Stroke Prevention and Treatment for Youth with Sickle Cell Anemia: Current Practice and Challenges and Promises for the Future.

Purpose Of Review: Sickle cell anemia (SCA) is an autosomal recessive inherited hemoglobinopathy that results in a high risk of stroke. SCA primarily affects an underserved minority population of children who are frequently not receiving effective, multi-disciplinary, preventative care. This article reviews primary and secondary stroke prevention and treatment for children with SCA for the general adult and pediatric neurologist, who may play an important role in providing critical neurologic evaluation and care to these children.

Childhood stroke.

Stroke is an important cause of neurological morbidity in children; most survivors have permanent neurological deficits that affect the remainder of their life. Stroke in childhood, the focus of this Primer, is distinguished from perinatal stroke, defined as stroke before 29 days of age, because of its unique pathogenesis reflecting the maternal-fetal unit. Although approximately 15% of strokes in adults are haemorrhagic, half of incident strokes in children are haemorrhagic and half are ischaemic.

Multicenter Research Data of Epilepsy Management in Patients With Sturge-Weber Syndrome.

Background: Epilepsy in typical Sturge-Weber syndrome (SWS) is common, and many questions remain regarding the treatment outcomes. We analyzed a large multicenter database with focus on neurological drug treatment in different demographic and SWS characteristic groups.

Methods: A total of 268 patients with brain involvement and a history of seizures were selected from a research data registry generated from a multicenter cross-sectional questionnaire.

Burden of Neurological Disorders Across the US From 1990-2017: A Global Burden of Disease Study.

Importance: Accurate and up-to-date estimates on incidence, prevalence, mortality, and disability-adjusted life-years (burden) of neurological disorders are the backbone of evidence-based health care planning and resource allocation for these disorders. It appears that no such estimates have been reported at the state level for the US.

Objective: To present burden estimates of major neurological disorders in the US states by age and sex from 1990 to 2017.

Comparative study of posterior and anterior circulation stroke in childhood: Results from the International Pediatric Stroke Study.

Objective: To compare risk factors, clinical presentation, and outcomes after posterior circulation arterial ischemic stroke (PCAIS) and anterior circulation arterial ischemic stroke (ACAIS) in neonates and children.

Methods: In this international multicenter observational study including neonates and children up to 18 years of age with arterial ischemic stroke (AIS), we compared clinical and radiologic features according to stroke location.

Results: Of 2,768 AIS cases, 507 (18%) were located in the posterior circulation, 1,931 (70%) in the anterior circulation, and 330 (12%) involved both.

Physical and Family History Variables Associated With Neurological and Cognitive Development in Sturge-Weber Syndrome.

Background: Sturge-Weber syndrome (SWS) is caused by a somatic mutation in GNAQ leading to capillary venous malformations in the brain presenting with various neurological, ophthalmic, and cognitive symptoms of variable severity. This clinical variability makes accurate prognosis difficult. We hypothesized that the greater extent of physical factors (extent of skin, eye, and brain involvement), presence of possible genetic factors (gender and family history), and age of seizure onset may be associated with greater symptom severity and need for surgery in patients with SWS.

Global, Regional, and Country-Specific Lifetime Risks of Stroke, 1990 and 2016.

Background: The lifetime risk of stroke has been calculated in a limited number of selected populations. We sought to estimate the lifetime risk of stroke at the regional, country, and global level using data from a comprehensive study of the prevalence of major diseases.

Methods: We used the Global Burden of Disease (GBD) Study 2016 estimates of stroke incidence and the competing risks of death from any cause other than stroke to calculate the cumulative lifetime risks of first stroke, ischemic stroke, or hemorrhagic stroke among adults 25 years of age or older.

Child and Adolescent Health From 1990 to 2015: Findings From the Global Burden of Diseases, Injuries, and Risk Factors 2015 Study.

Importance: Comprehensive and timely monitoring of disease burden in all age groups, including children and adolescents, is essential for improving population health.

Objective: To quantify and describe levels and trends of mortality and nonfatal health outcomes among children and adolescents from 1990 to 2015 to provide a framework for policy discussion.

Evidence Review: Cause-specific mortality and nonfatal health outcomes were analyzed for 195 countries and territories by age group, sex, and year from 1990 to 2015 using standardized approaches for data processing and statistical modeling, with subsequent analysis of the findings to describe levels and trends across geography and time among children and adolescents 19 years or younger.

Arterial Ischemic Stroke in Children and Young Adults.

Purpose Of Review: This article reviews risk factors, recurrence risk, evaluation, management, and outcomes of arterial ischemic stroke in children and young adults.

Recent Findings: The risk for recurrence and mortality appear to be low for neonatal and childhood stroke. Most children have relatively mild deficits, but those who have greater neurologic deficits, poststroke epilepsy, or strokes early in life are at risk for lower overall cognitive function.

Recurrent Fat Embolic Strokes in a Patient With Duchenne Muscular Dystrophy With Long Bone Fractures and a Patent Foramen Ovale.

Background: Individuals with Duchenne muscular dystrophy have an increased risk of long bone fractures. Such fractures are sometimes associated with brain dysfunction due to fat embolism syndrome, although this syndrome has seldom been documented in muscular dystrophy patients.

Patient Description: We describe a child with Duchenne muscular dystrophy who developed fat embolism syndrome with neurological dysfunction following multiple long bone fractures.

Herpesvirus Infections and Childhood Arterial Ischemic Stroke: Results of the VIPS Study.

Background: Epidemiological studies demonstrate that childhood infections, including varicella zoster virus, are associated with an increased risk of arterial ischemic stroke (AIS). Other herpesviruses have been linked to childhood AIS in case reports. We sought to determine whether herpesvirus infections, which are potentially treatable, increase the risk of childhood AIS.

Noninvasive brain stimulation: the potential for use in the rehabilitation of pediatric acquired brain injury.

Noninvasive brain stimulation (NIBS) offers the potential to modulate neural activity and recovery after acquired brain injury. There are few studies of NIBS in children, but a survey of those studies might provide insight into the potential for NIBS to modulate motor rehabilitation, seizures, and behavior in children. We surveyed the published literature prior to July 2014 for articles pertaining to children and NIBS with a focus on case series or trials.

Out-of-pocket costs for childhood stroke: the impact of chronic illness on parents' pocketbooks.

Objective: Direct costs for children who had stroke are similar to those for adults. There is no information regarding the out-of-pocket costs families encounter. We described the out-of-pocket costs families encountered in the first year after a child's ischemic stroke.

Recurrent central nervous system white matter changes in charcot-Marie-tooth type X disease.

Introduction: X-linked Charcot-Marie-Tooth (CMT1X) disease is caused by mutations in the GJB1 gene. We describe a young man who presented with recurrent central nervous symptoms and transient white matter changes in the setting of a novel mutation in the GJB1 gene.

Methods: Evaluation included clinical examination, neuroimaging, electrophysiological, and molecular genetic studies.