Staring Spells: How to Distinguish Epileptic Seizures from Nonepileptic Staring.

Objective: To determine the nature of staring spells and factors distinguishing epileptic from nonepileptic staring spells, we studied the clinical and demographic features of children with staring spells referred to a regional new-onset seizure clinic.

Study Design: Our retrospective chart review encompassed 2818 consecutive patients evaluated in the new-onset seizure clinic between September 22, 2015, and March 19, 2018. We identified 121 patients with newly presenting staring spells.

Creatine transporter deficiency impairs stress adaptation and brain energetics homeostasis.

The creatine transporter (CrT) maintains brain creatine (Cr) levels, but the effects of its deficiency on energetics adaptation under stress remain unclear. There are also no effective treatments for CrT deficiency, the second most common cause of X-linked intellectual disabilities. Herein, we examined the consequences of CrT deficiency in brain energetics and stress-adaptation responses plus the effects of intranasal Cr supplementation.

Evaluation of pediatric patients in new-onset seizure clinic (NOSc).

Aim: We evaluated the clinical and demographic features of children presenting with unprovoked seizures at a regional new-onset seizure clinic (NOSc).

Methods: We retrospectively reviewed charts of 492 consecutive patients evaluated in the NOSc at the Childrne's Healthcare of Atlanta RESULTS: Nonepileptic events (NEE) were diagnosed in 102 (24%) and epileptic seizures in the remaining 326 (76%). Patients with NEE were younger than patients with epileptic seizure (5.

Epidemiology of traumatic brain injury-associated epilepsy and early use of anti-epilepsy drugs: An analysis of insurance claims data, 2004-2014.

Background: About 2.8 million TBI-related emergency department visits, hospitalizations and deaths occurred in 2013 in the United States. Post-traumatic epilepsy (PTE) can be a disabling, life-long outcome of TBI.

Language and social functioning in children and adolescents with epilepsy.

Individuals with epilepsy have difficulties with social function that are not adequately accounted for by seizure severity or frequency. This study examined the relationship between language ability and social functioning in 193 children with epilepsy over a period of 36months following their first recognized seizure. The findings show that children with persistent seizures have poorer language function, even at the onset of their seizures, than do their healthy siblings, children with no recurrent seizures, and children with recurrent but not persistent seizures.

Design and implementation of the first randomized controlled trial of coenzyme CoQ₁₀ in children with primary mitochondrial diseases.

We report the design and implementation of the first phase 3 trial of CoenzymeQ₁₀ (CoQ₁₀) in children with genetic mitochondrial diseases. A novel, rigorous set of eligibility criteria was established. The trial, which remains open to recruitment, continues to address multiple challenges to the recruitment of patients, including widely condoned empiric use of CoQ₁₀ by individuals with proven or suspected mitochondrial disease and skepticism among professional and lay mitochondrial disease communities about participating in placebo-controlled trials.

Cyclocreatine treatment improves cognition in mice with creatine transporter deficiency.

The second-largest cause of X-linked mental retardation is a deficiency in creatine transporter (CRT; encoded by SLC6A8), which leads to speech and language disorders with severe cognitive impairment. This syndrome, caused by the absence of creatine in the brain, is currently untreatable because CRT is required for creatine entry into brain cells. Here, we developed a brain-specific Slc6a8 knockout mouse (Slc6a8-/y) as an animal model of human CRT deficiency in order to explore potential therapies for this syndrome.

Importance of muscle light microscopic mitochondrial subsarcolemmal aggregates in the diagnosis of respiratory chain deficiency.

The purpose of this study was to evaluate relationships between subsarcolemmal mitochondrial aggregates and electron transport chain deficiencies in skeletal muscle with the objective of establishing an association between mitochondrial accumulation and electron transport chain complex deficiency. We conducted a large-scale, retrospective study to evaluate factors associated with subsarcolemmal mitochondrial aggregates (percent) in pediatric patients who received muscle biopsies for suspected respiratory chain disorders. Patients were included if they had histochemical stains for assessment of mitochondrial pathology and had biochemical testing for muscle electron transport chain complex activities.

Ictal MEG onset source localization compared to intracranial EEG and outcome: improved epilepsy presurgical evaluation in pediatrics.

Purpose: Magnetoencephalography (MEG) has been shown a useful diagnostic tool for presurgical evaluation of pediatric medically intractable partial epilepsy as MEG source localization has been shown to improve the likelihood of seizure onset zone (SOZ) sampling during subsequent evaluation with intracranial EEG (ICEEG). We investigated whether ictal MEG onset source localization further improves results of interictal MEG in defining the SOZ.

Methods: We identified 20 pediatric patients with one habitual seizure during MEG recordings between October 2007 and April 2011.

Aberrant high-gamma oscillations in the somatosensory cortex of children with cerebral palsy: a meg study.

Objective: Our study is to investigate somatosensory dysfunction in children with spastic cerebral palsy (CP) using magnetoencephalography (MEG) and synthetic aperture magnetometry (SAM).

Methods: Six children with spastic CP and six age- and gender-matched typically developing children were studied using a 275-channel MEG system while their left and right index fingers were stimulated in random order. The latency and amplitude of somatosensory evoked magnetic fields were analyzed at sensor level.

Enzyme inducing antiepileptic drugs are associated with mitochondrial proliferation and increased cytochrome c oxidase activity in muscle of children with epilepsy.

Purpose: To evaluate the effects of epilepsy-related factors associated with mitochondrial pathology and function in skeletal muscle of children with suspected mitochondrial disorders.

