Language and developmental plasticity after perinatal stroke.

The mature human brain is lateralized for language, with the left hemisphere (LH) primarily responsible for sentence processing and the right hemisphere (RH) primarily responsible for processing suprasegmental aspects of language such as vocal emotion. However, it has long been hypothesized that in early life there is plasticity for language, allowing young children to acquire language in other cortical regions when LH areas are damaged. If true, what are the constraints on functional reorganization? Which areas of the brain can acquire language, and what happens to the functions these regions ordinarily perform? We address these questions by examining long-term outcomes in adolescents and young adults who, as infants, had a perinatal arterial ischemic stroke to the LH areas ordinarily subserving sentence processing. We compared them with their healthy age-matched siblings. All participants were tested on a battery of behavioral and functional imaging tasks. While stroke participants were impaired in some nonlinguistic cognitive abilities, their processing of sentences and of vocal emotion was normal and equal to that of their healthy siblings. In almost all, these abilities have both developed in the healthy RH. Our results provide insights into the remarkable ability of the young brain to reorganize language. Reorganization is highly constrained, with sentence processing almost always in the RH frontotemporal regions homotopic to their location in the healthy brain. This activation is somewhat segregated from RH emotion processing, suggesting that the two functions perform best when each has its own neural territory.

Electrographic Seizures and Predictors of Epilepsy after Pediatric Arteriovenous Malformation Rupture.

OBJECTIVE: To assess clinical and electroencephalogram (EEG) predictors of epilepsy and to describe the percentage of electrographic seizures (ES) and development of epilepsy among patients with spontaneous intracerebral hemorrhage (ICH) due to arteriovenous malformation (AVM) rupture. STUDY DESIGN: Retrospective review of patients admitted to the pediatric intensive care unit with ICH secondary to AVM rupture over 11 years. Clinical variables were collected by review of the electronic medical record. Seizures were described as acute symptomatic (7 days after AVM rupture), subacute (7-30 days after AVM rupture) and remote (greater than 30 days after AVM rupture). Outcome metrics included mortality, and the development of epilepsy post discharge. Descriptive statistics were used. RESULTS: Forty-three patients met inclusion criteria with a median age of 12.2 years (IQR 7.3-14.8) and 49% (21/43) were female. Sixteen percent (7/43) presented with a clinical seizure prior to EEG placement. EEG was performed in 62% (27/43) of patients; one had electrographic status epilepticus without clinical signs. Sixteen percent (7/43) of patients were diagnosed with epilepsy, with a median time to diagnosis of 1.34 years (IQR 0.55-2.07) after AVM rupture. One-year epilepsy free survival was 84% (95% CI 70%-98%) and two-year epilepsy free survival was 79% (95% CI 63%-95%) Remote seizures were associated with epilepsy (p<0.001), but acute symptomatic seizures were not (p=0.16). CONCLUSIONS: EEG-confirmed seizures are uncommon in patients with ICH secondary to AVM rupture; however when identified, the seizure burden appears to be high. Patients with seizures 30 days after AVM rupture are more likely to develop epilepsy.

Neurological phenotype of adenosine deaminase 2 deficient patients: a cohort study.

BACKGROUND AND PURPOSE: Patients with adenosine deaminase 2 (ADA2) deficiency can present with various neurological manifestations due to vasculopathies and autoinflammation. These include ischaemic and hemorrhagic stroke, but less clearly defined neurological symptoms have also been reported. METHODS: In this cohort study, patients with confirmed ADA2 deficiency from seven university hospitals in the Netherlands were included. The frequency and recurrence rates of neurological manifestations before and after initiation of tumor necrosis factor α (TNF-α) inhibiting therapy were analyzed. RESULTS: Twenty-nine patients were included with a median age at presentation of 5 years (interquartile range 1-17). Neurological manifestations occurred in 19/29 (66%) patients and were the presenting symptom in 9/29 (31%) patients. Transient ischaemic attack (TIA)/ischaemic stroke occurred in 12/29 (41%) patients and was the presenting symptom in 8/29 (28%) patients. In total, 25 TIAs/ischaemic strokes occurred in 12 patients, one after initiation of TNF-α inhibiting therapy and one whilst switching between TNF-α inhibitors. None was large-vessel occlusion stroke. Two hemorrhagic strokes occurred: one aneurysmatic subarachnoid hemorrhage and one spontaneous intracerebral hemorrhage. Most neurological symptoms, including cranial nerve deficits, vertigo, ataxia and seizures, were caused by TIAs/ischaemic strokes and seldom recurred after initiation of TNF-α inhibiting therapy. CONCLUSIONS: Neurological manifestations, especially TIA/ischaemic stroke, are common in patients with ADA2 deficiency and frequently are the presenting symptom. Because it is a treatable cause of young stroke, for which antiplatelet and anticoagulant therapy are considered contraindicated, awareness amongst neurologists and pediatricians is important. Screening for ADA2 deficiency in young patients with small-vessel ischaemic stroke without an identified cause should be considered.

