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Find video protocols related to scientific articles indexed in Pubmed.
Academic achievement in school-aged children with active epilepsy: A population-based study.
Epilepsia
PUBLISHED: 09-04-2014
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To provide population-based data on the performance of school-aged children with epilepsy on measures of academic achievement and factors associated with this performance after controlling for IQ.
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Pathological Demand Avoidance in a population-based cohort of children with epilepsy: Four case studies.
Res Dev Disabil
PUBLISHED: 08-29-2014
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Childhood epilepsy is associated with a range of neurobehavioural comorbidities including Attention-Deficit/Hyperactivity Disorder (ADHD), Autism Spectrum Disorder (ASD), motor impairments and emotional problems. These difficulties frequently have a greater impact on quality of life than seizures. Pathological Demand Avoidance (PDA) is a term increasingly in use in the UK and Europe to describe behaviours associated with an extreme resistance to demands and requests and the need to be in control in social interactions. In a population-based group of 85 children with epilepsy, four (5%) were identified as displaying significant symptoms of PDA, were assessed using the Extreme Demand Avoidance Questionnaire (EDA-Q) and are described in detail. As well as significant symptoms of PDA, the four children met criteria for a range of neurobehavioural disorders; all four had cognitive impairment (IQ<85) and met DSM-IV-TR criteria for ADHD. Three, in addition, met criteria for ASD and Developmental Coordination Disorder (DCD) and two for Oppositional Defiant Disorder (ODD). All four experienced their first seizure before 5 years of age. School and parent reports indicated very significant functional impairment and management concerns, particularly with respect to complying with everyday demands. Symptoms of PDA should be considered when evaluating neurobehavioural comorbidity in childhood epilepsy.
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A girl with tuberous sclerosis complex presenting with severe epilepsy and electrical status epilepticus during sleep, and with high-functioning autism and mutism.
Cogn Behav Neurol
PUBLISHED: 06-27-2014
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Most patients with tuberous sclerosis complex (TSC) suffer from epilepsy, and many have cognitive and behavioral problems like severe intellectual disability, autism, and hyperactivity. Only rare patients with TSC and autism have a normal intelligence quotient. We report a 13-year-old girl with definite TSC who had early-onset severe epilepsy, autistic behavior, and moderate developmental delay. By school age, however, she had normal intelligence; her intelligence quotient was at least 70 based on a Stanford-Binet test that she refused to complete. She showed good reading, writing, and language comprehension skills, and the special abilities of hyperlexia, hypermnesia, and hypercalculia. However, she did not speak. Criteria of the Diagnostic and Statistical Manual of Mental Disorders, 4th edition, and her Childhood Autism Rating Scale score of 36 indicated mild to moderate autism. She had severe electroencephalographic abnormalities: hypsarrhythmia, multifocal or generalized epileptiform discharges, and electrical status epilepticus during sleep, with a continuous left temporal focus. Magnetic resonance imaging showed many cortical tubers in all brain lobes, and subependymal nodules. We discuss possible explanations for her lack of speech. Considered as speech apraxia, her mutism could be either a symptom of her TSC or a component of her autism. Another possibility is that long-lasting electrical status epilepticus during sleep led to her autistic behavior and language arrest. Still another possibility is that a disinhibited mammalian target of rapamycin (mTOR) pathway was at the root of all of her neuropsychiatric symptoms.
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Neurobehavioral comorbidities in children with active epilepsy: a population-based study.
Pediatrics
PUBLISHED: 05-28-2014
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In addition to recurrent epileptic seizures, children with epilepsy can have coexisting cognitive and behavioral difficulties but the spectrum and prevalence of such difficulties are uncertain.
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A novel SLC2A1 mutation linking hemiplegic migraine with alternating hemiplegia of childhood.
Cephalalgia
PUBLISHED: 05-15-2014
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Hemiplegic migraine (HM) and alternating hemiplegia of childhood (AHC) are rare episodic neurological brain disorders with partial clinical and genetic overlap. Recently, ATP1A3 mutations were shown to account for the majority of AHC patients. In addition, a mutation in the SLC2A1 gene was reported in a patient with atypical AHC. We therefore investigated whether mutations in these genes may also be involved in HM. Furthermore, we studied the role of SLC2A1 mutations in a small set of AHC patients without ATP1A3 mutations.
