Seminars in Pediatric Neurology

Although rare in children, arterial ischemic stroke (AIS) is associated with increased mortality and neurological morbidity. The incidence of AIS after the neonatal period is approximately 1-2/100,000/year, with an estimated mortality of 3-7%. A significant proportion of children surviving AIS experience life-long neurological deficits including hemiparesis, epilepsy, and cognitive delays. The low incidence of childhood AIS coupled with atypical clinical-presentation and lack of awareness contribute to delay in diagnosis and consequently, the early initiation of treatment...

Perinatal stroke is a well-defined heterogenous group of disorders involving a focal disruption of cerebral blood flow between 20 weeks gestation and 28 days of postnatal life. The most focused lifetime risk for stroke occurs during the first week after birth. The morbidity of perinatal stroke is high, as it is the most common cause of hemiparetic cerebral palsy which results in lifelong disability that becomes more apparent throughout childhood. Perinatal strokes can be classified by the timing of diagnosis (acute or retrospective), vessel involved (arterial or venous), and underlying cause (hemorrhagic or ischemic)...

No abstract text is available yet for this article.

Pediatric stroke is unfortunately not a rare condition. It is associated with severe disability and mortality because of the complexity of potential clinical manifestations, and the resulting delay in seeking care and in diagnosis. Neuroimaging plays an important role in the multidisciplinary response for pediatric stroke patients. The rapid development of adult endovascular thrombectomy has created a new momentum in health professionals caring for pediatric stroke patients. Neuroimaging is critical to make decisions of identifying appropriate candidates for thrombectomy...

Pediatric hemorrhagic stroke (HS) accounts for a large proportion of childhood strokes, 1 of the top 10 causes of pediatric deaths. Morbidity and mortality lead to significant socio-economic and psychosocial burdens. To understand published data on recognizing and managing children with HS, we conducted a systematic review of the literature presented here. We searched PubMed, Embase, CINAHL and the Cochrane Library databases limited to English language and included 174 studies, most conducted in the USA (52%)...

The field of pediatric stroke has historically been hampered by limited evidence and small patient cohorts. However the landscape of childhood stroke is rapidly changing due in part to increasing awareness of the importance of pediatric stroke and the emergence of dedicated pediatric stroke centers, care pathways, and alert systems. Acute pediatric stroke management hinges on timely diagnosis confirmed by neuroimaging, appropriate consideration of recanalization therapies, implementation of neuroprotective measures, and attention to secondary prevention...

Moyamoya is a progressive cerebrovascular disorder that leads to stenosis of the arteries in the distal internal carotid, proximal middle cerebral and proximal anterior cerebral arteries of the circle of Willis. Typically a network of collaterals form to bypass the stenosis and maintain cerebral blood flow. As moyamoya progresses it affects the anterior circulation more commonly than posterior circulation, and cerebral blood flow becomes increasingly reliant on external carotid supply. Children with moyamoya are at increased risk for ischemic symptoms including stroke and transient ischemic attacks (TIA)...

Up to more than half of previously healthy children presenting with their first arterial ischemic stroke have a cerebral arteriopathy. Cerebral arteriopathies during childhood can be congenital, reflecting abnormal vessel development, or acquired when caused by disruption of vascular homeostasis. Distinguishing different types of cerebral arteriopathies in children can be challenging but of great clinical value as they may dictate different disease and treatment courses, and clinical and radiologic outcomes...

Given the advancements in prenatal testing, child neurologists are becoming involved in earlier stages of patient care, often being consulted during the gestational stage rather than during the postnatal period. Thus, it is essential that pediatric neurologists understand the strengths and limitations of prenatal testing when counseling families. In this review we separate prenatal testing into screening and diagnostic testing. On the one hand, screening testing is noninvasive and does not have an increased risk for miscarriage...

The risk of seizure is increased in premature neonates compared to full term infants, with a distinct profile of etiologies, timing and character. Despite improvements in neonatal care, preterm infants with seizure continue to have higher risk of abnormal neurodevelopmental outcomes when compared to preterm infants without seizures, or to full term infants with seizures. Very limited evidence guides the care of this challenging population, therefore, management of the preterm neonate with seizure is largely extrapolated from the care of full-term neonates...