Seizures in children undergoing stem cell transplantation.

BACKGROUND: Neurological complications (NCs) are of major concern following hematological stem cell transplantation (HSCT), most of which present with seizures. PROCEDURES: We performed a retrospective study (2002-2018) of patients undergoing HSCT in order to analyze the incidence and aetiologies related to seizures. RESULTS: Of 155 children undergoing HSCT, 27 (17.4%) developed seizures at some point in 2 years of follow-up. The most frequent etiologies were central nervous system (CNS) infection (n = 10), drug toxicity (n = 8), and vascular disease (n = 5). A statistically significant association was found between seizure and the HSCT type (lower risk for a related identical donor, p = .010), prophylactic or therapeutic mycophenolate use (p = .043 and .046, respectively), steroid use (p = .023), selective CD45RA+ depletion (p = .002), pre-engraftment syndrome (p = .007), and chronic graft-versus-host disease (GVHD) severity (p = .030). Seizures predicted evolution to life-threatening complications and admission to intensive care (p < .001) and higher mortality (p = .023). A statistically significant association was also found between seizures and sequelae in survivors (p = .029). Children who developed seizures had a higher risk of CNS infection and vascular disease (odds ratio 37.25 [95% CI: 7.45-186.05] and 12.95 [95% CI 2.24-74.80], respectively). CONCLUSIONS: Neurological complications highly impact survival and outcomes and need to be addressed when facing an HSCT procedure.

Neurovascular considerations in patients with Down syndrome and moyamoya syndrome.

In this article, we describe a rare and complex case of moyamoya syndrome in a 7-year-old boy with Down syndrome and atlantoaxial subluxation. The patient presented with an ischemic stroke in the left hemisphere and cervical cord compression with increased cord edema. Diagnostic digital subtraction angiography revealed unique patterns of vascular involvement, with retrograde flow through the anterior spinal artery, ascending cervical artery, occipital artery, and multiple leptomeningeal arteries compensating for bilateral vertebral artery occlusion. This case underscores the underreported phenomenon of upward retrograde flow through the anterior spinal artery in bilateral vertebral artery occlusion. We address the rare manifestation of posterior circulation involvement in moyamoya syndrome, highlighting the importance of considering atlantoaxial instability as a contributing factor, as the absence of atlantoaxial stability is a risk factor for vertebral artery dissection. This study contributes valuable insights into the intricate relationship of moyamoya syndrome, Down syndrome, and atlantoaxial instability, urging clinicians to consider multifaceted approaches in diagnosis and treatment. It also emphasizes the potential significance of the anterior spinal artery as a compensatory pathway in complex vascular scenarios.

Pediatric Moyamoya Revascularization Perioperative Care: A Modified Delphi Study.

