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Medial medullary syndrome (aka Dejerine syndrome) is a rare condition that develops following infarction of the medial medulla and is classically defined by the presence of Dejerine's triad of contralateral weakness in upper and lower extremities, contralateral hemisensory loss of vibration and proprioception, and ipsilateral tongue weakness. It is typically caused by occlusion of the vertebral artery or one of its branches. We report the case of a 6-year-old girl who suffered a medial medullary infarction, and she was diagnosed with atypical Dejerine syndrome. Medial medullary infarct leading to atypical Dejerine syndrome has not been reported in this young of a patient in the literature to date. [ABSTRACT FROM AUTHOR]

Acute ischemic stroke (AIS) in childhood is a relatively rare but significant condition that can result in long-term disabilities. There is a lack of standardized strategies for diagnosing and treating pediatric AIS due to limited evidence-based data on thrombolytic and endovascular treatments in children. This comprehensive literature review focuses on the experience of a single center in Italy and aims to highlight the main peculiarities of endovascular treatment (EVT) for AIS in childhood. The review covers the diagnostic workup, the endovascular procedures, and the need for a specific thrombectomy program for pediatric AIS. The review discusses the indications and considerations for thrombectomy in children, including the risk of complications and the challenges of extrapolating results from adult studies. The diagnostic protocols for pediatric AIS are also discussed, emphasizing the use of MRI to avoid X-ray and contrast medium exposure in children. The combination of intravenous thrombolysis and mechanical thrombectomy has been examined, considering the differences between pediatric and adult thrombi. Technical considerations related to the size of pediatric patients are addressed, including the use of large bore catheters and potential concerns with access points. The organization of a thrombectomy program for pediatric AIS is discussed, emphasizing the need for specialized facilities and expertise. Although evidence for EVT in the pediatric population is based on case series, the importance of specialized centers and the lack of validated guidelines are evident., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2023 The Authors. Published by Elsevier Ltd.)

Most children admitted to the pediatric intensive care unit with a new or reoccurring hematology or oncology diagnosis are at high risk for developing a hematologic or oncologic emergency. Although these children represent a low percentage of pediatric critical care admissions, their acuity is high, and their care is complicated and challenging. Nurses are an essential part of the interprofessional team of providers who care for these critically ill patients. Experience recognizing acute decompensation and excellent assessment and communication skills significantly improve patient outcomes., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Abstract Background There are few studies evaluating risk factors for poststroke epilepsy (PSE) after an arterial ischemic stroke (AIS) in childhood. This study aimed to evaluate clinical and radiological predictors for PSE in a cohort of children with a first-ever AIS. Methods A retrospective analysis of a single-center prospective consecutive cohort of children beyond neonatal age with a first-ever AIS admitted at the Pontifical Catholic University of Chile's Clinical Hospital between 2003 and 2013. All participants had a brain magnetic resonance imaging at the time of diagnosis. All children underwent follow-up for at least three years with an annual clinical evaluation. We used the current epilepsy definition of the International League Against Epilepsy. Studied variables include demographics, clinical manifestations at onset, stroke risk factors, and radiological characteristics of AIS. Cox proportional hazards regression analysis was used to evaluate PSE risk adjusted for clinical and radiological variables. Results Among 98 children who met the study criteria, 41 (41.8%) with PSE. Following multivariate analysis, it was determined that the predictors of PSE include young age at AIS (hazard ratio [HR] = 0.91; confidence interval [CI] = 0.84–0.99), the occurrence of acute symptomatic seizures (HR = 3.29; CI = 1.35–8.01), cortical infarction (HR = 5.01; CI = 2.00–12.6), and multifocal infarction (HR = 3.27; CI = 1.01–10.8). Conclusion Seizures, young age, cortical lesions, and multiple infarction at the time of stroke are independent risk factors for PSE in children following a first-ever AIS. Highlights • This cohort study evaluates risk factors for epilepsy following a first-ever arterial ischemic stroke during the childhood. • We identify both clinical and radiological predictors of post-stroke epilepsy in children beyond neonatal age. • This study adds new information regarding predictors of post-stroke epilepsy in childhood. [ABSTRACT FROM AUTHOR]

