Long-term clinical and radiological trajectories of craniocervical vasculopathy in children with PHACE syndrome.

AIM: To describe the rates of stroke and craniocervical vasculopathy progression in children with posterior fossa malformations, hemangioma, arterial anomalies, coarctation of the aorta/cardiac defects, and eye abnormalities (PHACE) syndrome. METHOD: A single-center, retrospective natural history study of children with PHACE syndrome. Clinical and sequential neuroimaging data were reviewed to study the characteristics and progression of vasculopathy and calculate the rates of arterial ischemic stroke (AIS) and transient ischemic stroke (TIA). Vasculopathy progression was defined as worsening or new vascular findings on follow-up magnetic resonance angiography. RESULTS: Thirty-four children with cerebrovascular abnormalities at the PHACE syndrome diagnosis were studied (age range = 2 to 18 years, 85% females). Median age at the initial diagnosis was 5.5 months (interquartile range = 1-52 months); median age at the last follow-up was 8 years 6 months (range = 2-18 years). Overall, 10 (29%) patients had radiological progression of their vasculopathy, with a cumulative progression-free rate of 73% (95% confidence interval [CI] = 0.57-0.89), and a cumulative TIA-free and AIS-free rate of 87% (95% CI = 0.745-0.99). Vasculopathy was continuously progressive in six patients (18%) at the last follow-up. Three patients (9%) had TIA and all had progressive vasculopathy. One patient had presumed perinatal AIS at the initial PHACE diagnosis, while no other patient experienced an AIS during the follow-up. INTERPRETATION: In children with PHACE syndrome, craniocervical vasculopathy is non-progressive and asymptomatic in the majority of cases. The risk of ischemic stroke in these children is very low. Larger and prospective studies are necessary to confirm these findings.

Cerebral Venous Sinus Thrombosis in Preterm Infants.

BACKGROUND: Neonatal cerebral venous sinus thrombosis (CVST) can lead to brain injury and neurodevelopmental impairments. Previous studies of neonatal CVST have focused on term infants, and studies of preterm infants are lacking. In this study, we examined the clinical and radiological features, treatment and outcome of CVST in preterm infants. METHODS: This was a retrospective, consecutive cohort study of preterm infants (gestational age <37 weeks) with radiologically confirmed CVST. All magnetic resonance imaging/MRV and CT/CTV scans were re-reviewed to study thrombus characteristics and pattern of brain injury. Outcome was assessed by the validated pediatric stroke outcome measure at the most recent clinic visit. RESULTS: Twenty-six preterm infants with CVST were studied. Of these, 65% were moderate-late preterm (32-37 weeks), 27% very preterm (28-32 weeks), and 8% extreme preterm (<28 weeks). Most (73%) were symptomatic at presentation with seizures or abnormal exam. Transverse (85%) and superior sagittal (42%) sinuses were common sites of thrombosis. Parenchymal brain injury was predominantly periventricular (35%) and deep white matter (31%) in location. Intraventricular hemorrhage occurred in 46%. Most infants (69%) were treated with anticoagulation. No treated infant (including eleven with pretreatment hemorrhage) had new or worsening post-treatment hemorrhage. Outcomes ranged from no deficits (50%), mild-moderate (25%), and severe (25%) impairment. CONCLUSIONS: In our sample of preterm infants with CVST, more than one-quarter were asymptomatic. White matter brain lesions predominated and one-half had neurological deficits at follow-up. Anticoagulation of preterm CVST in this small cohort appeared to be safe. Larger studies of preterm CVST are needed.

Fronto-Parietal and White Matter Haemodynamics Predict Cognitive Outcome in Children with Moyamoya Independent of Stroke.

Moyamoya disease is a major arteriopathy characterised by progressive steno-occlusion of the arteries of the circle of Willis. Studies in adults with moyamoya suggest an association between abnormal fronto-parietal and white matter regional haemodynamics and cognitive impairments, even in the absence of focal infarction. However, these associations have not been investigated in children with moyamoya. We examined the relationship between regional haemodynamics and ratings of intellectual ability and executive function, using hypercapnic challenge blood oxygen level-dependent magnetic resonance imaging of cerebrovascular reactivity in a consecutive cohort of children with confirmed moyamoya. Thirty children were included in the final analysis (mean age: 12.55 ± 3.03 years, 17 females, 15 idiopathic moyamoya and 15 syndromic moyamoya). Frontal haemodynamics were abnormal in all regardless of stroke history and comorbidity, but occipital lobe haemodynamics were also abnormal in children with syndromic moyamoya. Executive function deficits were noted in both idiopathic and syndromic moyamoya, whereas intellectual ability was impaired in syndromic moyamoya, even in the absence of stroke. Analysis of the relative effect of regional abnormal haemodynamics on cognitive outcomes demonstrated that executive dysfunction was predominantly explained by right parietal and white matter haemodynamics independent of stroke and comorbidity, while posterior circulation haemodynamics predicted intellectual ability. These results suggest that parietal and posterior haemodynamics play a compensatory role in overcoming frontal vulnerability and cognitive impairment.