BACKGROUND: Surgical revascularization decreases the long-term risk of stroke in children with moyamoya arteriopathy but can be associated with an increased risk of stroke during the perioperative period. Evidence-based approaches to optimize perioperative management are limited and practice varies widely. Using a modified Delphi process, we sought to establish expert consensus on key components of the perioperative care of children with moyamoya undergoing indirect revascularization surgery and identify areas of equipoise to define future research priorities. METHODS: Thirty neurologists, neurosurgeons, and intensivists practicing in North America with expertise in the management of pediatric moyamoya were invited to participate in a three-round, modified Delphi process consisting of a 138-item practice patterns survey, anonymous electronic evaluation of 88 consensus statements on a 5-point Likert scale, and a virtual group meeting during which statements were discussed, revised, and reassessed. Consensus was defined as ≥ 80% agreement or disagreement. RESULTS: Thirty-nine statements regarding perioperative pediatric moyamoya care for indirect revascularization surgery reached consensus. Salient areas of consensus included the following: (1) children at a high risk for stroke and those with sickle cell disease should be preadmitted prior to indirect revascularization; (2) intravenous isotonic fluids should be administered in all patients for at least 4 h before and 24 h after surgery; (3) aspirin should not be discontinued in the immediate preoperative and postoperative periods; (4) arterial lines for blood pressure monitoring should be continued for at least 24 h after surgery and until active interventions to achieve blood pressure goals are not needed; (5) postoperative care should include hourly vital signs for at least 24 h, hourly neurologic assessments for at least 12 h, adequate pain control, maintaining normoxia and normothermia, and avoiding hypotension; and (6) intravenous fluid bolus administration should be considered the first-line intervention for new focal neurologic deficits following indirect revascularization surgery. CONCLUSIONS: In the absence of data supporting specific care practices before and after indirect revascularization surgery in children with moyamoya, this Delphi process defined areas of consensus among neurosurgeons, neurologists, and intensivists with moyamoya expertise. Research priorities identified include determining the role of continuous electroencephalography in postoperative moyamoya care, optimal perioperative blood pressure and hemoglobin targets, and the role of supplemental oxygen for treatment of suspected postoperative ischemia.

Lipoprotein (a): Does It Play a Role in Pediatric Ischemic Stroke and Thrombosis?

PURPOSE OF REVIEW: The goal of this paper is to describe the current understanding of lipoprotein (a) (Lp(a)), clinical practice guidelines, and the potential pathophysiological mechanisms that appear to increase the risk of cardiovascular and thromboembolic events, specifically within the pediatric population. RECENT FINDINGS: The proatherogenic and pro-thrombotic properties of Lp(a) may increase the risk of atherothrombotic disease. In adults, atherosclerotic plaques increase thrombotic risk, but antifibrinolytic and proinflammatory properties appear to have an important role in children. Although it is not well established in neonates, recent studies indicate the risk of incident thrombosis and ischemic stroke are approximately fourfold higher in children with elevated Lp(a) which also increases their risk of recurrent events. Despite this higher risk, Pediatric Lp(a) screening guidelines continue to vary among different medical societies and countries. The inconsistency is likely related to inconclusive evidence outside of observational studies and the lack of specific therapies for children with elevated levels. Additional research is needed to improve understanding of the pro-thrombotic mechanisms of Lp(a), appropriate screening guidelines for Lp(a) in the pediatric population, and to elucidate the short and long term effects of elevated Lp(a) on the risk of pediatric thrombosis and stroke.

Impact of edema formation on functional outcome in pediatric stroke patients.

BACKGROUND: Quantitative lesion net water uptake (NWU) has been described as an imaging biomarker reflecting vasogenic edema as an early indicator of infarct progression. We hypothesized that edema formation measured by NWU is higher in children compared to adults but despite this functional outcome may be better in children. METHODS: This study analyzed children enrolled in the Save ChildS Study who had baseline and follow-up computed tomography available and the data were compared to adult patients. RESULTS: Some 207 patients, of whom 13 were children and 194 were adults, were analyzed. Median NWU at baseline was 7.8% (IQR: 4.3-11.3), and there were no significant differences between children and adults (7.5% vs. 7.8%; p = 0.87). The early edema progression rate was 3.0%/h in children and 2.3%/h in adults. Median ΔNWU was 15.1% in children and 10.5% in adults. Children had significantly more often excellent (mRS 0-1; children 10/13 = 77% vs. adults 28/196 = 14%; p < 0.0001) and favorable clinical outcomes (mRS 0-2, 12/13 = 92% vs. 39/196 = 20%; p < 0.0001). CONCLUSIONS: In this study, clinical outcomes in children with large vessel occlusion strokes were better than in adults despite similar clinical and imaging characteristics and similar edema formation. This may be impacted by the generally better outcomes of children after strokes but may demonstrate that the degree of early ischemic changes using Alberta Stroke Program Early Computed Tomography Score (ASPECTS) and edema progression rate may not be a reason for exclusion from endovascular thrombectomy.

Decompressive hemicraniectomy in pediatric malignant arterial ischemic stroke: a case-based review.

