Seizure Characteristics and EEG Features in Intoxication Type and Energy Deficiency Neurometabolic Disorders in the Pediatric Intensive Care Unit: Single-Center Experience Over 10 Years.

BACKGROUND: Acute metabolic crises in inborn errors of metabolism (such as urea cycle disorders, organic acidemia, maple syrup urine disease, and mitochondrial disorders) are neurological emergencies requiring management in the pediatric intensive care unit (PICU). There is a paucity of data pertaining to electroencephalograms (EEG) characteristics in this cohort. We hypothesized that the incidence of background abnormalities and seizures in this cohort would be high. Neuromonitoring data from our center's PICU over 10 years are presented in this article. METHODS: Data were collected by retrospective chart review for patients with the aforementioned disorders who were admitted to the PICU at our institution because of metabolic/neurologic symptoms from 2008 to 2018. Descriptive statistics (χ2 test or Fisher's exact test) were used to study the association between EEG parameters and outcomes. RESULTS: Our cohort included 40 unique patients (8 with urea cycle disorder, 7 with organic acidemia, 3 with maple syrup urine disease, and 22 with mitochondrial disease) with 153 admissions. Presenting symptoms included altered mentation (36%), seizures (41%), focal weakness (5%), and emesis (28%). Continuous EEG was ordered in 34% (n = 52) of admissions. Twenty-three admissions were complicated by seizures, including eight manifesting as status epilepticus (seven nonconvulsive and one convulsive). Asymmetry and focal slowing on EEG were associated with seizures. Moderate background slowing or worse was noted in 75% of EEGs. Among those patients monitored on EEG, 4 (8%) died, 3 (6%) experienced a worsening of their Pediatric Cerebral Performance Category (PCPC) score as compared to admission, and 44 (86%) had no change (or improvement) in their PCPC score during admission. CONCLUSIONS: This study shows a high incidence of clinical and subclinical seizures during metabolic crisis in patients with inborn errors of metabolism. EEG background features were associated with risk of seizures as well as discharge outcomes. This is the largest study to date to investigate EEG features and risk of seizures in patients with neurometabolic disorders admitted to the PICU. These data may be used to inform neuromonitoring protocols to improve mortality and morbidity in inborn errors of metabolism.

Ictal-Interictal Continuum in the Pediatric Intensive Care Unit.

BACKGROUND: The ictal-interictal continuum (IIC) consists of several electroencephalogram (EEG) patterns that are common in critically ill adults. Studies focused on the IIC are limited in critically ill children and have focused primarily on associations with electrographic seizures (ESs). We report the incidence of the IIC in the pediatric intensive care unit (PICU). We then compare IIC patterns to rhythmic and periodic patterns (RPP) not meeting IIC criteria looking for associations with acute cerebral abnormalities, ES, and in-hospital mortality. METHODS: This was a retrospective review of prospectively collected data for patients admitted to the PICU at Children's National Hospital from July 2021 to January 2023 with continuous EEG. We excluded patients with known epilepsy and cerebral injury prior to presentation. All patients were screened for RPP. The American Clinical Neurophysiology Society standardized Critical Care EEG terminology for the IIC was applied to each RPP. Associations between IIC and RPP not meeting IIC criteria, with clinical and EEG variables, were calculated using odds ratios (ORs). RESULTS: Of 201 patients, 21% (42/201) had RPP and 12% (24/201) met IIC criteria. Among patients with an IIC pattern, the median age was 3.4 years (interquartile range (IQR) 0.6-12 years). Sixty-seven percent (16/24) of patients met a single IIC criterion, whereas the remainder met two criteria. ESs were identified in 83% (20/24) of patients and cerebral injury was identified in 96% (23/24) of patients with IIC patterns. When comparing patients with IIC patterns with those with RPP not qualifying as an IIC pattern, both patterns were associated with acute cerebral abnormalities (IIC OR 26 [95% confidence interval {CI} 3.4-197], p = 0.0016 vs. RPP OR 3.5 [95% CI 1.1-11], p = 0.03), however, only the IIC was associated with ES (OR 121 [95% CI 33-451], p < 0.0001) versus RPP (OR 1.3 [0.4-5], p = 0.7). CONCLUSIONS: Rhythmic and periodic patterns and subsequently the IIC are commonly seen in the PICU and carry a high association with cerebral injury. Additionally, the IIC, seen in more than 10% of critically ill children, is associated with ES. The independent impact of RPP and IIC patterns on secondary brain injury and need for treatment of these patterns independent of ES requires further study.

