Epilepsy-Related Outcomes in Children With Neonatal Cerebellar Injury.

OBJECTIVE: Acute brain injury is a frequent perinatal neurologic complication that can involve the cerebellum. Although short-term outcomes of infants with neonatal cerebellar injury are well described, neurologic sequelae in older children are underreported. Here, we describe epilepsy-related outcomes in young children who suffered from neonatal cerebellar injuries. METHODS: In-house automated software identified patients with neonatal brain injuries who were evaluated at our institution both as neonates (≤28 days) and as children (≥1 year). Neonatal hospital course, neuroimaging, and outcomes related to epilepsy were reviewed from the medical record. Patients were stratified into 2 groups based on neonatal brain injuries: those with cerebellar injury and those without cerebellar involvement. RESULTS: Of the 282 neonates followed through childhood over the decade-long study period, 33 (12%) experienced neonatal brain injury. All 33 cases involved supratentorial injury, and 5 (15%) also included cerebellar injury. The development of epilepsy was significantly less likely in the group with cerebellar involvement (40%) compared to that with cerebellar sparing (82%; P = 0.043). In some cases, children with cerebellum-sparing injuries required admission for seizure control and developed drug-resistant epilepsy as well as status epilepticus. These outcomes occurred less frequently in the group with cerebellar involvement. CONCLUSIONS: Epilepsy-related sequelae may occur less frequently when the cerebellum is involved in neonatal brain injury. Larger prospective studies are needed to clarify how cerebellocortical networks impact functional brain connectivity and epilepsy longitudinally.

Imaging Review of Common and Rare Causes of Stroke in Children.

Vascular injury is increasingly recognized as an important cause of mortality and morbidity in children (29 days to 18 years of age). Since vascular brain injury in children appears to be less common than in adults, the index of suspicion for vascular brain injury is usually lower. In this review article, we describe frequent and rare conditions underlying pediatric stroke including cardioembolic, viral, autoimmune, post-traumatic, and genetic etiologies. Furthermore, we provide a neuroimaging correlate for clinical mimics of pediatric stroke. This review highlights the role of multimodal noninvasive neuroimaging in the early diagnosis of pediatric stroke, providing a problem-solving approach to the differential diagnosis for the neuroradiologist, emergency room physician, and neurologist.

Nonaneurysmal Subarachnoid Hemorrhage in Sickle Cell Disease: Description of a Case and a Review of the Literature.

Descriptions of the natural history of cerebrovascular complications of sickle cell disease (SCD) characterize ischemic stroke as common during childhood and hemorrhagic stroke as more common in adulthood. Childhood ischemic stroke is attributed to vasculopathy with moyamoya syndrome. Hemorrhagic stroke is commonly attributed to aneurysms accompanying HbSS cerebral vasculopathy in SCD. However, a growing body of literature highlights multiple contributing factors to hemorrhagic stroke in children. Primary hemorrhagic stroke is one of the most devastating neurological complications of SCD. We describe the case of an 18-year-old female affected by HbSS genotype SCD presenting with reversible cerebral vasoconstriction syndrome (RCVS) as well as features of posterior reversible encephalopathy syndrome and convexity subarachnoid hemorrhage (SAH) after transfusion of red blood cells. We reviewed the existing literature dealing with SCD, blood transfusion, and hemorrhagic strokes. To our knowledge, this case presentation is unique with convexity SAH predominantly attributable to a RCVS spectrum disorder occurring in the setting of a recent blood transfusion in an adolescent female with SCD. As this case illustrates, neurological deterioration accompanied by intracranial hemorrhage in children and young adults with SCD after blood transfusion should raise suspicion for RCVS as part of a complex cerebral vasculopathy. A better understanding of the risk factors leading to hemorrhagic stroke may help prevent this severe complication in subjects with SCD. Neuroimaging including angiography in these subjects may enable prompt diagnosis and management.

A Case of Lenticulostriate Stroke Due to Minor Closed Head Injury in a 2-Year-Old Child: Role of Mineralizing Angiopathy.

OBJECTIVE: Cerebral infarction due to minor head injury is rare. Mineralizing angiopathy is considered a predisposing factor for lenticulostriate stroke after minor closed head injury. This entity is characterized by infarction of the basal ganglia and most often occurs in young children, from infancy to 2 years of age. Symptoms usually occur immediately after the injury. METHODS AND RESULTS: We present the case of a previously healthy 2-year-old female child presenting with right facial hemiparesis and aphasia, along with right arm and leg weakness that occurred immediately after a fall from the couch onto a carpeted floor. A brain computed tomography scan revealed small and linear calcifications of the basal ganglia and subcortical frontoparietal area, in the absence of signs of intracranial hemorrhage. Brain magnetic resonance imaging with stroke protocol revealed restriction of water diffusion in the left basal ganglia on diffusion-weighted imaging sequences as well as apparent diffusion coefficient sequences, suggesting an acute infarct of the left basal ganglia within the distribution of the lenticulostriate vessels. The patient was treated with aspirin and conservative management and had gradual improvement over the next few days, with full recovery of the aphasia, facial nerve function, and arm and leg mobility, within 2 months. CONCLUSIONS: Some infants can present with the onset of stroke-like symptoms after minor head injuries. Presence of linear calcifications of the basal ganglia noticed on brain computed tomography in many of these patients suggests that mineralizing angiopathy may be a predisposing factor for lenticulostriate stroke after minor closed head injury in infants. Brain magnetic resonance imaging to further delineate possible cerebral infarction is indicated.