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Distinct neurological disorders with ATP1A3 mutations.
Lancet Neurol
PUBLISHED: 04-18-2014
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Genetic research has shown that mutations that modify the protein-coding sequence of ATP1A3, the gene encoding the ?3 subunit of Na(+)/K(+)-ATPase, cause both rapid-onset dystonia parkinsonism and alternating hemiplegia of childhood. These discoveries link two clinically distinct neurological diseases to the same gene, however, ATP1A3 mutations are, with one exception, disease-specific. Although the exact mechanism of how these mutations lead to disease is still unknown, much knowledge has been gained about functional consequences of ATP1A3 mutations using a range of in-vitro and animal model systems, and the role of Na(+)/K(+)-ATPases in the brain. Researchers and clinicians are attempting to further characterise neurological manifestations associated with mutations in ATP1A3, and to build on the existing molecular knowledge to understand how specific mutations can lead to different diseases.
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Screening, intervention and outcome in autism and other developmental disorders: the role of randomized controlled trials.
J Autism Dev Disord
PUBLISHED: 02-21-2014
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We draw attention to a number of important considerations in the arguments about screening and outcome of intervention in children with autism and other developmental disorders. Autism screening in itself never provides a final clinical diagnosis, but may well identify developmental deviations indicative of autism-or of other developmental disorders-that should lead to referral for further clinical assessment. Decisions regarding population or clinic screening cannot be allowed to be based on the fact that prospective longitudinal RCT designs over decades could never be performed in complex developmental disorders. We propose an alternative approach. Early screening for autism and other developmental disorders is likely to be of high societal importance and should be promoted and rigorously evaluated.
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Premature mortality in active convulsive epilepsy in rural Kenya: causes and associated factors.
Neurology
PUBLISHED: 01-17-2014
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We estimated premature mortality and identified causes of death and associated factors in people with active convulsive epilepsy (ACE) in rural Kenya.
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Temporal lobe impairment in West syndrome: Event-related potential evidence.
Ann. Neurol.
PUBLISHED: 01-13-2014
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Objectives: This study investigates auditory processing in infants with West syndrome (WS) using event-related potentials (ERPs). Methods: ERPs were measured in 25 infants with mainly symptomatic WS (age range 3-14 months) and 26 healthy term infants (age range 3-14 months) using an auditory novelty oddball paradigm. The ERP recordings were made during wakefulness and repeated in stage II sleep. Results: The obligatory components (P150, N250, and P350) and novelty response components (P300, Nc) were recordable during both sleep and wakefulness in patients and controls. All ERP latencies decreased with age in controls but not in the WS group [Age x group interaction F = 22.3, p < 0.0001]. These ERP latency alterations were not affected by pharmacological treatment for WS. Conclusions: This study demonstrated a persistently altered ERP signature in patients with a recent history of infantile spasms. The prolongation of auditory obligatory and novelty ERP in WS patients indicates a severe failure of temporal lobe maturation during infancy. It remains to be investigated if this predicts long-term cognitive impairments characteristic for this epileptic encephalopathy. ANN NEUROL 2014. © 2014 American Neurological Association.
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Intellectual development before and after the onset of infantile spasms: a controlled prospective longitudinal study in tuberous sclerosis.
Epilepsia
PUBLISHED: 01-13-2014
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Infantile spasms (IS) have long been suspected to be a risk factor for impairment in intellectual development, but there are no controlled, prospective longitudinal data in well-characterized conditions to confirm this suspicion. We tested the hypothesis in a longitudinal study of children with tuberous sclerosis (TS), who have a high risk of developing IS.
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Methylphenidate treatment of attention deficit hyperactivity disorder in young people with learning disability and difficult-to-treat epilepsy: Evidence of clinical benefit.
Epilepsia
PUBLISHED: 08-30-2013
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To establish the efficacy and safety of methylphenidate (MPH) treatment for attention deficit hyperactivity disorder (ADHD) in a group of children and young people with learning disability and severe epilepsy.
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Clinical features, proximate causes, and consequences of active convulsive epilepsy in Africa.
Epilepsia
PUBLISHED: 08-26-2013
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Epilepsy is common in sub-Saharan Africa (SSA), but the clinical features and consequences are poorly characterized. Most studies are hospital-based, and few studies have compared different ecological sites in SSA. We described active convulsive epilepsy (ACE) identified in cross-sectional community-based surveys in SSA, to understand the proximate causes, features, and consequences.