PURPOSE: Malignant stroke is a life-threatening emergency, with a high mortality rate (1-3). Despite strong evidence showing decreased morbidity and mortality in the adult population, decompressive hemicraniectomy (DCH) has been scarcely reported in the pediatric stroke population, and its indication remains controversial, while it could be a potential lifesaving option. METHODS AND RESULTS: We performed an extensive literature review on pediatric malignant arterial ischemic stroke (pmAIS) and selected 26 articles reporting 97 cases. Gathering the data together, a 67% mortality rate is observed without decompressive therapy, contrasting with a 95.4% survival rate with it. The median modified Rankin score (mRS) is 2.1 after surgery with a mean follow-up of 31.8 months. For the 33% of children who survived without surgery, the mRS is 3 at a mean follow-up of 19 months. As an illustrative case, we report on a 2-year-old girl who presented a cardioembolic right middle cerebral artery stroke with subsequent malignant edema and ongoing cerebral transtentorial herniation in the course of a severe myocarditis requiring ECMO support. A DCH was done 32 h after symptom onset. At the age of 5 years, she exhibits an mRS of 3. CONCLUSION: Pediatric stroke with malignant edema is a severe condition with high mortality rate if left untreated and often long-lasting consequences. DCH might minimize the vicious circle of cerebral swelling, increasing intracranial pressure and brain ischemia. Our literature review underscores DCH as an efficient therapeutic measure management of pmAIS even when performed after a significant delay; however, long-lasting morbidities remain high.

Practice variability in the perioperative management of pediatric moyamoya disease in North America.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: This practice variability survey highlights several important knowledge gaps and areas of equipoise that should be targets for future investigation and consensus-building efforts.

Cerebral arteriopathy and ischemic stroke in a pediatric MYH11 patient.

OBJECTIVES: 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. METHODS: A proband girl with an acute ischemic stroke underwent genetic analysis and 7T high-resolution MRI. RESULTS: 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. DISCUSSION: 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.

Pediatric Acute Stroke Protocols in the United States and Canada.

OBJECTIVE: To describe existing pediatric acute stroke protocols to better understand how pediatric centers might implement such pathways within the context of institution-specific structures. STUDY DESIGN: We administered an Internet-based survey of pediatric stroke specialists. The survey included questions about hospital demographics, child neurology and pediatric stroke demographics, acute stroke response, imaging, and hyperacute treatment. RESULTS: Forty-seven surveys were analyzed. Most respondents practiced at a large, freestanding children's hospital with a moderate-sized neurology department and at least 1 neurologist with expertise in pediatric stroke. Although there was variability in how the hospitals deployed stroke protocols, particularly in regard to staffing, the majority of institutions had an acute stroke pathway, and almost all included activation of a stroke alert page. Most institutions preferred magnetic resonance imaging (MRI) over computed tomography (CT) and used abbreviated MRI protocols for acute stroke imaging. Most institutions also had either CT-based or magnetic resonance-based perfusion imaging available. At least 1 patient was treated with intravenous tissue plasminogen activator (IV-tPA) or mechanical thrombectomy at the majority of institutions during the year before our survey. CONCLUSIONS: An acute stroke protocol is utilized in at least 41 pediatric centers in the US and Canada. Most acute stroke response teams are multidisciplinary, prefer abbreviated MRI over CT for diagnosis, and have experience providing IV-tPA and mechanical thrombectomy. Further studies are needed to standardize practices of pediatric acute stroke diagnosis and hyperacute management.

Successful Outcome in an Adolescent with Artery of Percheron Occlusion who was Treated with Tissue Plasminogen Activator.

BACKGROUND: Ischemic stroke is relatively rare in children, leading to a low level of suspicion and delayed diagnosis, particularly in cases of posterior circulation occlusion when symptoms are less indicative. Occlusion of the artery of Percheron (AOP) results in nonspecific neurologic symptoms, including drowsiness, aphasia or dysarthria, ophthalmoplegia, ataxia, and dysmetria. Previous reports, mainly in adults, described late diagnosis and severe residual disability. CASE REPORT: We report a case of a 16-year-old male who presented to the pediatric emergency department with altered mental status. There was no history of trauma or intoxication. The main symptoms included confusion, slurred speech, and multiple falls starting 1 h before arrival to the emergency department. No motor deficits or other focal signs were noticed. The patient's consciousness gradually decreased followed by apneic events. Routine laboratory tests, urinary toxic screen, and a computed tomography scan of the head were normal. A magnetic resonance imaging scan of the brain revealed bilateral restrictive changes in the thalamus. A diagnosis of AOP occlusion was made, and the patient was treated with tissue plasminogen activator (6 h after symptom onset). He was extubated on day 4 and discharged on the day 10 of admission without any neuropsychological deficit. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Posterior circulation stroke in the pediatric population is a diagnostic challenge that often results in suboptimal treatment and unfavorable outcomes. Prompt imaging studies in children with nonspecific altered mental status enable timely diagnosis and thrombolytic treatment that may substantially improve the outcome.

