 4.32). In spite of a normal MMSE score, which was an inclusion criterion, cognitive impairment was detected in 60% patients based on the MoCA score. The variable that correlated with a higher risk of cognitive impairment was the number of antiepileptic drugs (polytherapy: OR 2.71; CI 1.03‐7.15). The mean scores of visuospatial and executive function, naming ability, attention, language, abstraction, delayed recall and orientation among patients with mild cognitive impairment were significantly lower than those of patients with normal cognitive function. These findings suggest that mild cognitive impairment in cryptogenic epileptic patients is common. We suggest using MoCA as a screening test for patients with epilepsy.
Reflex seizures in Rett syndrome
Authors: Martínez Ana Roche , Colmenero M Itziar Alonso , Pereira Andreia Gomes , Vilaplana Francesc X Sanmartí , Morón Judith Armstrong , Marfa Mercé Pineda 
Published: August 2019

ABSTRACTReflex seizures are a rare phenomenon among epileptic patients, in which an epileptic discharge is triggered by various kinds of stimuli (visual, auditory, tactile or gustatory). Epilepsy is common in Rett syndrome patients (up to 70%), but to the authors' knowledge, no pressure or eating‐triggered seizures have yet been reported in Rett children. We describe three epileptic Rett patients with reflex seizures, triggered by food intake or proprioception. One patient with congenital Rett Sd. developed infantile epileptic spasms at around seven months and two patients with classic Rett Sd. presented with generalised tonic‐clonic seizures at around five years. Reflex seizures appeared when the patients were teenagers. The congenital‐Rett patient presented eating‐triggered seizures at the beginning of almost every meal, demonstrated by EEG recording. Both classic Rett patients showed self‐provoked pressure ‐triggered attacks, influenced by stress or excitement. Non‐triggered seizures were controlled with carbamazepine or valproate, but reflex seizures did not respond to antiepileptic drugs. Risperidone partially improved self‐provoked seizures. When reflex seizures are suspected, reproducing the trigger during EEG recording is fundamental; however, self‐provoked seizures depend largely on the patient's will. Optimal therapy (though not always possible) consists of avoiding the trigger. Stress modifiers such as risperidone may help control self‐provoked seizures.
Patients with generalised epilepsy have a higher white blood cell count than patients with focal epilepsy
Authors: Sarkis Rani A , Jehi Lara , Silveira Diosely , Janigro Damir , Najm Imad 
Published: August 2019

ABSTRACTBackground. Immunological alterations have been noted following seizures in the acute period, however little is known about the effect of type and severity of epilepsy on leukocyte count in the absence of seizures. Methods. We performed a retrospective chart review of adult epilepsy patients presenting for evaluation over a four‐month period. Demographics, epilepsy type and characteristics, number and type of antiepileptic drugs, as well as medical co‐morbidities were noted. Results. A total of 241 patients fulfilled study criteria. Variables correlating with leukocyte count were identified using univariate analysis. Based on multivariate analysis, only the correlation with type of epilepsy and use of more than two antiepileptic drugs remained statistically significant. Patients with generalised epilepsy had a higher leukocyte count (7.21 k/μL) compared to those with focal epilepsy (6.53 k/μL); the main difference was due to the number of monocytes. Conclusion. These findings raise the possibility that there are different neuro‐immune profiles between patients with generalised and focal epilepsy.
Late‐onset Lennox‐Gastaut syndrome as a phenotype of 15q11.1q13.3 duplication
Authors: Rocha João , Guerra Cátia , Oliveira Renata , Dória Sofia , Rego Ricardo , Rosas Maria José 
Published: August 2019

ABSTRACTThe clinical symptoms associated with chromosome 15q duplication syndrome manifest through a heterogeneous group of symptoms characterised by hypotonia, delay in motor skills and language development, cognitive and learning disabilities, autism spectrum disorder and refractory epilepsy. The late development of Lennox‐Gastaut syndrome in patients with 15q11q13 duplication is a possibility that physicians should be aware of. We report the case of a 27‐year‐old man with a neurodevelopmental syndrome due to a 15q duplication, with intellectual disability, psychiatric disturbances, and an epileptic phenotype diagnosed as late‐onset Lennox‐Gastaut syndrome.