Residency Training: A Practical Guide for Medical Students who are Planning a Future in Child Neurology.

At the time of graduation from medical school, medical students have been exposed primarily to adult neurology and have limited exposure to child neurology. Child neurology is a unique field that encompasses caring for children with neurological conditions ranging from routine to rare. There are many opportunities for a variety of unique careers in child neurology including both in the inpatient and outpatient setting.

The Effect of Smartphone Video on Lead Time to Diagnosis of Infantile Spasms.

Objective: To assess whether access to smartphone video capture of infantile spasms at initial presentation is associated with improved time to diagnosis and treatment.

Methods: We conducted a collaborative retrospective cohort study of 80 consecutive infants with confirmed infantile epileptic spasms syndrome initially presenting from 2015 to 2021 at 2 US pediatric centers. Statistical methods used included Mann-Whitney U test to assess the difference in lead times to electroencephalogram (EEG), diagnosis, and treatment between groups with and without video capture.

EEG Findings Enhance the Yield of Epilepsy Gene Panel Testing in Children.

Background: Epilepsies with encephalopathy are important to promptly and thoroughly evaluate because of their associated refractory nature and potential for neurologic regression.

Methods: The results of epilepsy gene panel testing were obtained for 109 pediatric patients at Nemours Children's Center in Jacksonville, Florida, from 04/2019 to 02/2021. Results were obtained from Invitae TM via the offered epilepsy gene panel test and Behind the Seizure program.

Advances in Epilepsy Surgery.

Background: A large number of patients have epilepsy that is intractable and adversely affects a child's lifelong experience with addition societal burden that is disabling and expensive. The last two decades have seen a major explosion of new antiseizure medication options. Despite these advances, children with epilepsy continue to have intractable seizures.

Patterns Specific to Pediatric EEG.

Misinterpretations of the EEG can result in erroneous diagnosis of epilepsy, causing considerable family anxiety, over protectiveness of children, and delays in normal development and developmental exploration. The burden of a chronic disease can result in expensive and unnecessary medical treatment. The misdiagnosis of epilepsy has been well documented in adults, but misdiagnosis in normal children can have a long-lasting impact on their health.

Time course of reversal of valproate-mediated inhibition of lamotrigine.

Purpose: Conversion to lamotrigine (LTG) monotherapy from sodium valproate (VPA) is complicated by the robust pharmacokinetic interaction between the two AEDs. This study examined changes in LTG serum concentrations immediately following VPA discontinuation.

Methods: Ten healthy female and male adult subjects were initiated on LTG (Lamictal) 10 mg orally every morning for 30 days and VPA (Depakote ER) 500 mg orally every morning for 14 days.

Early Diagnosis and Treatment of Lennox-Gastaut Syndrome.

Lennox-Gastaut syndrome (LGS) is a severe form of childhood-onset epilepsy associated with high morbidity and mortality. The peak period for manifestations of Lennox-Gastaut syndrome is between ages 3 and 5 years, a time of critical brain development and corresponding vulnerability to the electroclinical dysfunction arising from Lennox-Gastaut syndrome. Diagnosis is based on a triad of symptoms: multiple seizure types, cognitive impairment, and slow spike-and-wave pattern on electroencephalography.

Brain-responsive neurostimulation in patients with medically intractable mesial temporal lobe epilepsy.

Objective: Evaluate the seizure-reduction response and safety of mesial temporal lobe (MTL) brain-responsive stimulation in adults with medically intractable partial-onset seizures of mesial temporal lobe origin.

Methods: Subjects with mesial temporal lobe epilepsy (MTLE) were identified from prospective clinical trials of a brain-responsive neurostimulator (RNS System, NeuroPace). The seizure reduction over years 2-6 postimplantation was calculated by assessing the seizure frequency compared to a preimplantation baseline.

Brain-responsive neurostimulation in patients with medically intractable seizures arising from eloquent and other neocortical areas.

Objective: Evaluate the seizure-reduction response and safety of brain-responsive stimulation in adults with medically intractable partial-onset seizures of neocortical origin.

Methods: Patients with partial seizures of neocortical origin were identified from prospective clinical trials of a brain-responsive neurostimulator (RNS System, NeuroPace). The seizure reduction over years 2-6 postimplantation was calculated by assessing the seizure frequency compared to a preimplantation baseline.

Factors Influencing Patient Experience in Pediatric Neurology.

Introduction: Hospitals have begun to shift toward patient-centered care because of the pay-for-performance system that was established by the Patient Protection and Affordable Care Act. In pediatrics, the needs of both the caregiver and the pediatric patient have to be taken into account. Pediatric practices have been shifting toward a family-centered approach, although the primary drivers have not been well defined.

Survival following decompressive hemicraniectomy for hemiconvulsion-hemiplegia-epilepsy syndrome: case report.

Hemiconvulsion-hemiplegia-epilepsy (HHE) is an uncommon epileptic syndrome that affects young children. Typical management includes early initiation of benzodiazepines to abate the initial seizure activity quickly. Patients in whom epilepsy develops require prolonged use of antiepileptic agents.

Anti-NMDA-receptor antibody encephalitis in infants.

Purpose: Anti-N-methyl-d-aspartate (NMDA) receptor antibody encephalitis is an autoimmune disorder manifesting subacutely with prominent aberrant movements and psychiatric symptoms. The clinical course is one of progressive clinical deterioration that can be halted and often reversed by early diagnosis and treatment. Patterns of presentation and etiology of anti-NMDA-receptor antibody encephalitis are dependent on age and can be challenging to recognize in very young children.