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Pediatric neurology: the diagnostic process.
Handb Clin Neurol
PUBLISHED: 04-30-2013
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Pediatric neurology comprises a very large of number of conditions exhibiting symptoms and signs in several functional domains arising from damage and dysfunction to the developing nervous system. The diagnostic process involves ensuring that data from all possible domains are sought including those that are unaffected. The subsequent analysis involves fitting these data into patterns of classical natural history and rigorous investigation of the aspects that do not appear to fit. There may be a pattern of illness that is immediately recognized or something that is a fairly close fit. However, the aim is to develop a pathogenic sequence for the condition particularly so that conditions that have been lumped together for convenience are separated into distinct disease entities. The major presentations of pediatric neurology of fixed central motor impairments (the cerebral palsies), the epilepsies, and the progressive degenerative diseases are in the process of being split into such pathogenic sequences so that definitive treatments and possible primary prevention can be added to aims of simple diagnostic recognition. Much of this is at an early stage and pediatric neurology is still a young and fast developing specialty.
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Early developmental outcomes in children following convulsive status epilepticus: a longitudinal study.
Epilepsia
PUBLISHED: 01-28-2013
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Convulsive status epilepticus (CSE) is the most common pediatric neurologic emergency and is often associated with unfavorable neurodevelopmental outcomes. The early developmental trajectory of children following CSE has not been previously investigated, leaving a gap in our understanding of how these adverse long-term outcomes emerge.
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Behavioural comorbidity in Tanzanian children with epilepsy: a community-based case-control study.
Dev Med Child Neurol
PUBLISHED: 11-19-2011
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The aim of this study was to define the prevalence of and risk factors for behavioural disorders in children with epilepsy from a rural district of Tanzania by conducting a community-based case-control study.
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Death within 8 years after childhood convulsive status epilepticus: a population-based study.
Brain
PUBLISHED: 09-13-2011
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The risk of long-term mortality and its predictors following convulsive status epilepticus in childhood are uncertain. We report mortality within 8 years after an episode of convulsive status epilepticus, and investigate its predictors from a paediatric, prospective, population-based study from north London, UK. In the current study, we followed-up a cohort previously ascertained during a surveillance study of convulsive status epilepticus in childhood. After determining the survival status of the cohort members, we defined cause of death as that listed on their death certificates. We estimated a standardized mortality ratio to compare mortality in our cohort with that expected in the reference population. Multivariable Cox regression analysis was used to investigate any association between the clinical and demographic factors at the time of status epilepticus and subsequent risk of death. The overall case fatality was 11% (95% confidence interval 7.5-16.2%); seven children died within 30 days of their episode of convulsive status epilepticus and 16 during follow-up. The overall mortality in our cohort was 46 times greater than expected in the reference population, and was predominantly due to higher mortality in children who had pre-existing clinically significant neurological impairments when they had their acute episode of convulsive status epilepticus. Children without prior neurological impairment who survived their acute episode of convulsive status epilepticus were not at a significantly increased risk of death during follow-up. There were no deaths in children following prolonged febrile convulsions and idiopathic convulsive status epilepticus. A quarter of deaths during follow-up were associated with intractable seizures/convulsive status epilepticus, and the rest died as a complication of their underlying medical condition. On regression analysis, presence of clinically significant neurological impairments prior to convulsive status epilepticus was the only independent risk factor for mortality. In conclusion, there is a high risk of death within 8 years following childhood convulsive status epilepticus but most deaths are not seizure related. Presence of pre-existing clinically significant neurological impairments at the time of convulsive status epilepticus is the main risk factor for mortality within 8 years after the acute episode. The attributable role of convulsive status epilepticus on mortality remains uncertain, but appears less than is generally perceived.
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Behavioral problems in children with epilepsy in rural Kenya.