Abstract Background and purpose Safety and efficacy of intravenous (IV) thrombolysis and endovascular therapy in children with acute ischemic stroke (AIS) are unknown to date. We aimed to review and synthesize currently available evidence on these acute recanalization therapies in pediatric stroke patients. Methods We performed a systematic review and meta-analysis of all available data on safety and efficacy of acute treatment including thrombolysis and endovascular therapy in pediatric AIS patients aged <18 years. We searched the electronic databases Medline and Cochrane Library for eligible studies published from the earliest date available until August 31, 2016. Safety outcomes included intracerebral hemorrhage (ICH) post-treatment and in-hospital mortality. Efficacy outcomes included functional outcome 3–6 months after index stroke. Results We identified 222 records, of which 3 studies with a total of 16,987 pediatric stroke patients met our eligibility criteria of whom 181 received IV thrombolysis. No data exists from randomized trials and no data is available on endovascular thrombectomy. Risk of any ICH was increased in children receiving thrombolysis (risk ratio = 3.48, 95%CI: 1.66–7.29; p = 0.001) compared with controls, with no evidence of heterogeneity (I 2 = 0%). None of the included studies reported complete data on symptomatic ICH. In-hospital mortality was similar between pediatric stroke patients treated with thrombolysis and controls (risk ratio = 1.44, 95%CI: 0.39–5.40; p = 0.586), with evidence of heterogeneity (I 2 = 62%). Efficacy of revascularization therapies could not be analyzed due to lack of outcome data. Conclusions Our analyses demonstrate a substantial lack of data on efficacy and safety of acute recanalization therapies in children with AIS. PROSPERO Registration Information URL: http://www.crd.york.ac.uk/PROSPERO. Unique identifier: CRD42016047140. Highlights • Substantial lack of evidence for recanalization therapies in children with acute ischemic stroke. • No adequate data on occurrence of symptomatic intracerebral hemorrhage following recanalization therapy. • Non-uniformity in post-stroke assessment of functional recovery complicates any attempt to synthesize data on efficacy. [ABSTRACT FROM AUTHOR]

Dystonia is a movement disorder that involves excessive, involuntary muscle contractions resulting in repetitive movements and/or abnormal posturing. One common cause of unilateral dystonia in childhood is ischemic stroke involving the basal ganglia and/or thalamus. Virtually nothing is known about neuropsychological outcomes in children who have dystonia following basal ganglia stroke. The present study explored whether or not children with secondary dystonia experience additional cognitive challenges when compared to children with similar patterns of brain injury, but no dystonia. We examined intellectual function, academics, and several aspects of executive function in children with unilateral basal ganglia stroke during childhood, comparing those with dystonia and those without. Although groups did not differ in terms of lesion size, we found significantly lower performance on measures of verbal and nonverbal reasoning, inhibitory control, and academic ability in children with secondary dystonia compared to those without. In contrast, there were no significant group differences on parent ratings of their child’s executive function in daily life. These findings suggest that maladaptive reorganization following basal ganglia stroke may contribute to the development of secondary dystonia and also to poor intellectual and academic outcomes in this group. [ABSTRACT FROM AUTHOR]

Objective: To determine the outcome and prognostic factors of stroke in children presenting at Military Hospital Rawalpindi. Study Design: Cross-sectional study. Place and Duration of Study: This study was conducted at the department of Pediatrics, Military Hospital Rawalpindi, from Oct 2012 to Mar 2014. Patients and Methods: Sixty consecutive children presenting with stroke were included in this study after taking written informed consent from the guardians/parents. A predesigned proforma was used to record patient’s demographic details along with the presenting complaints, type of stroke, underlying cause and outcome. Results: The mean age of the patients was 3.49 ± 3.29 (Mean ± SD) years. There were 35 (58.3%) male and 25 (41.7%) female children. Ischemic stroke was the most frequent and was observed in 37 (61.7%) patients followed by hemorrhagic (16.7%), sinovenous thrombosis (8.3%) and ischemia with hemorrhagic findings (6.7%). Mixed lesions and transient ischemic attacks were reported in 2 (3.3%) patient each. Mean length of hospital stay was 9 ± 6 (Mean ± SD) days. Sixteen (26.7%) children recovered completely while 41 (68.3%) children had some neurological deficit at discharge. Mild to moderate deficit was recorded in 21 (35.0%) children while 20 (33.3%) children had severe deficit. Three (5.0%) patients expired during hospital stay. Conclusion: Ischemic stroke was the most common cause of paediatric stroke. Important risk factors of peadiatric stroke included congenital heart diseases and intracranial infections. Poor prognostic factors included male gender, age less 5 years and congenital heart disease. [ABSTRACT FROM AUTHOR]