Pediatric Acute Ischemic Stroke by Age-Group: A Systematic Review and Meta-Analysis of Published Studies and Hospitalization Records.

BACKGROUND: Although stroke is rare among the pediatric population, it is nevertheless associated with serious or life-threatening consequences. The etiologic factors of acute ischemic stroke (AIS) are likely to vary over the course of childhood development. The incidence rates of AIS, not previously systematically examined by pediatric age subgroup, could guide studies of its etiology. OBJECTIVE: The aim of this study is to evaluate the incidence rate of AIS by age-group in the pediatric population (aged 0-17/18 years) and identify any common trends or sources of variability across different countries. METHODS: Rates of pediatric AIS were collated from a systematic literature review of published studies globally (1983-2020) and hospitalization records from Europe and the USA (2015-2018). Records that were included in the analysis reported the code or description used for AIS diagnosis and age-specific data for children aged 0-17/18 years. AIS incidence rates were summarized by age-group, data source, country, and geographic region. A meta-analysis was conducted to assess the heterogeneity of AIS rates in neonates. RESULTS: The pooled AIS incidence rate was 5.6 per 100,000 children across all records. When only records reporting the AIS incidence rates for children across the full age range (0-17/18 years) were analyzed, the pooled AIS incidence rate was 4.6 per 100,000 children and ranged from 7.0 per 100,000 (Germany) to 1.3 per 100,000 (Denmark). The highest pooled rates were observed in the 0-28-day age-group (24.6 per 100,000 live births), declining to the lowest rates in the 5-9-year age-group, and rising again in the 10-17/18-year age-group. AIS rates were the most heterogeneous in the 0-28-day age-group and across European countries. Significantly higher AIS rates in neonates were observed from hospital databases (35.9 per 100,000) than in the literature (19.4 per 100,000). AIS rates may be underestimated as pediatric AIS events are rare and challenging to diagnose, and limited age-specific data are available. CONCLUSIONS: Incidence rates of pediatric AIS by age-groups followed a consistent overall pattern of a reverse J-shaped curve, with the highest rates in neonates, across predominantly European and North American countries. Further research is warranted to examine if this pattern is observed in other geographic regions and to identify AIS risk factors specific to different phases of childhood development.

The resilience of the developing reading system: multi-modal evidence of incident and recovery after a pediatric stroke.

Decades of neuroscientific findings have elucidated the highly specialized brain areas involved in reading, especially along the ventral occipitotemporal stream where the critical step of recognizing words occurs. We report on a 14-year-old female with temporary dyslexia after a left ventral occipitotemporal ischemic stroke. Our longitudinal multimodal findings show that the resolution of the reading impairment was associated with heightened activity in the left posterior superior and inferior temporal gyri. Our findings highlight the role of the left inferior temporal gyrus in reading and the importance of perilesional and ipsilateral cortical areas for functional recovery after childhood stroke.

Mechanical thrombectomy in a pediatric patient with sedation aided by contralateral intra-arterial propofol injection: feasibility in an extreme condition.

Pediatric patients undergoing mechanical thrombectomy may be challenging for the anesthesiologists as regards the best anesthetic choice, especially if concomitant to severe comorbidities such as heart failure. A 16-year-old patient affected by arrhythmogenic right ventricle dysplasia/cardiomyopathy underwent mechanical thrombectomy. He was not eligible for deep sedation or general anesthesia since he has been suffering from severe heart failure. The patient stillness was obtained by intra-arterial injection of propofol from the contralateral internal carotid artery. The procedure has been well tolerated, without cardiorespiratory impairment. The case stresses the growing importance to tailor a proper anesthesiologic plan during mechanical thrombectomy, especially in extreme conditions.

Current trends in pediatric moyamoya: a survey of international practitioners.