Epilepsy Behav
PUBLISHED: 08-16-2011
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The aims of this study were to record behavioral problems in children with epilepsy (CWE), compare the prevalence with that reported among healthy children without epilepsy, and investigate the risk factors. A child behavioral questionnaire for parents comprising 15 items was administered to the main caregiver of 108 CWE and 108 controls matched for age in Kilifi, Kenya. CWE had a higher mean score for reported behavioral problems than controls (6.9 vs 4.9, t=4.7, P<0.001). CWE with active epilepsy also recorded more behavioral problems than those with inactive epilepsy (8.2 vs 6.2, t=-2.9, P=0.005). A significantly greater proportion of CWE (49% vs 26% of controls) were reported to have behavioral problems. Active epilepsy, cognitive impairment, and focal seizures were the most significant independent covariates of behavioral problems. Behavioral problems in African CWE are common and need to be taken into consideration in planning comprehensive clinical services in this region.
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Co-morbidity of epilepsy in Tanzanian children: a community-based case-control study.
Seizure
PUBLISHED: 08-09-2011
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To define the prevalence and associations of co-morbidity and school attendance in older children with epilepsy (CWE) from a rural district of Tanzania by conducting a community-based case-control study.
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Language deficits and altered hemispheric lateralization in young people in remission from BECTS.
Epilepsia
PUBLISHED: 05-13-2011
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Benign epilepsy with centrotemporal spikes (BECTS) is associated with language disturbances during the active phase of the seizure disorder. However, it is not yet known whether these deficits are reversible or have long-term impact on the development of language skills. We report on a study conducted in 13 patients in remission from BECTS and 13 age-matched controls, who underwent neuropsychological assessments. We also recorded event-related potentials (ERPs) during a verb generation task, to determine cortical lateralization of language. The BECTS group showed significant language deficits compared to controls as well as a pattern of atypical ERP lateralization in frontal regions. These findings support the view that BECTS in childhood may lead to enduring effects on brain maturation and language development.
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Academic achievement in children with epilepsy: a review.
Epilepsy Res.
PUBLISHED: 03-09-2011
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To examine published studies which have focussed on academic achievement in children with epilepsy with respect to prevalence rates of academic difficulties and possible correlates of academic achievement.
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Depression and anxiety in childhood epilepsy: a review.
Seizure
PUBLISHED: 02-02-2011
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Population based studies suggest that symptoms of depression and anxiety are more frequent in children and adolescents with epilepsy compared with the general population. In terms of the manifestations of symptoms of depression and anxiety, there would appear to some symptoms unique to epilepsy in that they are associated with seizures and/or antiepileptic medications but these idiosyncratic symptoms remain under reported and have not been extensively studied. In terms of correlates of significant symptoms of depression and anxiety in children with epilepsy, some reports indicate that seizure variables (e.g., seizure frequency) and use of polytherapy are associated with increases in symptoms whereas other studies have not found this relationship. Child and family attitude/adaptation to epilepsy may also be risk factors for depression and anxiety but more research is needed in this area. The assessment of symptoms of depression and anxiety in children with epilepsy can be challenging given the possible role of seizures and AEDs, and comprehensive assessment will involve the use of screening measures, diagnostic interviews and a consideration of epilepsy specific factors. There have been few studies carried out with respect to the treatment of symptoms and depression and anxiety in children and adolescents with epilepsy. There is a significant need for a greater understanding of the nature of symptoms of depression and anxiety in children with epilepsy to inform treatment decisions. While treatment of epilepsy specific symptoms of depression and anxiety may involve an evaluation of the current epilepsy treatment protocols, there may also be a need for pharmacological and/or psychotherapeutic interventions in the treatment of symptoms of depression and anxiety which are not epilepsy specific.
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Alternating hemiplegia of childhood: metabolic studies in the largest European series of patients.
Eur. J. Paediatr. Neurol.
PUBLISHED: 01-25-2011
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Alternating hemiplegia of childhood (AHC) is a rare disorder with diagnosis based on clinical criteria, as no laboratory, neuroradiological or genetic markers are currently available. The pathogenic mechanisms are still an enigma. Some hypotheses have been proposed such as hemiplegic migraine variant, epileptic mechanism, channelopathy and mitochondrial disorder, but none of these has been confirmed. Our aim was to analyze the results of metabolic studies performed on a series of 157 European patients who fulfilled diagnostic criteria for AHC. We tried to find a common metabolic abnormality, related with AHC. We did not find significant abnormalities in basic metabolic screening, at different ages. Neurotransmitters in cerebrospinal fluid (n = 26) were normal in all of the patients. Mitochondrial respiratory chain enzyme activities were analyzed in 19 muscle biopsies; in 4 cases, different MRC enzyme deficiencies were demonstrated, ranging from mild-unspecific deficiencies to more profound and probably primary defects. Although we did not find specific metabolic markers in our series, some metabolic disorders such as pyruvate dehydrogenase deficiency, MELAS, cerebral glucose transporter defect and neurotransmitter deficiency can exhibit symptoms similar to those of AHC and need to be ruled out before a diagnosis of AHC can be established. Further studies including high-throughput diagnostic technologies seem necessary to elucidate the etiology of this severe and enigmatic disorder.