Abstract CD59 is involved in lymphocyte signal transduction and regulates complement-mediated cell lysis by inhibiting the membrane attack complex. In the cases reported so far, congenital isolated CD59 deficiency was associated with recurrent episodes of hemolytic anemia, peripheral neuropathy, and strokes. Here, we report on a patient from a consanguineous Turkish family, who had a first episode of hemolytic anemia at one month of age and presented at 14 months with acute Guillain-Barré syndrome (GBS). The child suffered repeated infection-triggered relapses leading to the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Although partly steroid-responsive, the polyneuropathy failed to be stabilized by a number of immunosuppressive agents. At the age of 6 years, he developed acute hemiparesis and showed progressive stenosis of proximal cerebral arteries, evolving into Moyamoya syndrome (MMS) with recurrent infarctions leading to death at 8 years of age. Post-mortem genetic analysis revealed a pathogenic p.(Asp49Valfs*31) mutation in CD59. Re-analysis of brain biopsy specimens showed absent CD59 expression and severe endothelial damage. Whereas strokes are a known feature of CD59 deficiency, MMS has not previously been described in this condition. Therefore, we conclude that in MMS combined with hemolysis or neuropathy CD59 deficiency should be considered. Establishing the diagnosis and targeted therapy with eculizumab might have prevented the lethal course in our patient. Highlights • Congenital isolated CD59 deficiency leads torecurrent episodes of hemolytic anemia, peripheral neuropathy, and strokes. • In this 4th patient with thep.Asp49Valfs*31 mutation in CD59 reported, brain endothelium showed absent CD59 expression. • Moyamoya syndrome developed while systemicvasculitis could be excluded. • Targeted therapy against complement factor C5 with eculizumab might have prevented the lethal course in our patient. [ABSTRACT FROM AUTHOR]

This cross-sectional retrospective clinical research study examines a large group of children followed within a pediatric stroke program and a developmental attention-deficit/hyperactivity disorder (ADHD) clinic at the Hospital for Sick Children, between May 2004 and June 2016. All children with a history of stroke who participated in a neuropsychological assessment between the ages of 4 and 18 years were considered for inclusion. From a sample of 275 participants with a history of stroke, 36 children (13.1%) received a diagnosis of secondary ADHD. Children with secondary ADHD were younger at the time of stroke and more likely to be identified as having a presumed perinatal stroke and persistent seizures than children without secondary ADHD diagnoses. There were no differences in pattern of lesion, size, or laterality between children who developed secondary ADHD and those who did not. Children with secondary ADHD had the lowest scores across all cognitive and academic measures compared to children with stroke-only and developmental ADHD. Findings highlight the added risk of receiving a diagnosis of secondary ADHD following pediatric stroke. Implications for future research and directed intervention are discussed. [ABSTRACT FROM AUTHOR]

Pseudoxanthoma elasticum (PXE) is a rare genetic disorder characterized by fragmented and mineralized elastic fibers in the mid-dermis of the skin, eye, digestive tract and cardiovascular system. Clinical presentation includes typical skin lesions, ocular angioid streaks, and multisystem vasculopathy. The age of onset varies considerably from infancy to old age, but the diagnosis is usually made in young adults due to frequent absence of pathognomonic skin and ocular manifestations in early childhood. We report two children with PXE presenting with isolated multisystem vasculopathy and early-onset stroke. In the first patient, diagnosis was delayed until typical dermatologic alterations appeared; in the second patient, next-generation sequencing (NGS) study led to early diagnosis and specific follow-up, underlying the crucial role in idiopathic pediatric stroke of early genetic testing using NGS-based panels. [ABSTRACT FROM AUTHOR]

