ObjectiveTo report our experience and investigate 5 original hypotheses: (1) multiple types of epileptic seizures occur in alternating hemiplegia of childhood (AHC), and these can be the initial presentation; (2) epileptiform abnormalities often appear well after clinical seizures; (3) nonepileptic reduced awareness spells (RAS) occur frequently; (4) epilepsy is commonly drug resistant but may respond to vagal nerve stimulation (VNS); and (5) status epilepticus (SE) is common and is usually refractory and recurrent.MethodsWe analyzed a cohort of 51 consecutive patients with AHC.ResultsThirty-two of 51 patients had epilepsy: 18 focal seizures, frontal more frequently than temporal, and then posterior. Eleven had primary generalized seizures (tonic-clonic, myoclonic, and/or absence). Epileptic seizures preceded other AHC paroxysmal events in 8 (lag 5.63 ± 6.55 months; p = 0.0365). In 7 of 32, initial EEGs were normal, with the first epileptiform EEG lagging behind by 3.53 ± 4.65 years (p = 0.0484). RAS occurred equally in patients with epilepsy (16 of 32) and patients without epilepsy (10 of 19, p = 1.0). Twenty-eight patients had video-EEG; captured RAS showed no concomitant EEG changes. Nineteen patients (59%) were drug resistant. VNS resulted in >50% reduction in seizures in 5 of 6 (p < 0.04). Twelve patients (38%) had SE (9 of 12 multiple episodes), refractory/superrefractory in all (p < 0.001), and 4 of 12 had regression after SE.ConclusionsEpilepsy in AHC can be focal or generalized. Epileptic seizures may be the first paroxysmal symptom. EEG may become epileptiform only on follow-up. Epilepsy, although frequently drug resistant, can respond to VNS. RAS are frequent and nonepileptic. SE often recurs and is usually refractory/superrefractory. Our observations are consistent with current data on AHC-ATP1A3 pathophysiology.
Our data establish a detailed profile of motor function in alternating hemiplegia of childhood, argue against the presence of worse motor function in older patients, identify tools helpful in evaluating this population, and identify oropharyngeal function as the more severely affected domain, suggesting that brain areas controlling this function are more affected than others.
There has been considerable interest in applying electroencephalography (EEG) and functional near-infrared spectroscopy (fNIRS) simultaneously for multimodal assessment of brain function. EEG–fNIRS can provide a comprehensive picture of brain electrical and hemodynamic function and has been applied across various fields of brain science. The development of wearable, mechanically and electrically integrated EEG–fNIRS technology is a critical next step in the evolution of this field. A suitable system design could significantly increase the data/image quality, the wearability, patient/subject comfort, and capability for long-term monitoring. Here, we present a concise, yet comprehensive, review of the progress that has been made toward achieving a wearable, integrated EEG–fNIRS system. Significant marks of progress include the development of both discrete component-based and microchip-based EEG–fNIRS technologies; modular systems; miniaturized, lightweight form factors; wireless capabilities; and shared analogue-to-digital converter (ADC) architecture between fNIRS and EEG data acquisitions. In describing the attributes, advantages, and disadvantages of current technologies, this review aims to provide a roadmap toward the next generation of wearable, integrated EEG–fNIRS systems.