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Evidence of a non-progressive course of alternating hemiplegia of childhood: study of a large cohort of children and adults.
Brain
PUBLISHED: 10-24-2010
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Alternating hemiplegia of childhood is a neurological disorder characterized by episodes of hemiplegia, various non-epileptic paroxysmal events and global neurological impairment. Characterization of the evolution and outcome into adulthood has not been sufficiently investigated. The goal of this study was to elucidate the natural history of alternating hemiplegia within a large cohort of 157 patients, as part of the European Network for Research on Alternating Hemiplegia project. A questionnaire was formulated to determine the severity of both paroxysmal and global neurological impairment and address progression of the disorder by allocating data to specific age epochs up to and over 24 years of age. Patients in early age groups were consistently present in subsequent later age groups and for each patient, data were collected for each corresponding age epoch. The study was based on predominantly retrospective and, for a period of 2 years, prospective data. At inclusion, patients were aged from 9 months to 52 years. The median age at diagnosis was 20 months. All patients experienced hemiplegic attacks; 86.5% reported episodes of bilateral weakness, 88% dystonic attacks, 53% epileptic seizures, 72% developed chorea and/or dystonia and 92% mental retardation. When data over the course of the illness were examined for the whole cohort, the severity of symptoms did not appear to change, with the exception of abnormal ocular movements and hypotonia that regressed, but did not disappear into adulthood (from 86 to 36% and 76 to 36%, respectively). No statistically significant correlation between a history of severe paroxysmal hemiplegic/dystonic episodes and a worse neurological outcome was identified. Seven patients died, some of whom experienced severe plegic attacks or epileptic seizures at the time of death. History of severe plegic/dystonic attacks was not found to be an aggravating factor for deceased patients. Our results provide evidence that the natural history of alternating hemiplegia is highly variable and unpredictable for individual patients. However, we did not find evidence to support a steadily progressive and degenerative course of the disorder when patients were analysed as a group. For a minority of patients, a risk of sudden death was associated with more severe neurological impairment. The European Network for Research on Alternating Hemiplegia Registry, validated by our study, includes all major neurological signs and symptoms of alternating hemiplegia and may thus be used as a precedent for the progressive inclusion and follow-up of patients as well as a reference for genetic studies and treatment trials.
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Ophthalmological, cognitive, electrophysiological and MRI assessment of visual processing in preterm children without major neuromotor impairment.
Dev Sci
PUBLISHED: 08-18-2010
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Many studies report chronic deficits in visual processing in children born preterm. We investigated whether functional abnormalities in visual processing exist in children born preterm but without major neuromotor impairment (i.e. cerebral palsy). Twelve such children (< 33 weeks gestation or birthweight < 1000 g) without major neuromotor impairment and 12 born full-term controls were assessed at 8-12 years of age by means of ophthalmological assessment (visual acuity, colour vision, stereopsis, stereoacuity, visual fields, ocular motility, motor fusion), cognitive tests of visual-motor, visual-perceptual and visual-spatial skills and pattern-reversal visual evoked potentials (PR-VEPs). All participants also underwent magnetic resonance imaging (MRI) of the brain and neuromotor assessments. No significant differences were found between the groups on the ophthalmological, visual cognitive, neurological, neuromotor or MRI measures. The P100 component of the PR-VEP showed a significantly shorter latency in the preterm compared with the full-term participants. Whilst this P100 finding suggests that subtle abnormalities may exist at the neurophysiological level, we conclude that visual dysfunction is not systematically associated with preterm birth in the context of normal neurological status.
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Febrile seizures are a syndrome of secondarily generalized hippocampal epilepsy.