Background: Pediatric ischemic stroke is an important cause of morbidity and mortality. As previous studies of children after stroke showed, dyslipidemias were very common in Polish and other European populations. Thus, looking for genetic factors predisposing to pediatric stroke, its symptoms, and outcome, we have analyzed 2 polymorphisms of the upstream stimulating factor 1 (USF-1) gene.Materials and Methods: The study group consisted of 82 children with stroke, 156 parents, and 146 controls. We used 2 alternative methods: the case-control model and the analysis of families using the transmission disequilibrium test. The 2 polymorphisms, rs2516839 and rs3737787, were genotyped using the TaqMan Pre-Designed SNP Genotyping Assay. The Statistica 10.0 software was used in all statistical analyses.Results: We did not observe any statistical differences in genotype and allele frequencies between patients and controls. There were also no significant differences in the transmission of alleles from the parents to the affected children. However, we have observed that the TT genotype of the rs2516839 polymorphism was more common in patients with epilepsy and dysarthria, whereas the TT genotype of the rs3737787 polymorphism was more frequent in the group of patients with a decrease in intellectual functioning.Conclusions: Our study did not show any associations between the 2 analyzed polymorphisms of the USF-1 gene and pediatric ischemic stroke. However, we have observed an influence of specific genotypes on the outcome of stroke, including epilepsy, dysarthria, and a decrease in intellectual functioning. [ABSTRACT FROM AUTHOR]

Background: Risk factors for pediatric stroke are poorly understood and require study to improve prevention. Total cholesterol and triglyceride values peak to near-adult levels before puberty, a period of increased stroke incidence. The role of lipids in childhood arterial ischemic stroke has been minimally investigated.Methods: We performed a cross-sectional analysis of lipid and Lp(a) concentrations in children with arterial ischemic stroke in the International Pediatric Stroke Study to compare the prevalence of dyslipidemia and high- or low-ranking lipid values in our dataset with reported population values. We analyzed sex, body mass index, race, ethnicity, family history, and stroke risk factors for associations with dyslipidemia, high non-high-density lipoprotein cholesterol, and hypertriglyceridemia.Results: Compared with the National Health and Nutrition Examination Survey, a higher proportion of children ≥5 years with arterial ischemic stroke had dyslipidemia (38.4% versus 21%), high total cholesterol (10.6% versus 7.4%), high non-high-density lipoprotein cholesterol (23.1% versus 8.4%), and low high-density lipoprotein cholesterol (39.8% versus 13.4%). The lipid values that corresponded to one standard deviation above the mean (84th percentile) in multiple published national studies generally corresponded to a lower ranking percentile in children aged five years or older with arterial ischemic stroke. Dyslipidemia was more likely associated with an underweight, overweight, or obese body mass index compared with a healthy weight. Ethnic background and an acute systemic illness were also associated with abnormal lipids.Conclusions: Dyslipidemia and hypertriglyceridemia may be more prevalent in children with arterial ischemic stroke compared with stroke-free children. [ABSTRACT FROM AUTHOR]

Background Cerebrovascular complications of Lyme neuroborreliosis (LNB) are poorly documented in the paediatric population. Methods We performed a retrospective analysis from prospectively registered cases of acute ischemic stroke (AIS) from the Swiss NeuroPaediatric Stroke Registry (SNPSR) from 2000 to 2015. Only cases with serologically confirmed LNB were included. In addition, a literature review on paediatric stroke cases secondary to Lyme neuroborreliosis in the same time frame was performed. Results 4 children out of 229 children with arterial ischemic childhood stroke and serologically confirmed LNB were identified in the SNPSR giving a global incidence of 1.7%. Median age was 9.9 years. A prior history of tick bites or erythema migrans (EM) was reported in two cases. Clinical presenting signs were suggestive of acute cerebellar/brainstem dysfunction. On imaging, three children demonstrated a stroke in the distribution of the posterior inferior cerebellar artery. The remaining fourth child had a “stroke-like” picture with scattered white matter lesions and a multifocal vasculitis with prominent basilar artery involvement. Lymphocytic pleocytosis as well as intrathecal synthesis of Borrelia burgdorferi antibodies were typical biological features. Acute intravenous third generation cephalosporins proved to be effective with rapid improvement in all patients. No child had recurrent stroke. Data from the literature concerning eight patients gave similar results, with prominent posterior circulation stroke, multifocal vasculitis and abnormal CSF as distinctive features. Conclusions Lyme Neuroborreliosis accounts for a small proportion of paediatric stroke even in an endemic country. The strong predilection towards posterior cerebral circulation with clinical occurrence of brainstem signs associated with meningeal symptoms and CSF lymphocytosis are suggestive features that should rapidly point to the diagnosis. This can be confirmed by appropriate serological testing in the serum and CSF. Clinicians must be aware of this rare neurological complication of Lyme disease that demands specific antibiotic treatment. [ABSTRACT FROM AUTHOR]