Successful Manual Aspiration Thrombectomy in a Pediatric Patient.

BACKGROUND: Timely recognition of stroke at major pediatric medical centers is improving, and although treatment guidelines for pediatric stroke exist, no extensive study establishing the efficacy of surgical or thrombolytic treatment has been completed. Extrapolation of adult guidelines to pediatric patients remains the mainstay of treatment in the absence of adequate information regarding safety and efficacy in children. Recent trials have demonstrated revascularization and clinical improvement after endovascular retrieval therapy in adults with acute large vessel occlusive stroke. Furthermore, successful mechanical thrombectomy using a variety of techniques has been documented in numerous children and adolescents. PATIENT DESCRIPTION: We present a 15-year-old boy with altered mental status and left hemiparesis due to acute ischemic stroke secondary to blockage of the right internal carotid artery terminus, most likely precipitated by end-stage heart failure and cardiac embolism. Mechanical aspiration thrombectomy using the Penumbra aspiration catheter without any adjunct surgical equipment or thrombolytic therapy was used to remove thrombus and treat the patient's acute ischemic stroke. He experienced complete artery recanalization with a Thrombolysis in Cerebral Infarction (TICI) score of 2C after the procedure. He also exhibited an 8 point improvement in his pediatric National Institutes of Health Stroke Scale score within 24 hours. CONCLUSIONS: Mechanical aspiration thrombectomy is commonly used in adult hospitals but infrequently employed in pediatric patients with arterial ischemic stroke. Given its efficacy in our patient, we encourage a larger systematic trial to evaluate the use of mechanical thrombectomy in pediatric patients with acute ischemic stroke.

Emergence of the primary pediatric stroke center: impact of the thrombolysis in pediatric stroke trial.

BACKGROUND AND PURPOSE: In adult stroke, the advent of thrombolytic therapy led to the development of primary stroke centers capable to diagnose and treat patients with acute stroke rapidly. We describe the development of primary pediatric stroke centers through preparation of participating centers in the Thrombolysis in Pediatric Stroke (TIPS) trial. METHODS: We collected data from the 17 enrolling TIPS centers regarding the process of becoming an acute pediatric stroke center with capability to diagnose, evaluate, and treat pediatric stroke rapidly, including use of thrombolytic therapy. RESULTS: Before 2004, <25% of TIPS sites had continuous 24-hour availability of acute stroke teams, MRI capability, or stroke order sets, despite significant pediatric stroke expertise. After TIPS preparation, >80% of sites now have these systems in place, and all sites reported increased readiness to treat a child with acute stroke. Use of a 1- to 10-Likert scale on which 10 represented complete readiness, median center readiness increased from 6.2 before site preparation to 8.7 at the time of site activation (P≤0.001). CONCLUSIONS: Before preparing for TIPS, centers interested in pediatric stroke had not developed systematic strategies to diagnose and treat acute pediatric stroke. TIPS trial preparation has resulted in establishment of pediatric acute stroke centers with clinical and system preparedness for evaluation and care of children with acute stroke, including use of a standardized protocol for evaluation and treatment of acute arterial stroke in children that includes use of intravenous tissue-type plasminogen activator. CLINICAL TRIAL REGISTRATION URL: http://www.clinicaltrials.gov. Unique identifier: NCT01591096.

Neuropsychiatric effects of guanfacine in children with mild tourette syndrome: a pilot study.

The objective of this study was to evaluate the neuropsychiatric effects of the alpha-2a adrenergic agonist guanfacine in children with Tourette syndrome (TS). Twenty-four children with TS participated in a 4-week, double-blind, placebo-controlled study of guanfacine. Tic severity, neuropsychologic functioning, and parent ratings of behavior were evaluated pre- and post-treatment. The sample had mild tic severity and subtle neuropsychologic dysfunction pretreatment. Post-treatment, patients receiving guanfacine were rated by parents as significantly improved (compared to placebo) on one measure of executive function (parent-rated metacognition). Improvement on tic severity, performance-based neuropsychologic measures, and all other parent ratings were not significantly better than placebo. At a moderate dose and short-term treatment duration, guanfacine did not provide significant neuropsychiatric benefits in this group of children with mild TS.