Dev Med Child Neurol
PUBLISHED: 08-16-2010
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the objective of this study was to examine in detail the semiology of febrile seizures, particularly to look for features that might suggest focality. In prolonged febrile seizures there is acute evidence of hippocampal involvement. Retrospective data relates mesial temporal sclerosis to such early prolonged febrile seizures. Animal models of prolonged seizures causing hippocampal damage show limbic seizures at low dose of the precipitants.
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Clinical and neurophysiologic features of active convulsive epilepsy in rural Kenya: a population-based study.
Epilepsia
PUBLISHED: 07-09-2010
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Epilepsy is common in sub-Saharan Africa but is poorly characterized. Most studies are hospital-based, and may not reflect the situation in rural areas with limited access to medical care. We examined people with active convulsive epilepsy (ACE), to determine if the clinical features could help elucidate the causes.
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Iron deficiency and acute seizures: results from children living in rural Kenya and a meta-analysis.
PLoS ONE
PUBLISHED: 04-09-2010
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There are conflicting reports on whether iron deficiency changes susceptibility to seizures. We examined the hypothesis that iron deficiency is associated with an increased risk of acute seizures in children in a malaria endemic area.
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Atypical brain response to novelty in rural African children with a history of severe falciparum malaria.
J. Neurol. Sci.
PUBLISHED: 02-03-2010
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Plasmodium falciparum is the most common parasitic infection of the central nervous system causing neuro-cognitive deficits in 5-26% of paediatric cases. The burden cannot be reliably estimated because of lack of sensitive, culture-fair and robust assessments in rural settings. Auditory and visual brain event related potentials (ERPs) are used to compare novelty processing in children exposed to severe malaria with community controls. Fifty children previously admitted and discharged from Kilifi District Hospital with severe falciparum malaria were selected and compared with 77 unexposed age matched children. The results showed that up to 14% of children exposed to severe malaria had significantly different responses to novelty compared to unexposed children. Children exposed to severe malaria had smaller P3a amplitudes to novelty in both auditory [F (3, 119)=4.545, p=0.005] and visual [F (3, 119)=6.708, p<0.001] paradigms compared to unexposed children. In the auditory domain the differences in processing of novelty were not related to early component processing. The percentage of children with severe malaria showing impaired performance using ERPs is within the range previously reported using neuropsychological tests. The overall pattern suggests that severe malaria affects prefrontal and temporal cortices normally activated by stimulus novelty.
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Auditory and visual novelty processing in normally-developing Kenyan children.
Clin Neurophysiol
PUBLISHED: 01-18-2010
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The aim of this study was to describe the normative development of the electrophysiological response to auditory and visual novelty in children living in rural Kenya.
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Comparative studies on the topical administration of mucopolysaccharide and heparin ointments in nonhuman primates.
Clin. Appl. Thromb. Hemost.
PUBLISHED: 12-02-2009
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Mucopolysaccharide polysulfate (MPS) represents a mammalian-derived sulfated polysaccharide. Because the origin and structure of heparins is similar to MPS, this study was conducted to compare 2 ointment formulations containing MPS or heparin with a placebo ointment on tissue factor pathway inhibitor (TFPI) released in nonhuman primates (Macaca mulatta). A primate colony composed of 18 animals, housed at Loyola University Medical Center, was used in compliance with an Institutional Animal Care and Use Committee (IACUC)-approved protocol. Mucopolysaccharide polysulfate (4.5%), heparin (4.5%), and a placebo ointment were topically applied to individual groups of primates in a crossover study for periods of up to 2 weeks. Blood samples were drawn on days 1, 2, 5, 7, and 10. The anticoagulant effects (activated partial thromboplastin time [APTT], Heptest, thrombin time [TT]), TFPI antigen and functional levels, thrombin activatable fibrinolytic inhibitor (TAFI), and antiheparin platelet factor 4 antibodies (AHPF4 abs) were measured in citrated plasma. All data were compiled as mean +/- 1 standard deviation and compared in groups. Topical administration of both the MPS and heparin ointments resulted in no measurable anticoagulant effects in the primate model; however, MPS produced a concentration-dependent release of TFPI antigen and a functional activity that was stronger than the effects observed with heparin. A decrease in TAFI activation was also observed in the MPS-treated primates. In addition, in the heparin-treated group, a slight increase in AHPF4 abs was observed. In conclusion, MPS showed a stronger release of TFPI than heparin that was not associated with a strong anticoagulant effect. Moreover, MPS downregulated TAFI, resulting in an enhanced fibrinolytic effect.