Pediatric anti-NMDA (N-methyl D-aspartate) receptor encephalitis.

Background: We report the clinical features and course of pediatric patients presenting with anti-N-methyl D-aspartate receptor (NMDA-R) encephalitis.

Methods: Single-center 4-year observational study of pediatric encephalitis associated with NMDA-R antibodies in the serum and/or the cerebrospinal fluid.

Results: Three girls with anti-NMDA-R encephalitis were identified.

Seizures from valproate-carbapenem interaction.

Background: Antiepileptic medication interactions can complicate the management of epilepsy, by either increasing or reducing the effective serum concentrations thereby causing adverse effects or loss of seizure control.

Results: A 14-year-old girl with well-controlled juvenile absence epilepsy lost control of her seizures acutely following the administration of carbapenem for pneumonia. Serum valproate concentrations fell by 90% within 48 hours following carbapenem and returned to baseline following its discontinuation.

Is lower IQ in children with epilepsy due to lower parental IQ? A controlled comparison study.

Aim: The aim of this study was to determine the relationship between parent and child Full-scale IQ (FSIQ) in children with epilepsy and in typically developing comparison children and to examine parent-child IQ differences by epilepsy characteristics.

Method: The study participants were 97 children (50 males, 47 females; age range 8-18y; mean age 12y 3mo, SD 3y1mo) with recent-onset epilepsy including idiopathic generalized (n=43) and idiopathic localization-related epilepsies (n=54); 69 healthy comparison children (38 females, 31 males; age range 8-18y; mean age 12y 8mo, SD 3y 2mo), and one biological parent per child. All participants were administered the Wechsler Abbreviated Scale of Intelligence (WASI).

Pediatric epileptology.

Challenges facing children with epilepsy are understanding the neurobiology of pharmacoresistance of epileptic encephalopathies and the development of effective surgical treatment options for those with "non-lesional" epilepsy. Although, understanding the genetics of childhood epilepsy has advanced, an effective treatment intervention has not occurred. Recently, understanding the neurobiology of hamartin and tuberin in the development of epilepsy and cognitive impairment associated with tuberous sclerosis complex allowed the development of sirolimus and everolimus to be used in human clinical trials.

Absence epilepsy with focal clinical and electrographic seizures.

Primary generalized seizures occur in half of all children with epilepsy. Correct classification carries important diagnostic, therapeutic, and prognostic information. Approximately half of all patients with generalized juvenile myoclonic epilepsy may have a partial semiology or focal electroencephalographic features although similar findings have not been described in absence epilepsy.

Brain development in children with new onset epilepsy: a prospective controlled cohort investigation.

Purpose: To characterize prospective neurodevelopmental changes in brain structure in children with new and recent-onset epilepsy compared to healthy controls.

Methods: Thirty-four healthy controls (mean age 12.9 years) and 38 children with new/recent-onset idiopathic epilepsy (mean age 12.

Children with new-onset epilepsy exhibit diffusion abnormalities in cerebral white matter in the absence of volumetric differences.

The purpose of this investigation was to examine the diffusion properties of cerebral white matter in children with recent onset epilepsy (n=19) compared to healthy controls (n=11). Subjects underwent DTI with quantification of mean diffusion (MD), fractional anisotropy (FA), axial diffusivity (D(ax)) and radial diffusivity (D(rad)) for regions of interest including anterior and posterior corpus callosum, fornix, cingulum, and internal and external capsules. Quantitative volumetrics were also performed for the corpus callosum and its subregions (anterior, midbody and posterior) and total lobar white and gray matter for the frontal, parietal, temporal and occipital lobes.

Adolescent and caregiver experiences with epilepsy.

Epilepsy during adolescence can impede the development of psychosocial independence and typical biological maturational processes. We examined in parallel the experiences and perceptions of adolescent patients with epilepsy and their caregivers. Specifically, we focused on frequency and type of seizures, comorbid conditions, adherence to therapies, productivity, clinical and quality of life consequences of seizures, estimated use and content of seizure emergency plans, and the patient-physician relationship.

Thalamofrontal circuitry and executive dysfunction in recent-onset juvenile myoclonic epilepsy.

Purpose: Thalamofrontal abnormalities have been identified in chronic primary generalized epilepsy, specifically in juvenile myoclonic epilepsy (JME). These regions also underlie executive functioning, although their relationship has yet to be examined in JME. This study examined the relationship between thalamic and frontal volumes and executive function in recent-onset JME compared to healthy control subjects and recent-onset benign childhood epilepsy with centrotemporal spikes (BCECTS), a syndrome not typically associated with thalamocortical or executive dysfunction.

Metabolic effects of AEDs: impact on body weight, lipids and glucose metabolism.

Epilepsy is a chronic disorder often requiring years of treatment. Accordingly, adverse effects of epilepsy and its treatment can impact general health for many decades. The psychological consequences of epilepsy are well documented, although the metabolic consequences of the treatment of epilepsy have only recently received attention.

Growing up with epilepsy: a two-year investigation of cognitive development in children with new onset epilepsy.

Purpose: To characterize patterns and determinants of normal and abnormal cognitive development in children with new onset epilepsy compared to healthy controls.

Methods: Longitudinal (2-year) cognitive growth was examined in 100 children, age 8-18 years, including healthy controls (n = 48) and children with new onset epilepsy (n = 52). Cognitive maturation was examined as a function of the presence/absence of two neurobehavioral comorbitiies (attention deficit hyperactivity disorder and/or academic problems) identified at the time of epilepsy diagnosis.