Distinct Clinical and Radiographic Phenotypes in Pediatric Patients With Moyamoya.

BACKGROUND: Given the expanding evidence of clinico-radiological differences between moyamoya disease (MMD) and moyamoya syndrome (MMS), we compared the clinical and radiographic features of childhood MMD and MMS to identify predictors of ischemic event recurrence. METHODS: We reviewed a pediatric moyamoya cohort followed between 2003 and 2019. Clinical and radiographic characteristics at diagnosis and follow-up were abstracted. Comparisons between MMD and MMS as well as between MMD and two MMS subgroups (neurofibromatosis [MMS-NF1] and sickle cell disease [MMS-SCD]) were performed. RESULTS: A total of 111 patients were identified. Patients with MMD presented commonly with transient ischemic attacks (TIAs) (35 % MMD versus 13% MMS-NF1 versus 9.5% MMS-SCD; P = 0.047). Symptomatic stroke presentation (MMD 37% versus MMS-NF1 4% versus 33%; P = 0.0147) and bilateral disease at diagnosis (MMD 73% versus MMS-NF1 22 % versus MMS-SCD 67%; P = 0.0002) were uncommon in MMS-NF1. TIA recurrence was common in MMD (hazard ratio 2.86; P = 0.001). The ivy sign was absent on neuroimaging in a majority of patients with MMS-SCD (MMD 67% versus MMS-NF1 52% versus MMS-SCD 9.5%; P = 0.0002). Predictors of poor motor outcome included early age at diagnosis (odds ratio [OR] 8.45; P = 0.0014), symptomatic stroke presentation (OR 6.6; P = 0.019), and advanced Suzuki stage (OR 3.59; P = 0.019). CONCLUSIONS: Moyamoya exhibits different phenotypes based on underlying etiologies. Frequent TIAs is a common phenotype of MMD and symptomatic stroke presentation a common feature of MMD and MMS-SCD, whereas unilateral disease and low infarct burden are common in MMS-NF1. In addition, absence of ivy sign is a common phenotype in MMS-SCD.

Childhood arterial ischemic stroke due to mineralizing angiopathy: an 18-year single-center experience.

Mineralizing angiopathy is a unique, age-specific stroke syndrome characterized by basal ganglia infarction and lenticulostriate calcification after minor head injury in early childhood. There is limited understanding of the pathophysiology, course, and clinical outcome of this syndrome. We describe the clinical and radiographical phenotype of a single-center, consecutively enrolled cohort of children with mineralizing angiopathy from January 2002 to January 2020 and provide a comparative analysis to previously published literature. Fourteen children were identified. Previously unreported findings include: stroke onset in eight children older than 18 months; presence of basal ganglia hemorrhage in four; multifocal basal ganglia infarcts in three; presence of additional non-basal ganglia calcifications in three; and presence of thrombophilia in one. Seven children had moderate-to-severe neurological deficits. There was no symptomatic stroke recurrence (mean follow-up 3y 7mo, SD 1y 7mo). Our expanded phenotype highlights distinct characteristics of mineralizing angiopathy in children and has the potential to inform future research. What this paper adds Children with mineralizing angiopathy are often misdiagnosed as having a limb fracture despite normal x-rays. A magnetic resonance imaging-only approach may miss this entity. Non-contrast computed tomography, in addition to MRI is recommended to identify calcifications in idiopathic arterial ischemic stroke. Most children have moderate-to-severe neurological sequela.

Swallowing, Oral Motor, Motor Speech, and Language Impairments Following Acute Pediatric Ischemic Stroke.

BACKGROUND AND PURPOSE: Following adult stroke, dysphagia, dysarthria, and aphasia are common sequelae. Little is known about these impairments in pediatric stroke. We assessed frequencies, co-occurrence and associations of dysphagia, oral motor, motor speech, language impairment, and caregiver burden in pediatric stroke. METHODS: Consecutive acute patients from term birth-18 years, hospitalized for arterial ischemic stroke (AIS), and cerebral sinovenous thrombosis, from January 2013 to November 2018 were included. Two raters reviewed patient charts to detect documentation of in-hospital dysphagia, oral motor dysfunction, motor speech and language impairment, and caregiver burden, using a priori operational definitions for notation and assessment findings. Other variables abstracted included demographics, preexisting conditions, stroke characteristics, and discharge disposition. Impairment frequencies were obtained by univariate and bivariate analysis and associations by simple logistic regression. RESULTS: A total of 173 patients were stratified into neonates (N=67, mean age 2.9 days, 54 AIS, 15 cerebral sinovenous thrombosis) and children (N=106, mean age 6.5 years, 73 AIS, 35 cerebral sinovenous thrombosis). Derived frequencies of impairments included dysphagia (39% neonates, 41% children); oral motor (6% neonates, 41% children); motor speech (37% children); and language (31% children). Common overlapping impairments included oral motor and motor speech (24%) and dysphagia and motor speech (23%) in children. Associations were found only in children between stroke type (AIS over cerebral sinovenous thrombosis) and AIS severity (more severe deficit at presentation) for all impairments except feeding impairment alone. Caregiver burden was present in 58% patients. CONCLUSIONS: For the first time, we systematically report the frequencies and associations of dysphagia, oral motor, motor speech, and language impairment during acute presentation of pediatric stroke, ranging from 30% to 40% for each impairment. Further research is needed to determine long-term effects of these impairments and to design standardized age-specific assessment protocols for early recognition following stroke.