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Worster-Drought syndrome: poorly recognized despite severe and persistent difficulties with feeding and speech.
Dev Med Child Neurol
PUBLISHED: 10-12-2009
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Worster-Drought syndrome (WDS), or congenital suprabulbar paresis, is a permanent movement disorder of the bulbar muscles causing persistent difficulties with swallowing, feeding, speech, and saliva control owing to a non-progressive disturbance in early brain development. As such, it falls within the cerebral palsies. The aim of this study was to describe the physical and neuropsychological profiles of children with WDS.
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Impaired everyday memory associated with encephalopathy of severe malaria: the role of seizures and hippocampal damage.
Malar. J.
PUBLISHED: 09-16-2009
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Seizures are common in children admitted with severe falciparum malaria and are associated with neuro-cognitive impairments. Prolonged febrile seizures are associated with hippocampal damage and impaired memory. It was hypothesized that severe malaria causes impaired everyday memory which may be associated with hippocampal damage.
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The surgical treatment of Landau-Kleffner syndrome.
Epilepsia
PUBLISHED: 08-18-2009
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The medical management of Landau-Kleffner syndrome is usually effective for seizure control and eventual seizure remission. However, the response for language and behavior is often poor. Surgery, in the form of multiple subpial transections (MSTs) to include Wernickes area has been suggested as a way forward if electrophysiologic lateralization can be demonstrated. Surgical series in the literature are few and outcome from surgery variable.
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Congenital perisylvian dysfunction - is it a spectrum?
Dev Med Child Neurol
PUBLISHED: 06-22-2009
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This study examines the overlap between children with bulbar cerebral palsy (Worster-Drought syndrome [WDS]) and perisylvian polymicrogyria.
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Socioeconomic deprivation independent of ethnicity increases status epilepticus risk.
Epilepsia
PUBLISHED: 01-31-2009
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A higher incidence of convulsive status epilepticus (CSE) has been reported in nonwhite compared to white populations. Socioeconomic factors can be intricately involved in observed ethnic "effects," and the importance of socioeconomic status on health conditions is widely recognized. Understanding the effect of socioeconomic factors on CSE would provide insights into etiology and management, leading to the development of novel prevention strategies.
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Recognition memory is impaired in children after prolonged febrile seizures.
Brain
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Children with a history of a prolonged febrile seizure show signs of acute hippocampal injury on magnetic resonance imaging. In addition, animal studies have shown that adult rats who suffered febrile seizures during development reveal memory impairments. Together, these lines of evidence suggest that memory impairments related to hippocampal injury may be evident in human children after prolonged febrile seizures. The current study addressed this question by investigating memory abilities in 26 children soon after a prolonged febrile seizure (median: 37.5 days) and compared their results to those of 37 normally developing children. Fifteen patients were reassessed at a mean of 12.5 months after their first assessment to determine the transiency of any observed effects. We used the visual paired comparison task to test memory abilities in our group, as this task does not depend on verbal abilities and also because successful performance on the task has been proven to depend on the presence of functional hippocampi. Our findings show that patients perform as well as controls in the absence of a delay between the learning phase and the memory test, suggesting that both groups are able to form representations of the presented stimulus. However, after a 5-min delay, patients recognition memory is not different from chance, and comparison of patients and controls points to an accelerated forgetting rate in the prolonged febrile seizure group. The patients performance was not related to the time elapsed from the acute event or the duration of the prolonged febrile seizure, suggesting that the observed effect is not a by-product of the seizure itself or a delayed effect of medication administered to terminate the seizure. By contrast, performance was related to hippocampal size; participants with the smallest mean hippocampal volumes revealed the biggest drop in performance from the immediate to the delayed paradigm. At follow-up, children were still showing deficiencies in recognizing a face after a 5-min delay. Similarly, this suggests that the observed memory impairments are not a transient effect of the prolonged febrile seizures. This is the first report of such impairments in humans, and it is clinically significant given the links between mesial temporal sclerosis and prolonged febrile seizures. The persistence of these impairments a year onwards signals the potential benefits of intervention in these children who run the risk of developing episodic memory deficits in later childhood.
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Role of ESSENCE for preschool children with neurodevelopmental disorders.
Brain Dev.