Acute ischemic stroke (AIS) in children is rare with almost 40% diagnosed as cryptogenic. One possible mechanism associated with AIS is an elevated Lipoprotein (a) [Lp(a)] level. Here, we discuss the case of an 11-year old boy who presented with multiple thrombotic strokes secondary to elevated Lp(a), which was identified as the only risk factor and immediately treated with lipoprotein apheresis (LA). Eighteen months post-AIS, he is still receiving LA treatments and has made remarkable progress in his recovery without another cerebrovascular event. [ABSTRACT FROM AUTHOR]

Introduction and aim. Childhood stroke affects 2-8 per 100,000 children each year. It can occur at any age, including newborns, infants, and prenatally. Despite the possible serious consequences of untreated pediatric stroke, there are still no clear guidelines for the management of this disease entity. We describe a case of ischemic stroke in a child treated by mechanical thrombectomy. Description of the case. A 14-year-old previously healthy patient was admitted to the Department of Pediatric Neurology because of sudden onset of drooping of the left corner of the mouth and weakness of the left limbs, which had occurred 5 hours earlier. An angio-CT showed occlusion of the internal carotid artery and right middle cerebral artery. The patient was qualified for treatment by mechanical thrombectomy. During the procedure, a dissection of the internal carotid artery in the extracranial section was diagnosed -- a stent was implanted in this place. Reperfusion of the occluded arteries was achieved. An improvement of neurological condition was observed, with left limb paresis resolving in the following days. The patient was transferred to the Rehabilitation Clinic with further improvement. Conclusion. Although mechanical thrombectomy is the standard treatment of ischemic stroke among adults, there are no guidelines for treating this condition in childhood to date. The use of reperfusion treatment may lead to reduction of disability and mortality rates in this group of patients as well. However, more data are needed to confirm this assumption. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the relationship between hematological indices and iron status with the infarct size, number and location in Pediatric stroke. Material and methods: Observational cross sectional study. 16 (8 Male and 8 Female) pediatric stroke patients presenting in Pediatric Neurology clinic included in the study. Hemoglobin (Hb) level, Hematocrit (HCT) and RBC indices (MCV, MCH and MCHC), platelet count and Iron status parameters (Serum Iron, TIBC and Serum Ferritin) of these patients were analysed. Area of diffusion restriction on MRI brain was measured as a surrogate marker of the severity of infarction and its correlation with RBC indices, platelet count and Iron status parameters was evaluated. Results: Mean age of studied population at presentation was 45.218±47.80 months. Mean Hb, HCT, MCV, MCH, MCHC and platelet count of these patients were 9.229±2.829gm/dl, 29.531±7.002%, 73.650 ±7.237fL, 23.650 ±.3.709pg, 30.393 ±3.000 g/dl, 331.667±201.472 x109/L respectively. Mean Serum Ferritin, TIBC and Serum Iron were 89.639 ±119.788 ng/ml, 286.125± 83.858 ug/dl, and 82.375± 59.452 ug/dl. No statistically significant correlation was seen between any of the RBC indices, Total Platelet Count and Serum Iron status indicators with mean total area of diffusion restriction in the whole cohort, in the group of patients having Serum Ferritin levels 15 ng/ml. Conclusion: Despite the fact that most of the literature reports association between Iron deficiency anemia with stroke, we did not find any correlation between the various RBC indices, total Platelet count and Iron status indicators with area of diffusion restriction in patients with stroke. Further studies are recommended to study the exact contributors to patho - physiology of stroke associated with Iron deficiency.