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.

Neuromonitoring in Children with Cerebrovascular Disorders.

BACKGROUND: Cerebrovascular disorders are an important cause of morbidity and mortality in children. The acute care of a child with an ischemic or hemorrhagic stroke or cerebral sinus venous thrombosis focuses on stabilizing the patient, determining the cause of the insult, and preventing secondary injury. Here, we review the use of both invasive and noninvasive neuromonitoring modalities in the care of pediatric patients with arterial ischemic stroke, nontraumatic intracranial hemorrhage, and cerebral sinus venous thrombosis. METHODS: Narrative review of the literature on neuromonitoring in children with cerebrovascular disorders. RESULTS: Neuroimaging, near-infrared spectroscopy, transcranial Doppler ultrasonography, continuous and quantitative electroencephalography, invasive intracranial pressure monitoring, and multimodal neuromonitoring may augment the acute care of children with cerebrovascular disorders. Neuromonitoring can play an essential role in the early identification of evolving injury in the aftermath of arterial ischemic stroke, intracranial hemorrhage, or sinus venous thrombosis, including recurrent infarction or infarct expansion, new or recurrent hemorrhage, vasospasm and delayed cerebral ischemia, status epilepticus, and intracranial hypertension, among others, and this, is turn, can facilitate real-time adjustments to treatment plans. CONCLUSIONS: Our understanding of pediatric cerebrovascular disorders has increased dramatically over the past several years, in part due to advances in the neuromonitoring modalities that allow us to better understand these conditions. We are now poised, as a field, to take advantage of advances in neuromonitoring capabilities to determine how best to manage and treat acute cerebrovascular disorders in children.

International Prevalence and Mechanisms of SARS-CoV-2 in Childhood Arterial Ischemic Stroke During the COVID-19 Pandemic.

BACKGROUND: Data from the early pandemic revealed that 0.62% of children hospitalized with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) had an acute arterial ischemic stroke (AIS). In a larger cohort from June 2020 to December 2020, we sought to determine whether our initial point estimate was stable as the pandemic continued and to understand radiographic and laboratory data that may clarify mechanisms of pediatric AIS in the setting of SARS-CoV-2. METHODS: We surveyed international sites with pediatric stroke expertise to determine numbers of hospitalized SARS-CoV-2 patients <18 years, numbers of incident AIS cases among children (29 days to <18 years), frequency of SARS-CoV-2 testing for children with AIS, and numbers of childhood AIS cases positive for SARS-CoV-2 June 1 to December 31, 2020. Two stroke neurologists with 1 neuroradiologist determined whether SARS-CoV-2 was the main stroke risk factor, contributory, or incidental. RESULTS: Sixty-one centers from 21 countries provided AIS data. Forty-eight centers (78.7%) provided SARS-CoV-2 hospitalization data. SARS-CoV-2 testing was performed in 335/373 acute AIS cases (89.8%) compared with 99/166 (59.6%) in March to May 2020, P<0.0001. Twenty-three of 335 AIS cases tested (6.9%) were positive for SARS-CoV-2 compared with 6/99 tested (6.1%) in March to May 2020, P=0.78. Of the 22 of 23 AIS cases with SARS-CoV-2 in whom we could collect additional data, SARS-CoV-2 was the main stroke risk factor in 6 (3 with arteritis/vasculitis, 3 with focal cerebral arteriopathy), a contributory factor in 13, and incidental in 3. Elevated inflammatory markers were common, occurring in 17 (77.3%). From centers with SARS-CoV-2 hospitalization data, of 7231 pediatric patients hospitalized with SARS-CoV-2, 23 had AIS (0.32%) compared with 6/971 (0.62%) from March to May 2020, P=0.14. CONCLUSIONS: The risk of AIS among children hospitalized with SARS-CoV-2 appeared stable compared with our earlier estimate. Among children in whom SARS-CoV-2 was considered the main stroke risk factor, inflammatory arteriopathies were the stroke mechanism.

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.

Cerebral Sinus Venous Thrombosis in Infants after Surgery for Congenital Heart Disease.