Aim To determine the neuropsychological abnormalities that occur in alternating hemiplegia of childhood (AHC) and report on our experience in managing them. Method Patients underwent evaluations according to our standardized AHC pathway. Data were entered into our prospective AHC database and then analyzed. Results Of the cohort of 25 consecutive patients (ages 15mo–42y), eight had initial chief complaints about cognition, 14 language, five attention, and 11 behavior. As compared to population norms means, neuropsychological and behavioral assessment tools (including Child Behavior Checklist, Vineland Adaptive Behavior Scales, Peabody Picture Vocabulary, and Wechsler Intelligence Quotient tests) showed significant impairments in multiple domains: cognition, expressive and receptive language, executive function/attention, and behavior (p<0.05 in all comparisons). Evaluations generated management recommendations in all patients. Twenty had neuropsychiatric diagnoses: 10 attention‐deficit/hyperactivity disorder (ADHD), seven disruptive behavior, and three anxiety disorder. Eight out of nine patients with ADHD who were prescribed medications responded to pharmacotherapy. Interpretation Patients with AHC have developmental difficulties related to impairments in multiple neuropsychological domains. This supports the hypothesis that the underlying AHC pathophysiology involves diffuse neuronal dysfunction. Testing generated recommendations to help manage these difficulties. Patients with AHC also have a range of neuropsychiatric diagnoses, the most common being ADHD which responds to pharmacotherapy. What this paper adds Patients with alternating hemiplegia of childhood (AHC) have developmental difficulties with underlying neuropsychological impairments. The findings in this study are consistent with an underlying AHC pathophysiology which involves diffuse neuronal, probably largely GABAergic, dysfunction. Patients with AHC have a range of neuropsychiatric diagnoses, the most common being attention‐deficit/hyperactivity disorder.
Millions of children are subjected to abuse, neglect, and displacement, and millions more are at risk for not achieving their developmental potential. Although there is a global movement to change this, driven by children's rights, progress is slow and impeded by political considerations. The United Nations Convention on the Rights of the Child, a global comprehensive commitment to children's rights ratified by all countries in the world except the United States (because of concerns about impingement on sovereignty and parental authority), has a special General Comment on "Implementing Child Rights in Early Childhood." More recently, the World Health Organization and United Nations Children's Fund have launched the Nurturing Care Framework for Early Childhood Development (ECD), which calls for public policies that promote nurturing care interventions and addresses 5 interrelated components that are necessary for optimal ECD. This move is also complemented by the Human Capital Project of the World Bank, providing a focus on the need for investments in child health and nutrition and their long-term benefits. In this article, we outline children's rights under international law, the underlying scientific evidence supporting attention to ECD, and the philosophy of nurturing care that ensures that children's rights are respected, protected, and fulfilled. We also provide pediatricians anywhere with the policy and rights-based frameworks that are essential for them to care for and advocate for children and families to ensure optimal developmental, health, and socioemotional outcomes. These recommendations do not necessarily reflect American Academy of Pediatrics policy.
Impact:1. Sleep in early life is essential for proper functional brain development, which is essential for the brain to integrate and process information. This process may be impaired in infants born preterm.2. The connection between preterm birth, early development of brain functional connectivity, and sleep is poorly understood. 3 3. This review discusses how sleep and brain functional connectivity develop in early life, how these processes might become impaired, and the challenges associated with understanding these processes. Potential solutions to these challenges are presented to provide direction for future research.
Early childhood (birth-8 years), particularly the first 3 years, is the most critical time in development because of the highly sensitive developing brain. Providing appropriate developmental care (i.e., nurturing care, as defined by the World Health Organization [WHO]) during early childhood is key to ensuring a child's holistic development. Pediatricians are expected to play a critical role in supporting early childhood development (ECD) through providing developmental services such as developmental monitoring, anticipatory guidance, screening, and referral to medical and/or community-based services when delay is identified. Pediatricians are also expected to serve as advocates within their clinics and communities for improved delivery of ECD services, such as advocating for increasing funding for ECD initiatives, increasing insurance coverage of ECD services, and working to increase other pediatricians' awareness of the principles of ECD and how to deliver developmental services. However, this does not always occur. Typically, pediatricians' training and practice emphasizes treating disease rather than enhancing ECD. Pediatricians are further hindered by a lack of uniformity across nations in guidelines for developmental monitoring and screening. In this article, we present the vision of the International Pediatric Association (IPA) of the roles that pediatricians, academic departments, medical training programs, and pediatric associations should fulfill to help support ECD, including raising ECD to higher levels of priority in routine pediatric care. First, we present the challenges that face these goals in supporting ECD. We then propose, with supportive literature, strategies and resources to overcome these challenges in collaboration with local and international stakeholders, including the IPA, the WHO, UNICEF, and the World Bank.
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