Acute basilar artery occlusion (ABAO) is an infrequent but potentially fatal complication that can cause strokes in both adults and children. Traumatic vertebral artery dissection (VAD) is one of the most common causes of ABAO in young patients. We present a case of an 11-year-old boy with VAD complicated by basilar artery occlusion 2 days after a fight with classmates that caused severe neurological deficits. He did not have any direct head trauma or concomitant risk factors. Clinical symptoms included nausea, vomiting, and rapid alteration of consciousness. Magnetic resonance imaging showed total occlusion of the basilar artery, and angiography confirmed VAD from the third to the fourth segments. A history of such subtle precipitating events should be noted when diagnosing young patients with brainstem strokes. A delay in the diagnosis of ABAO is frequently due to misleading symptoms and signs and the lack of awareness of this rare condition.

Objective To retrospectively evaluate the midterm therapeutic effect of enlarged encephalo-duro-myo-synangiosis (EDMS) for moyamoya disease (MMD) in young children. Methods Seventy-seven children diagnosed with MMD by digital subtraction angiography (DSA) or magnetic resonance angiography (MRA) were treated between January 2011 and December 2014 in our center. Their clinical features, imaging, and operative reports were analyzed. Results Four patients presented with intracerebral hemorrhage, whereas 73 presented with ischemic symptoms. Revascularization procedures were performed unilaterally on 11 left hemispheres and 9 right hemispheres, and 57 had bilateral surgeries. The average length of surgery was 143 ± 24 minutes, with 28 ± 9 mL of blood loss. The mean period of follow-up was 28.43 ± 15.31 months. Cerebral blood flow increased 3 months after the operations in the previously affected regions. Collateralization from the deep temporal artery, superficial temporal artery, and the middle meningeal artery was found by DSA or MRA. In cases where single-photon emission computed tomography was obtained, it demonstrated better perfusion postoperatively. The ischemic symptoms were relieved in 118 (88.06%) hemispheres, and remained stable in 5 (3.73%) hemispheres. There were 12 radiographic cerebral infarctions (8.96%) within 1 month postoperatively. Conclusions Enlarged EDMS is safe and effective for MMD in young children. Extensive and multilayered revascularization could significantly preserve neurologic function. The long-term effect on posterior circulation disease development needs further investigation. [ABSTRACT FROM AUTHOR]

Noninvasive brain stimulation has been widely used for adults with stroke to improve upper limb motor function. A recent study by Kirton and colleagues (Kirton A, Ciechanski P, Zewdie E, Andersen J, Nettel-Aguirre A, Carlson H, Carsolio L, Herrero M, Quigley J, Mineyko A, Hodge J, Hill M. Neurology 88: 259-267, 2017) applied noninvasive brain stimulation to children with congenital hemiparesis but found no significant effect of noninvasive brain stimulation on motor function. Here, we explore theories about cortical reorganization in both adult and children with hemiparesis and discuss how to improve the approaches of noninvasive brain stimulation to generate optimal motor improvement and development for children with congenital hemiparesis. [ABSTRACT FROM AUTHOR]

In this paper we present a case study in which a 14-year-old, right-handed stroke patient with severe weakness, spasticity, and motor dysfunction of the left upper extremity participated in a three-month distal robotic training program. The robotic device was compliant to the patient’s movements and was able to modulate the level of assistance continuously throughout the trial (i.e., online adaptive modulation). Standard clinical and robotic evaluations of upper extremity motor performance were conducted before and after robotic training. There were improvements in upper extremity spasticity and motor functions. In addition, robotic training lead to positive changes in wrist active range of motion and kinematics: movements were smoother and there was a noticeable decrease in the level of robotic intervention required to complete each trial. In sum, results of the present case study demonstrate that distal upper extremity robotic rehabilitation that features the proposed adaptive control algorithm promoted positive changes in upper limb motor coordination and function after pediatric stroke. [ABSTRACT FROM AUTHOR]