OBJECTIVE: To determine the prevalence of and risk factors for cerebral sinus venous thrombosis (CSVT) in neonates undergoing congenital heart disease (CHD) repair. STUDY DESIGN: Neonates who had CHD repair with cardiopulmonary bypass and postoperative brain magnetic resonance imaging (MRI) between 2013 and 2019 at a single tertiary care center were identified from institutional databases. Demographic, clinical, and surgical data were abstracted from these databases and from the medical record; 278 neonates with CHD had cardiopulmonary bypass, 184 of whom had a postoperative brain MRI. RESULTS: Eight patients (4.3%) had a CSVT. Transposition of the great arteries with an intact ventricular septum (P < .01) and interrupted aortic arch (P = .02) were associated with an increased risk for CSVT. Other risk factors for CSVT included cross-clamp time (98 [IQR, 77.5-120] minutes vs 67 [IQR, 44-102] minutes; P = .03), units of platelets (3.63 [IQR, 3-4] vs 2.17 [IQR, 1-4]; P < .01) and packed red blood cells (0.81 [IQR, 0.25-1] vs 1.21 [IQR, 1-1]; P = .03) transfused intraoperatively, and time between surgery and MRI (10 [IQR, 7-12.5] days vs 20 [IQR, 12-35] days; P < .01). Five patients (62.5%) were treated with anticoagulation. All patients had complete or partial resolution of their CSVT, regardless of treatment. CONCLUSIONS: Brain MRI after cardiopulmonary bypass in neonates revealed a low prevalence of CSVT (4.3%). Further studies are needed to establish best practices for surveillance, prevention, and treatment of CSVT in this population.

Incidence and predictors of epilepsy in children with congenital heart disease.

OBJECTIVE: Children with CHD may be at increased risk for epilepsy. While the incidence of perioperative seizures after surgical repair of CHD has been well-described, the incidence of epilepsy is less well-defined. We aim to determine the incidence and predictors of epilepsy in patients with CHD. METHODS: Retrospective cohort study of patients with CHD who underwent cardiopulmonary bypass at <2 years of age between January, 2012 and December, 2013 and had at least 2 years of follow-up. Clinical variables were extracted from a cardiac surgery database and hospital records. Seizures were defined as acute if they occurred within 7 days after an inciting event. Epilepsy was defined based on the International League Against Epilepsy criteria. RESULTS: Two-hundred and twenty-one patients were identified, 157 of whom were included in our analysis. Five patients (3.2%) developed epilepsy. Acute seizures occurred in 12 (7.7%) patients, only one of whom developed epilepsy. Predictors of epilepsy included an earlier gestational age, a lower birth weight, a greater number of cardiac surgeries, a need for extracorporeal membrane oxygenation or a left ventricular assist device, arterial ischaemic stroke, and a longer hospital length of stay. CONCLUSIONS: Epilepsy in children with CHD is rare. The mechanism of epileptogenesis in these patients may be the result of a complex interaction of patient-specific factors, some of which may be present even before surgery. Larger long-term follow-up studies are needed to identify risk factors associated with epilepsy in these patients.

Mechanical Thrombectomy for Acute Ischemic Stroke: Considerations in Children.

The use of mechanical thrombectomy for the treatment of acute childhood arterial ischemic stroke with large vessel occlusion is increasing, with mounting evidence for its feasibility and safety. Despite this emerging evidence, clear guidelines for patient selection, thrombectomy technique, and postprocedure care do not exist for the pediatric population. Due to unique features of stroke in children, neurologists and interventionalists must consider differences in patient size, anatomy, collateral vessels, imaging parameters, and expected outcomes that may impact appropriate patient selection and timing criteria. In addition, different causes of stroke and comorbidities in children must be considered and may alter the safety and efficacy of thrombectomy. To optimize the success of endovascular intervention in children, a multidisciplinary team should take into account these nuanced considerations when determining patient eligibility, developing a procedural approach, and formulating a postprocedure neurological monitoring and therapeutic plan.

A Stroke Alert Protocol Decreases the Time to Diagnosis of Brain Attack Symptoms in a Pediatric Emergency Department.

OBJECTIVE: To determine whether a stroke alert system decreases the time to diagnosis of children presenting to the emergency department (ED) with acute-onset focal neurologic deficits. STUDY DESIGN: We performed a retrospective comparison of clinical and demographic information for patients who presented to the ED of a tertiary children's hospital with acute-onset focal neurologic deficits during the 2.5 years before (n = 14) and after (n = 65) the implementation of a stroke alert system. The primary outcome was the median time to neuroimaging analyzed using a Wilcoxon rank-sum test. RESULTS: The median time from ED arrival to neuroimaging for patients with acute-onset focal neurologic deficits decreased significantly after implementation of a stroke alert system (196 minutes; IQR, 85-230 minutes before [n = 14] vs 82 minutes; IQR, 54-123 minutes after [n = 65]; P < .01). Potential intravenous tissue plasminogen activator candidates experienced the shortest time to neuroimaging after implementation of a stroke alert system (54 minutes; IQR, 34-66 minutes [n = 13] for intravenous tissue plasminogen activator candidates vs 89.5 minutes; IQR, 62-126.5 minutes [n = 52] for non-intravenous tissue plasminogen activator candidates; P < .01). CONCLUSIONS: A stroke alert system decreases the median time to diagnosis by neuroimaging of children presenting to the ED with acute-onset focal neurologic deficits by more than one-half. Such a protocol constitutes an important step in ensuring that a greater proportion of children with arterial ischemic stroke are diagnosed in a time frame that enables hyperacute treatment.

