Association of Quality of Life Domains and Clinical Symptoms in Patients With Familial Cerebral Cavernous Malformation.

Background: Familial cerebral cavernous malformation (fCCM) is characterized by multiple brain lesions affecting quality of life. PROMIS-29 (Patient-Reported Outcomes Measurement Information System 29) is a quality of life survey validated in some neurological diseases but not in fCCM. We assessed the reliability, validity, and association of PROMIS-29 in fCCM with clinical symptoms and with the modified Rankin Scale.

Paraclinoid aneurysm clipping: how I do it.

Paraclinoid aneurysms continue to present a surgical challenge, even in the modern endovascular era. Although these aneurysms are uncommon, surgical clipping remains the treatment of choice in select cases. Successful surgical management requires both extensive experience and a comprehensive understanding of paraclinoid anatomy.

Guidelines for the Diagnosis and Clinical Management of Cavernous Malformations of the Brain and Spinal Cord: Consensus Recommendations Based on a Systematic Literature Review by the Alliance to Cure Cavernous Malformation Clinical Advisory Board Experts Panel.

Background And Objectives: Despite many publications about cavernous malformations (CMs), controversy remains regarding diagnostic and management strategies. To update evidence-based guidelines for the clinical management of brain and spinal cord CMs.

Methods: The Alliance to Cure CMs, the patient support group in the United States advocating on behalf of patients and research in CM, convened a multidisciplinary writing group comprising expert CM clinicians to help summarize the existing literature related to the clinical care of CM, focusing on 5 topics: (1) epidemiology and natural history, (2) genetic testing and counseling, (3) diagnostic criteria and imaging standards, (4) neurosurgical considerations, and (5) neurological considerations.

Anterior communicating aneurysm clipping: How I do it.

Up to 40% of intracranial aneurysms arise from the anterior cerebral artery and anterior communicating artery (ACA-ACoA) complex. The vast variability of vessel anomalies and the surrounding critical structures correlate with severe morbidity and mortality rates in case of rupture. In the era of cutting-edge advantages of endovascular procedures, surgical expertise is reducing.

Imaging of Hemorrhagic Stroke in Children.

Hemorrhagic stroke (HS) is an important cause of neurologic morbidity and mortality in children and is more common than ischemic stroke between the ages of 1 and 14 years, a notable contradistinction relative to adult stroke epidemiology. Rapid neuroimaging is of the utmost importance in making the diagnosis of HS, identifying a likely etiology, and directing acute care. Computed tomography and MR imaging with flow-sensitive MR imaging and other noninvasive vascular imaging studies play a primary role in the initial diagnostic evaluation.

Indications for cerebral revascularization for moyamoya syndrome in pediatric sickle cell disease determined by Delphi methodology.

Objective: Cerebral revascularization surgery (CRS) has been used to prevent stroke in children with sickle cell disease (SCD) and cerebral vasculopathy (e.g., moyamoya syndrome).

Can diffuse reflectance spectroscopy identify shuntodynia in pediatric hydrocephalus patients?

Significance: Shuntodynia is patient reported pain at the site of the implanted ventriculoperitoneal (VP) shunt. Pediatric hydrocephalus requiring shunt placement is a chronic and prevalent standard of care treatment and requires lifetime management. Shuntodynia is a subjective measure of shunt dysfunction.

Prevalence and Predictors of Hereditary Hemorrhagic Telangiectasia and Capillary-Malformation Arteriovenous Malformation Syndrome Among Children with Neurovascular Malformations.

Objective: To investigate the prevalence and predictors of hereditary hemorrhagic telangiectasia (HHT) and capillary-malformation arteriovenous malformation (CM-AVM) syndrome among children with no prior personal or family history of these diseases who presented with an arteriovenous shunt lesion.

Study Design: A retrospective chart review was completed on patients aged 0 through 21 years with arteriovenous shunt lesions evaluated at our Cerebrovascular Center. Diagnosis of definite or suspected HHT or CM-AVM was based on clinical features and genetic testing.

Cerebral revascularization surgery reduces cerebrovascular events in children with sickle cell disease and moyamoya syndrome: Results of the stroke in sickle cell revascularization surgery retrospective study.

Background: Recent studies suggest that cerebral revascularization surgery may be a safe and effective therapy to reduce stroke risk in patients with sickle cell disease and moyamoya syndrome (SCD-MMS).