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Early Symptomatic Syndromes Eliciting Neurodevelopmental Examinations (ESSENCE) has been proposed as a guide to the neurodevelopmental needs of the under 5s. The problems are their multiplicity, the presence of partial features of specific conditions e.g. autism spectrum disorder and attention deficit/hyperactivity disorder and the young age of the children. For these reasons, child development teams often leave families to cope with very difficult situations. This paper includes epilepsy and the cerebral palsies to see if providing precise diagnostic categories and therapeutic targets can be achieved. It includes a discussion of causal sequences which have yet to be applied comprehensively to the neurodevelopment disorders.
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Prediction of seizure outcome in childhood epilepsies in countries with limited resources: a prospective study.
Dev Med Child Neurol
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To identify predictors of seizure control in newly presenting children with epilepsy in countries with limited resources.
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Sepiapterin reductase deficiency: a treatable mimic of cerebral palsy.
Ann. Neurol.
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Sepiapterin reductase deficiency (SRD) is an under-recognized levodopa-responsive disorder. We describe clinical, biochemical, and molecular findings in a cohort of patients with this treatable condition. We aim to improve awareness of the phenotype and available diagnostic and therapeutic strategies to reduce delayed diagnosis or misdiagnosis, optimize management, and improve understanding of pathophysiologic mechanisms.
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Seizures in 204 comatose children: incidence and outcome.
Intensive Care Med
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Seizures are common in comatose children, but may be clinically subtle or only manifest on continuous electroencephalographic monitoring (cEEG); any association with outcome remains uncertain.
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Cognitive deficits following exposure to pneumococcal meningitis: an event-related potential study.
BMC Infect. Dis.
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Pneumococcal meningitis (PM) is a severe and life-threatening disease that is associated with cognitive impairment including learning difficulties, cognitive slowness, short-term memory deficits and poor academic performance. There are limited data on cognitive outcomes following exposure to PM from Africa mainly due to lack of culturally appropriate tools. We report cognitive processes of exposed children as measured by auditory and visual event-related potentials.
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Epilepsy in Tanzanian children: association with perinatal events and other risk factors.
Epilepsia
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To define the prevalence and risk factors for epilepsy in children in a rural district of Tanzania by conducting a community-based case-control study.
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Speech and Language Disorders in Kenyan Children: Adapting Tools For Regions With Few Assessment Resources.
J Psychol Afr
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This study sought to adapt a battery of Western speech and language assessment tools to a rural Kenyan setting. The tool was developed for children whose first language was KiGiryama, a Bantu language. A total of 539 Kenyan children (males=271, females=268, ethnicity=100% Kigiryama. Data were collected from 303 children admitted to hospital with severe malaria and 206 age-matched children recruited from the village communities. The language assessments were based upon the Content, Form and Use (C/F/U) model. The assessment was based upon the adapted versions of the Peabody Picture Vocabulary Test, Test for the Reception of Grammar, Renfrew Action Picture Test, Pragmatics Profile of Everyday Communication Skills in Children, Test of Word Finding and language specific tests of lexical semantics, higher level language. Preliminary measures of construct validity suggested that the theoretical assumptions behind the construction of the assessments were appropriate and re-test and inter-rater reliability scores were acceptable. These findings illustrate the potential to adapt Western speech and language assessments in other languages and settings, particularly those in which there is a paucity of standardised tools.
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The role of magnetic resonance imaging in the follow-up of children with convulsive status epilepticus.
Dev Med Child Neurol
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The aim of this study was to determine the yield of magnetic resonance imaging (MRI) after an episode of childhood convulsive status epilepticus (CSE) and to identify the clinical predictors of an abnormal brain scan.
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What is Visualize?

JoVE Visualize is a tool created to match the last 5 years of PubMed publications to methods in JoVE's video library.

How does it work?

We use abstracts found on PubMed and match them to JoVE videos to create a list of 10 to 30 related methods videos.

Video X seems to be unrelated to Abstract Y...

In developing our video relationships, we compare around 5 million PubMed articles to our library of over 4,500 methods videos. In some cases the language used in the PubMed abstracts makes matching that content to a JoVE video difficult. In other cases, there happens not to be any content in our video library that is relevant to the topic of a given abstract. In these cases, our algorithms are trying their best to display videos with relevant content, which can sometimes result in matched videos with only a slight relation.