Abnormal neurodevelopmental outcomes are associated with multiple factors including prematurity, intrauterine infection, maternal comorbidities as well as fetal anomalies. Within the past decade, new standardized terminology in placental pathology has emerged, emphasizing the current understanding of processes that play a role in placental dysfunction. Factors playing a major role in the abnormal development of the placenta include abnormalities in blood flow and perfusion of the fetal and maternal compartments of the placenta termed fetal vascular malperfusion and maternal vascular malperfusion, respectively...

Perinatal palliative care (PnPC) focuses on enhancing family and newborn quality-of-life in the setting of a life-limiting fetal condition while simultaneously ensuring medical care delivery that is soundly in line with the family's goals and values. The inclusion of PnPC in the multidisciplinary care of a family facing severe fetal neurologic diagnoses allows for skilled exploration of the values and experience that ultimately drive creation of goals of care. The parental experience of receiving a life-limiting fetal diagnosis pushes parents into an emotional journey, which typically follows a recognizable progression of stages...

Fetal and neonatal neurology is increasingly recognized as a subspecialty within child neurology and fellowship training programs are emerging. Most child neurologists have not received formal training in the interpretation of fetal data and the practice of fetal neurology consultation. However, they can be valuable members of the fetal care team and bring important perspective to the diagnosis of fetal neurologic conditions. With a systematic approach and a planned format for counseling, child neurologists without formal training in fetal consultations can apply their postnatal neurology expertise to the prenatal neurology patient...

In this article we review the last 40 years of progress in fetal neurosurgery with special attention to current controversies and upcoming challenges in the field. We surveyed the published literature describing prenatal interventions for spinal malformations, cerebral malformations, and hydrocephalus. Even the most mature treatment paradigm, intrauterine repair of myelomeningocele, stands to benefit from advances in imaging and therapeutic modalities to improve patient selection, refine surgical techniques, validate novel biologic therapies, and streamline postoperative patient care...

Gene-environment (G x E) interactions significantly influence neurologic outcomes. The maternal-placental-fetal (MPF) triad, neonate, or child less than 2 years may first exhibit significant brain disorders. Neuroplasticity during the first 1000 days will more likely result in life-long effects given critical periods of development. Developmental origins and life-course principles help recognize changing neurologic phenotypes across ages. Dual diagnostic approaches are discussed using representative case scenarios to highlight time-dependent G x E interactions that contribute to neurologic sequelae...

Neurogenetic and metabolic diseases often present in the neonatal period, masquerading as other disorders, most commonly as neonatal encephalopathy and seizures. Advancements in our understanding of inborn errors of metabolism are leading to an increasing number of therapeutic options. Many of these treatments can improve long-term neurodevelopment and seizure control. However, the treatments are frequently condition-specific. A high index of suspicion is required for prompt identification and treatment. When suspected, simultaneous metabolic and molecular testing are recommended along with concurrent treatment...

Neonatal hydrocephalus represents an important pathological condition with significant impact on medical care and neurocognitive development. This condition requires early recognition, appropriate medical and surgical management, and long-term surveillance by clinicians and pediatric neurosurgeons. Common etiologies of neonatal and infant hydrocephalus include intraventricular hemorrhage related to prematurity with subsequent post-hemorrhagic hydrocephalus, myelomeningocele, and obstructive hydrocephalus due to aqueductal stenosis...

No abstract text is available yet for this article.

The severe acute respiratory syndrome coronavirus 2 pandemic has markedly, and likely permanently, changed health care. This includes changing the obstetric and perinatal care of mothers and infants, and by extension, the care of their families. Infection during pregnancy is associated with an increased risk for severe coronavirus disease 2019 illness and related complications that can significantly impact maternal health and the health of the neonate. Viral transmission from mother to fetus is possible, but rare during pregnancy, and current health care policies focusing on maternal masking, and hand washing allows infected mothers to safely care for neonates (including nursing or feeding with expressed breast milk)...

Congenital brain malformations are abnormalities present at birth that can result from developmental disruptions at various embryonic or fetal stages. The clinical presentation is nonspecific and can include developmental delay, hypotonia, and/or epilepsy. An informed combination of imaging and genetic testing enables early and accurate diagnosis and management planning. In this article, we provide a streamlined approach to radiologic phenotyping and genetic evaluation of brain malformations. We will review the clinical workflow for brain imaging and genetic testing with up-to-date ontologies and literature references...