Methods: We performed a multicenter, retrospective study of children with SCD-MMS treated with conservative management alone (conservative group)-chronic blood transfusion and/or hydroxyurea-versus conservative management plus surgical revascularization (surgery group). We monitored cerebrovascular event (CVE) rates-a composite of strokes and transient ischemic attacks.

Cerebral Arterial Growth in Childhood.

Background: Improved understanding of cerebral arterial growth in children may lead to advances in the diagnosis and treatment of pediatric cerebrovascular disease. We correlated cross-sectional diameters of major cerebral arterial structures with age, sex, head circumference, weight, and height in children without cerebrovascular disease.

Methods: Children with normal brain magnetic resonance imaging (MRI) were retrospectively identified and stratified into 23 age cohorts from birth to age 18 years.

Deep Brain Stimulation for Pediatric Dystonia.

Dystonia is one of the most common pediatric movement disorders and can have a profound impact on the lives of children and their caregivers. Response to pharmacologic treatment is often unsatisfactory. Deep brain stimulation (DBS) has emerged as a promising treatment option for children with medically refractory dystonia.

Case Report: Cerebral Revascularization in a Child With Mucopolysaccharidosis Type I.

Mucopolysaccharidosis (MPS) type I is a rare lysosomal storage disorder caused by an accumulation of glycosaminoglycans (GAGs) resulting in multisystem disease. Neurological morbidity includes hydrocephalus, spinal cord compression, and cognitive decline. While many neurological symptoms have been described, stroke is not a widely-recognized manifestation of MPS I.

Fetal and postnatal MRI findings of Blake pouch remnant causing obstructive hydrocephalus.

Blake pouch remnant, also known as Blake pouch cyst or persistent Blake pouch, is a posterior fossa embryologic anomaly that is often seen in isolation with most affected patients being asymptomatic. However, even in isolation, Blake pouch remnant can result in obstructive hydrocephalus requiring early neurosurgical intervention making it an important diagnosis for the fetal radiologist to consider. We present a rare case of a patient with prenatally diagnosed "inferior vermian hypoplasia" on fetal MRI that went on to develop progressive obstructive hydrocephalus in infancy secondary to what was determined to be a Blake pouch remnant.

Cancer and Tumor-Associated Childhood Stroke: Results From the International Pediatric Stroke Study.

Background: The prevalence of cancer among children with stroke is unknown. This study sought to evaluate cancer- and tumor-associated childhood ischemic stroke in a multinational pediatric stroke registry.

Methods: Children aged 29 days to less than 19 years with arterial ischemic stroke or cerebral sinovenous thrombosis enrolled in the International Pediatric Stroke Study between January 2003 and June 2019 were included.

A Noninvasive Retrograde Flushing System for Shunted Hydrocephalus: Initial Case Series of 25 Patients.

Hydrocephalus is a common neurosurgical pathology associated with high patient morbidity and systemwide healthcare costs. A significant portion of these costs are related to the failure of ventricular shunting systems. Despite decades of research and technological development, the rate of shunt failure and revision has not significantly improved.

clearance of a proximal shunt malfunction in a child with hydrocephalus post cerebral arteriovenous malformation rupture noted intraoperatively.

Background: Hydrocephalus shunt malfunctions remain treated with surgical intervention only. Despite efforts at identifying or preventing CSF shunt obstruction, no evidence currently exists to restore CSF flow following proximal occlusion, non-invasively.

Case Description: We present direct intraoperative evidence in the case of a 5-year-old male who developed hydrocephalus subsequent to hemorrhagic presentation post cerebral arteriovenous malformation rupture.

Cervicocerebral quantitative arterial tortuosity: a biomarker of arteriopathy in children with intracranial aneurysms.

Objective: Although intracranial arterial aneurysms (IAAs) of childhood are usually idiopathic, it is possible that underlying arteriopathy escapes detection when using conventional diagnostic tools. Quantitative arterial tortuosity (QAT) has been studied as a biomarker of arteriopathy. The authors analyzed cervicocerebral QAT in children with idiopathic IAAs to assess the possibility of arteriopathy.

Quantitative Arterial Tortuosity Suggests Arteriopathy in Children With Cryptogenic Stroke.

Background And Purpose: Quantitative arterial tortuosity (QAT) is a ratio of vessel length between 2 points to the shortest linear distance between same points. QAT has been reported as an imaging biomarker of arteriopathy in pediatric arterial ischemic stroke (AIS) because of dissection and transient cerebral arteriopathy. We sought to determine whether QAT abnormalities are present in other subtypes of pediatric AIS.