Methods: This case-control study evaluated patients and age-matched controls with muscle biopsies at Cincinnati Children's Hospital Medical Center obtained between January, 2000 and December, 2008.

Results: A total of 65 epilepsy patients and 65 age-matched controls met the inclusion criteria.

Neuromagnetic measures of word processing in bilinguals and monolinguals.

Objective: This study aimed to use magnetoencephalography (MEG) to examine the question of whether Mandarin-English bilingual speakers recruit the same cortical areas or develop distinct language-specific networks without overlaps for word processing.

Methods: Eight healthy Mandarin-English bilingual adults and eight healthy English monolingual adults were scanned while single-word paradigms were audio-visually presented.

Results: Our results showed significantly stronger beta-band power suppression in the right inferior parietal lobe (IPL) covering the supramarginal gyrus (BA 40) and angular gyrus (BA 39) for bilinguals when processing Mandarin versus English.

Creatine transporter (CrT; Slc6a8) knockout mice as a model of human CrT deficiency.

Mutations in the creatine (Cr) transporter (CrT; Slc6a8) gene lead to absence of brain Cr and intellectual disabilities, loss of speech, and behavioral abnormalities. To date, no mouse model of CrT deficiency exists in which to understand and develop treatments for this condition. The purpose of this study was to generate a mouse model of human CrT deficiency.

Neuromagnetic evidence of impaired cortical auditory processing in pediatric intractable epilepsy.

Purpose: We aimed to determine the changes in neural correlates of auditory information processing such as auditory detection, encoding, and sensory discrimination in pediatric patients with intractable epilepsy.

Methods: In this magnetoencephalography (MEG) study, 10 patients and 10 age- and gender-matched healthy controls were investigated with the multi-feature mismatch negativity (MMN) paradigm. Latencies and amplitudes of M100, M150, M200, and MMN event-related fields were evaluated.

White matter abnormalities in children and adolescents with temporal lobe epilepsy.

Background And Purpose: The widespread propagation of synchronized neuronal firing in seizure disorders may affect cortical and subcortical brain regions. Diffusion tensor imaging (DTI) can noninvasively quantify white matter integrity. The purpose of this study was to investigate the abnormal changes of white matter in children and adolescents with focal temporal lobe epilepsy (TLE) using DTI.

High gamma oscillations of sensorimotor cortex during unilateral movement in the developing brain: a MEG study.

Recent studies in adults have found consistent contralateral high gamma activities in the sensorimotor cortex during unilateral finger movement. However, no study has reported on this same phenomenon in children. We hypothesized that contralateral high gamma activities also exist in children during unilateral finger movement.

Self-esteem and symptoms of depression in children with seizures: relationships with neuropsychological functioning and family variables over time.

Purpose: To test over time the relationships of neuropsychological functioning to mental health in children following a first recognized seizure and, of primary importance, to determine if the strength of these relationships differs based on risk and protective factors.

Methods: In a larger prospective study, 135 children with a first seizure (ages 8-14 years) and 73 healthy sibling controls completed neuropsychological testing at baseline and 36 months. Structured telephone interviews were used to obtain data from children on mental health and family environment; major caregiving parents provided data on demographic and family variables.

The effect of temperament and neuropsychological functioning on behavior problems in children with new-onset seizures.

The present study is part of a larger project that seeks to identify factors that predict children's behavioral, social, and cognitive adaptation to epilepsy. Children with seizures are more likely to have internalizing and externalizing behavior problems than either healthy children or children with other chronic illnesses. The present research examines risk factors for behavior problems.

Noninvasive localization of epileptogenic zones with ictal high-frequency neuromagnetic signals.

Object: Recent reports suggest that high-frequency epileptic activity is highly localized to epileptogenic zones. The goal of the present study was to investigate the potential usefulness of noninvasive localization of high-frequency epileptic activity for epilepsy surgery.

Methods: Data obtained in 4 patients, who had seizures during routine magnetoencephalography (MEG) tests, were retrospectively studied.

Gamma oscillations in the primary motor cortex studied with MEG.

In recent years, there has been a growing interest on the role of gamma band (>30 Hz) neural oscillations in motor control, although the function of this activity in motor control is unknown clearly. With the goal of discussing the high frequency sources non-invasively and precisely during unilateral index finger movement, we investigated gamma band oscillations in 20 right-handed normal adults with magnetoencephalography (MEG). The results showed that gamma band activity appeared only during finger movement.

Frequency and spatial characteristics of high-frequency neuromagnetic signals in childhood epilepsy.

Purpose: Invasive intracranial recordings have suggested that high-frequency oscillation is involved in epileptogenesis and is highly localized to epileptogenic zones. The aim of the present study is to characterize the frequency and spatial patterns of high-frequency brain signals in childhood epilepsy using a non-invasive technology.

Methods: Thirty children with clinically diagnosed epilepsy were studied using a whole head magnetoencephalography (MEG) system.

Results of the Sensory Profile in children with suspected childhood apraxia of speech.

Speech-sound disorders are common in preschool-age children, and are characterized by difficulty in the planning and production of speech sounds and their combination into words and sentences. The objective of this study was to review and compare the results of the Sensory Profile (Dunn, 1999) in children with a specific type of speech-sound disorder, childhood apraxia of speech (CAS), and to explore the relationship between sensory processing and sound-production deficits. Participants were identified prospectively through an interdisciplinary apraxia clinic at a tertiary care pediatric hospital, and results of the Sensory Profile were compiled and reviewed.