OBJECTIVE: Moyamoya angiopathy (MM) is a chronic, progressive steno-occlusive arteriopathy of the distal internal carotid artery and its proximal branches. MM is recognized as a shared end-pathway common to a broad range of inciting pathologies, suggesting that tailored management is important. Pediatric MM differs from MM in adults. Currently, there are many uncertainties and controversies regarding the diagnosis and management of children with MM. Hence, we conducted an international survey to identify the contemporary management trends followed worldwide. METHODS: A survey relating to lifestyle modifications, medical management, diagnosis, surgical management, and follow-up for pediatric MM was circulated across web-based platforms, through various international pediatric neurological and neurosurgical societies. Data collected included geographic region of practice, experience, responses to questions, and comments. RESULTS: One hundred twenty-seven responses were evaluated (104 neurosurgeons and 23 neurologists, from 32 countries, across 6 continents). We found wide variations in the recommendations for management and lifestyle modification, with significant differences between regions of practice. Eighty percent recommend restrictions on physical activity, particularly for symptomatic and non-operated patients. Eighty-four percent prescribe aspirin. Sixty-five percent perform indirect revascularization. Seventy-eight percent recommend performing a staged surgery for bilateral MM. Only 26% perform acetazolamide challenge SPECT to evaluate brain perfusion. Only 15% of responders were from highly experienced centers. CONCLUSION: This survey reflects the contemporary trends in management of pediatric MM, while highlighting the heterogeneity in the management approach of these patients. There is a need for multicenter, international studies to evaluate the safety, efficacy, and long-term outcome of various aspects of treatment of these patients.

Ischemic Cerebellar Stroke in a 4-Year-Old Boy After Chickenpox: An Atypical Vascular Involvement.

Cerebrovascular events in pediatric population are very rare. Up to 30% may result from varicella zoster (VZV) arteriopathy, usually as a delayed complication of varicella primary infection. The most typical pattern includes involvement of anterior brain circulation arteries, probably by VZV migration from the trigeminal ganglia. Strokes related with VZV usually have a good prognosis, but risk of recurrence is greater when compared to other stroke etiologies in this age group. We report the case of a 4-year-old boy, immunocompetent, who presented a basilar artery stenosis and a cerebellar stroke, an extremely rare presentation of VZV arteriopathy. The investigation workup and treatment are detailed, as the clinical and imaging follow-up after one year.

Pediatric Case of Life-Threatening Stroke Caused by Reversible Cerebral Vasoconstriction Syndrome with Spontaneous Cervical Internal Carotid Artery Vasospasm: A Case Report.

Pediatric reversible cerebral vasoconstriction syndrome (RCVS) and spontaneous cervical internal carotid artery (ICA) vasospasm are rare conditions; the former is commonly associated with a favorable prognosis. A healthy 13-year-old girl presented with thunderclap headache, followed by left hemiparesis, during a curling match. Six days after onset, left hemiparesis worsened to hemiplegia. Magnetic resonance imaging showed progressive cerebral infarction caused by severe right middle cerebral artery and cervical ICA stenosis. She became comatose because of impending uncal herniation. Emergent surgical decompression was performed. Then, 59 days after onset, her multiple stenoses improved, which was consistent with RCVS concomitant with spontaneous cervical ICA vasospasm. This is the first case of RCVS that concurrently developed spontaneous cervical ICA vasospasm. The patient developed life-threatening stroke due to the hemodynamic impairment of the affected intracranial and cervical arteries. Spontaneous extracranial supra-aortic artery vasospasm can be a poor prognostic predictor of RCVS.

Successful Endovascular Therapy in COVID-19 Associated Pediatric Ischemic Stroke.

Cerebrovascular diseases attributed to coronavirus disease 2019 (COVID-19) are uncommon but can result in devastating outcomes. Pediatric acute ischemic strokes are themselves rare and with very few large vessel occlusion related acute ischemic strokes attributed to COVID-19 described in the literature as of date. COVID-19 pandemic has contributed to acute stroke care delays across the world and with pediatric endovascular therapy still in its infancy, it poses a great challenge in facilitating good outcomes in children presenting with acute ischemic strokes in the setting of COVID-19. We present a pediatric patient who underwent endovascular therapy for an internal carotid artery occlusion related acute ischemic stroke in the setting of active COVID-19 and had an excellent outcome thanks to a streamlined stroke pathway involving the vascular neurology, neuro-interventional, neurocritical care, and anesthesiology teams.