Atlanto-occipital ligament calcification: a novel imaging finding in pediatric rotational vertebral artery occlusion.

We describe a 2-year-old girl with bow hunter syndrome complicated by vertebral artery dissection and multiple ischemic infarcts. Pediatric bow hunter syndrome is a rare and likely under-recognized disorder. Interestingly, our patient had atlanto-occipital ligament calcification on CT scan, an imaging finding that has not been reported in association with bow hunter syndrome and one that might help increase recognition of this dynamic disorder of the posterior circulation.

Clinical Determination of Brain Death in Children Supported by Extracorporeal Membrane Oxygenation.

BACKGROUND/OBJECTIVE: Children supported by extracorporeal membrane oxygenation (ECMO) are at risk of catastrophic neurologic injury and brain death. Timely determination of brain death is important for minimizing psychological distress for families, resource allocation, and organ donation. Reports of successful determination of brain death in pediatric patients supported by ECMO are limited. The determination of brain death by clinical criteria requires apnea testing, which has historically been viewed as challenging in patients supported by ECMO. We report eight pediatric patients who underwent a total of 14 brain death examinations, including apnea testing, while supported by veno-arterial ECMO (VA-ECMO), resulting in six cases of clinical determination of brain death. METHODS: We performed a retrospective review of the medical records of pediatric patients who underwent brain death examination while supported by VA-ECMO between 2010 and 2018 at a single tertiary care children's hospital. RESULTS: Eight patients underwent brain death examination, including apnea testing, while supported by VA-ECMO. Six patients met criteria for brain death, while two had withdrawal of technical support after the first examination. During the majority of apnea tests (n = 13/14), the ECMO circuit was modified to achieve hypercarbia while maintaining oxygenation and hemodynamic stability. The sweep flow was decreased prior to apnea testing in ten brain death examinations, carbon dioxide was added to the circuit during three examinations, and ECMO pump flows were increased in response to hypotension during two examinations. CONCLUSIONS: Clinical determination of brain death, including apnea testing, can be performed in pediatric patients supported by ECMO. The ECMO circuit can be effectively modified during apnea testing to achieve a timely rise in carbon dioxide while maintaining oxygenation and hemodynamic stability.

Successful Management of Febrile Infection-Related Epilepsy Syndrome Using Cytokine-Directed Therapy.

Here we describe a pediatric patient with febrile infection-related epilepsy syndrome with a good functional and neurologic outcome after treatment with early and aggressive cytokine-directed immunomodulatory therapy and a seizure management strategy that intentionally avoided a barbiturate coma. A 5-year-old previously healthy male presented with staring, behavioral arrest, and encephalopathy evolving to super-refractory status epilepticus. He had had onset of fever 5 days prior. He was treated with early and aggressive immunomodulatory therapy targeted to his evolving cytokine profile. He was also treated with the ketogenic diet, antiseizure medications, and continuous anesthetic infusions. Pentobarbital was purposely avoided. Now, 2½ years later, he attends mainstream school, has attention-deficit hyperactivity disorder (ADHD), mild neurocognitive impairment, and well-controlled epilepsy. By using cytokine-directed immunotherapy and avoiding a barbiturate coma, we were able to successfully treat a pediatric patient with febrile infection-related epilepsy syndrome and achieve a good outcome.

Seizures, Epilepsy, and Electroencephalography Findings in Pediatric Moyamoya Arteriopathy: A Scoping Review.