Purpose: Pediatric cerebral sinovenous thrombosis (CSVT) is an important, though less common subtype of pediatric stroke. It has been linked to several risk factors, including cranial procedures, with few studies highlighting this relationship. The aim of this study was to characterize the diagnosis and treatment of CSVT after cranial surgery. Methods: An institutional pediatric stroke research database was used to identify all CSVT cases diagnosed within 30 days of cranial surgery from November 2004 to December 2014. Thirteen subjects were retrospectively analyzed for clinical presentation, surgical details, radiographic characteristics, laboratory study results, treatment, and outcome. Diagnostic testing and treatment adhered to a consensus-based institutional stroke protocol. Results: Cranial vault reconstruction, subdural empyema evacuation, and tumor resection were each observed in three subjects. Eleven (85%) subjects had sinus exposure during surgery, and eight (73%) developed thrombus in a sinus within or adjacent to the operative field. Two (15%) had documented iatrogenic sinus injury. On post-operative testing, ten (77%) subjects had prothrombotic abnormalities. Seven (54%) were treated with anti-coagulation therapy (ACT) starting on a median of post-operative day (POD) 3 (IQR 1-3) for a median of 2.9 months (IQR 2.4-5.4). Median time to imaging evidence of partial or complete recanalization was 2.4 months (IQR 0.7-5.1). No symptomatic hemorrhagic complications were encountered. Conclusions: Pediatric CSVT may be encountered after cranial surgery, and decisions related to anti-coagulation are challenging. The risk of CSVT should be considered in pre-surgical planning and post-operative evaluation of cases with known risk factors. In our study, judicious use of ACT was safe in the post-operative period. [ABSTRACT FROM AUTHOR]

Preterm infants born before 29 gestation weeks incur major risk of subependymal/intracerebral/intraventricular hemorrhage. In mice, neonate brain endothelial cells are more prone than adult cells to secrete proteases under glutamate challenge, and invalidation of the Serpine 1 gene is accompanied by high brain hemorrhage risk up to five days after birth. We hypothesized that the structural and functional states of microvessels might account for age-dependent vulnerability in mice up to five days after birth and might represent a pertinent paradigm to approach the hemorrhage risk window observed in extreme preterms. Mass spectrometry proteome analyses of forebrain microvessels at days 5, 10 and in adult mice revealed 899 proteins and 36 enriched pathways. Microarray transcriptomic study identified 5873 genes undergoing at least two-fold change between ages and 93 enriched pathways. Both approaches pointed towards extracellular matrix, cell adhesion and junction pathways, indicating delayed microvascular strengthening after P5. Furthermore, glutamate receptors, proteases and their inhibitors exhibited convergent evolutions towards excitatory aminoacid sensitivity and low proteolytic control likely accounting for vascular vulnerability in P5 mice. Thus, age vascular specificities must be considered in future therapeutic interventions in preterms. Data are available on ProteomeXchange (identifier PXD001718) and NCBI Gene-Expression-Omnibus repository (identification GSE67870). [ABSTRACT FROM AUTHOR]

The updated American Heart Association/American Stroke Association guidelines include recommendation for thrombectomy in certain adult stroke cases. The safety and efficacy of thrombectomy in children are unknown. An 8-year-old girl experienced acute stroke symptoms on two occasions while therapeutically anticoagulated on Novalung. Computed tomography scans showed proximal vessel thrombi, which were retrieved using a Trevo device without hemorrhagic complications. Postprocedural assessment found respective decreases in the National Institutes of Health Stroke Scale score from 10 to 4 and 12 to 7. The indications for treatment and early benefits observed in our case are consistent with other pediatric thrombectomy cases reported. However, publication bias and the heterogeneity of reported cases prevent drawing conclusions about the safety and efficacy of thrombectomy in children. Anticipating that updates to adult stroke guidelines would likely incite stroke providers to consider thrombectomy in children, our institution developed guidelines for thrombectomy before the index patient. Establishing institutional guidelines before considering thrombectomy in children may optimize patient safety. [ABSTRACT FROM AUTHOR]

Endovascular treatment (EVT) for large vessel, acute ischemic stroke in children remains a subject of debate, with evidence for its benefits derived from case series and individual case reports. At present, guidelines are cautious in recommending EVT for children under 5 years of age, mainly due to a lack of data and incomplete cerebral and femoral vessel development. However, based on the small number of cases reported in the literature, EVT appears to be safe and effective for pediatric use and arterial diameters can accommodate currently available devices. Available evidence shows that in patients with a normal arterial development, a 6-Fr femoral approach can be safely used by 2 years of age. We describe the case of a 2-year-old child who benefited from late-window EVT for a basilar occlusion. The procedure was safely performed using a 6-Fr femoral introducer, a 6-Fr guide catheter, and a 5-Fr aspiration catheter. We discuss also normal cerebral and femoral vessel development, providing descriptive tables of compatibility between femoral and cerebral arterial diameters by age groups and currently available endovascular devices. [ABSTRACT FROM AUTHOR]