BACKGROUND: Although seizures are known to occur in children with moyamoya arteriopathy, data regarding characteristics, prevalence, and predictive factors for their development are less established. This study aimed to systematically review literature addressing seizures, epilepsy, and electroencephalography findings in the pediatric moyamoya population. METHODS: A scoping review was performed by searching PubMed and Ovid:Embase databases for articles that described seizures, epilepsy, and electroencephalography findings in patients aged 0 to 21 years with moyamoya arteriopathy. RESULTS: The search yielded 43 total articles that addressed the following topics in childhood moyamoya: seizures as the presenting symptom, epilepsy characteristics and management, characteristic electroencephalography findings including rebuildup with discussion of proposed mechanisms, and potential predictive clinical factors for the development of seizures preoperatively and the persistence of epilepsy postoperatively. In the reviewed literature, 9% to 19% of children with moyamoya had epilepsy, with over half of the cases lacking radiographic evidence of ischemia. Young age was the most consistent clinical factor associated with both seizures as the presenting symptom and with moyamoya-related epilepsy. Multiple studies report that seizures, electroencephalographic background abnormalities, and the rebuildup phenomenon improve after successful revascularization surgery. CONCLUSIONS: This scoping review provides a thorough investigation of the literature available to date on the clinical features of seizures in the pediatric moyamoya population. Literature on this topic is scarce and further studies assessing predictive factors for the development of epilepsy, prognosis as a result of having seizures, and seizure management in this population will help to fill existing knowledge gaps.

Theta-Alpha Variability on Admission EEG Is Associated With Outcome in Pediatric Cerebral Malaria.

PURPOSE: Pediatric cerebral malaria has high rates of mortality and neurologic morbidity. Although several biomarkers, including EEG, are associated with survival or morbidity, many are resource intensive or require skilled interpretation for clinical use. Automation of quantitative interpretation of EEG may be preferable in resource-limited settings, where trained interpreters are rare. As currently used quantitative EEG factors do not adequately describe the spectrum of variability seen in studies from children with cerebral malaria, the authors developed and validated a new quantitative EEG variable, theta-alpha variability (TAV). METHODS: The authors developed TAV, a new quantitative variable, as a composite of multiple automated EEG outputs. EEG records from 194 children (6 months to 14 years old) with cerebral malaria were analyzed. Independent EEG interpreters performed standard quantitative and qualitative analyses, with the addition of the newly created variable. The associations of TAV with other quantitative EEG factors, a qualitative assessment of variability, and outcomes were assessed. RESULTS: Theta-alpha variability was not highly correlated with alpha, theta, or delta power and was not associated with qualitative measures of variability. Children whose EEGs had higher values of TAV had a lower risk of death (odds ratio = 0.934, 95% confidence interval = 0.902-0.966) or neurologic sequelae (odds ratio = 0.960, 95% confidence interval = 0.932-0.990) compared with those with lower values. Receiver operating characteristic analysis in predicting death at a TAV threshold of 0.244 yielded a sensitivity of 74% and specificity of 70% for an area under the receiver operating characteristic curve of 0.755. CONCLUSIONS: Theta-alpha variability is independently associated with outcome in pediatric cerebral malaria and can predict death with high sensitivity and specificity. Automated determination of this newly created EEG factor holds promise as a potential method to increase the clinical utility of EEG in resource-limited settings by allowing interventions to be targeted to those at higher risk of death or disability.

Bilateral Retinal and Lateral Geniculate Nucleus Infarction in an Adolescent With Influenza.

We describe a 13-year-old female with influenza complicated by bilateral vision loss due to retinal and lateral geniculate nucleus (LGN) infarctions. She continues to have near-total vision loss in her left eye 3.5 years later. This is the second reported case of bilateral retinal and LGN infarctions in the setting of influenza. The mechanism of infarction remains to be determined, but it is important to recognize this entity and counsel patients appropriately as visual recovery may be poor.

Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy: An Emerging Cause of Meningoencephalomyelitis in Children and Adolescents.

Introduction: We describe 5 children with GFAP astrocytopathy with the goal of further characterizing this rare form of meningoencephalomyelitis. Methods: Retrospective chart review of patients diagnosed with GFAP astrocytopathy between 2019 and 2021. Results: Patients were 8-17 years old, and all were male. Fever, headache, and vomiting were common presenting symptoms, and weakness, tremor, and ataxia were common initial examination findings. Initial magnetic resonance imaging (MRI) showed spinal cord abnormalities in 2 patients and leptomeningeal enhancement in 1. Most patients had cerebral spinal fluid pleocytosis, and all screened negative for malignancy. Three patients progressed to coma, and all were treated with immunosuppressant therapy. By discharge, all patients had improved over their clinical nadir, although none had returned to baseline. Discussion: GFAP astrocytopathy is a recently recognized cause of meningoencephalomyelitis in children. Here, we expand our understanding of this entity with the goal of aiding those treating children with GFAP astrocytopathy.