• Perioperative pediatric moyamoya care practices vary substantially. • Experts restart antiplatelet agents at variable times after moyamoya surgery. • Equipoise exists regarding optimal postoperative blood pressure in moyamoya. • Optimal perioperative transfusion thresholds in children with moyamoya are unknown. Revascularization surgery decreases the long-term risk of stroke in children with moyamoya but carries an increased risk of perioperative ischemic events. Evidence-based approaches to safe perioperative management of children with moyamoya are limited. We aimed to understand practice variability in perioperative moyamoya care. Neurologists, neurosurgeons, and intensivists practicing in North America with expertise in perioperative pediatric moyamoya care participated in a 138-item anonymous survey focused on interdisciplinary perioperative care surrounding indirect revascularization surgery. Many perioperative care practices vary substantially between participants. Timing of resumption of antiplatelet therapy postoperatively, choice of sedative agents and vasopressors, goal blood pressures, rate and duration of intravenous fluid administration, and red blood cell transfusion thresholds are among the most variable practices. This practice variability survey highlights several important knowledge gaps and areas of equipoise that should be targets for future investigation and consensus-building efforts. [ABSTRACT FROM AUTHOR]

Mutations in the MYH11 gene result in smooth muscle cell dysfunction and are associated with familial thoracic aortic aneurysms and dissection. We describe a pediatric patient with a stroke and a pathogenic MYH11 IVS32G>A mutation, and a phenotype similar to ACTA2. A proband girl with an acute ischemic stroke underwent genetic analysis and 7T high-resolution MRI. A 12-year-old girl presented with a right middle cerebral artery occlusion. She received thrombolysis and underwent mechanical thrombectomy. An extensive stroke work-up was negative. A three-generation pedigree showed a splice site mutation of MYH11 IVS32G>A of the proband and three more family members. A 7T-MRI showed "broomstick-like" straightening of distal arterial segments, a V-shaped anterior corpus callosum and a post-stroke cystic area of encephalomalacia. This vascular appearance and parenchymal abnormalities typically present in patients with an ACTA2 phenotype. 7T-MRI also demonstrated thickening of the right middle cerebral arterial wall. This case suggests that MYH11 patients may have a similar angiographic and brain parenchymal phenotype to patients with ACTA2 mutations. This is the first report of arterial wall thickening in a MYH11 stroke patient using 7T-MRI. Patients with MYH11 mutations may display a focal cerebral steno-occlusive arteriopathy that may lead to stroke. [ABSTRACT FROM AUTHOR]

Background Sickle cell disease (SCD) is a common autosomal recessive inherited hemoglobin disorder in many countries. Neurological complications are among the most disabling complications in SCD. Stroke and cerebral vasculopathy can lead to further neurological insult. Ischemic insults, stroke, and silent infarcts are preventable causes of morbidity and mortality in SCD patients. Understanding the epidemiology and characteristics of such patients will help to prevent complications.  Methodology This is a retrospective study conducted in a tertiary care center in Saudi Arabia. Cases of SCD admitted to the pediatric ward between the years 2019 to 2023 were included in the study. Demographic data, clinical diagnosis, and frequency of prior admissions were collected. Brain imaging results were reviewed and included. Furthermore, the study assessed common risk factors leading to developing a stroke in SCD pediatric patients. Risk factors and clinical outcomes after stroke were also included. Results Eighty-one patients were enrolled. The mean age of stroke patients was 8.21±3.50 years while the mean age of non-stroke patients was 6.24±3.76 years. More than half of the patients were females in both the stroke (61.50%) and non-stroke groups (52.90%). Thirteen SCD patients (16%) were diagnosed with stroke. Previous history of stroke, high mean corpuscular volume (MCV), and low red blood cells count (RBC) were statistically significant risk factors for stroke (p<0.0001), (p<0.0001), (p<0.03), respectively.  Conclusion Stroke is one of the most devastating complications of SCD. The prevalence of stroke among SCD patients in our study was 16%. Transcranial Doppler ultrasound screening is the most important predictor of stroke., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Babeer et al.)