Investigation of light-induced lacrimation and pupillary responses in episodic migraine.

The purpose of this pilot study was to investigate the light-induced pupillary and lacrimation responses mediated by intrinsically photosensitive retinal ganglion cells (ipRGCs) in migraine. Ten participants with episodic migraine and normal tear production, as well as eleven visually normal controls participated in this study. Following an initial baseline trial (no light flash), participants received seven incremental and alternating red and blue light flashes. Pupillometry recording of the left eye and a 1-min anesthetized Schirmer's test of the right eye (using 0.5% proparacaine) were performed simultaneously. Intrinsic and extrinsic ipRGC photoactivities did not differ between migraine participants and controls across all intensities and wavelengths. Migraine participants, however, had significantly lower lacrimation than controls following the highest blue intensity. A positive correlation was found between melanopsin-driven post-illumination pupillary responses and lacrimation following blue stimulation in both groups. Our results show that participants with self-reported photophobia have normal ipRGC-driven responses, suggesting that photophobia and pupillary function may be mediated by distinct ipRGC circuits. The positive correlation between melanopsin-driven pupillary responses and light-induced lacrimation suggests the afferent arm of the light-induced lacrimation reflex is melanopsin-mediated and functions normally in migraine. Lastly, the reduced melanopsin-mediated lacrimation at the highest stimulus suggests the efferent arm of the lacrimation reflex is attenuated under certain conditions, which may be a harbinger of dry eye in migraine.

Long-term cognitive outcomes after cerebral sinovenous thrombosis in childhood.

AIM: To assess long-term cognitive function in children after cerebral sinovenous thrombosis (CSVT). METHOD: Children with CSVT, who had neuropsychological testing for intellectual ability, executive function, attention, language, or behavior, were included in a prospective observational study. Outcomes were compared with normative means using one-sample t-tests. Predictors of abnormal function were examined using logistic regression. RESULTS: Fifty children with CSVT were included (median age at diagnosis 2y 10mo, interquartile range 7d-6y 10mo; 35 males, 15 females). The median follow-up time was 4 years 2 months (interquartile range 2y 8mo-6y 4mo). Compared with normative means, children with CSVT had lower mean (± standard deviation) full-scale IQ, working memory, and processing speed scores (93.3±16, p=0.01; 93.6±16, p=0.04; 93.7±15.3, p=0.02 respectively). They also had lower scores in executive function, attention, and language domains. Refractory seizure at presentation was associated with a trend in behavioral problems (odds ratio [OR] 6.3, 95% confidence interval [CI] 0.9-46, p=0.07). Females were less likely to experience processing speed difficulties (OR 0.22, 95% CI 0.04-1.3, p=0.09). Incomplete recanalization was associated with a greater risk of abnormal verbal comprehension (OR 5.3, 95% CI 0.93-30.5, p=0.059). INTERPRETATION: Children with CSVT as a group performed below age expectations on standardized neuropsychological tests, although there was variability across individuals and cognitive domains. Larger studies are needed to evaluate predictors of cognitive deficits in children with CSVT.

Bow hunter syndrome: A rare yet important etiology of posterior circulation stroke.

A6-year-oldgirl presented with acute-onset headache andfluctuating right-sided weakness.HerPedNIHSSwas13. Brain MRI/MRA showed acute pontine arterial ischemic stroke(AIS)and remote right cerebellar and thalamic infarcts.No antecedent trauma or other stroke risk factors were identified. Clinical suspicion of bow hunter syndromewas raised. CTshowed congenital C2-C3 fusion and dynamic angiogramconfirmed the diagnosis. The management challenges of this rare condition are discussed below.

Validation of the pediatric stroke outcome measure for classifying overall neurological deficit.

BACKGROUND: The pediatric stroke outcome measure (PSOM) is a standardized, disease-specific outcome measure. We aimed to validate the overall classification of neurological deficit severity using PSOM. METHODS: We identified 367 neonates/children with arterial ischemic stroke (AIS) (Derivation Cohort). We analyzed the PSOM subscales (scored as 0 [no deficit], 0.5 [minimal/mild deficit; normal function], 1 [moderate deficit; slowing function], or 2 [severe deficit; missing function]) to derive severity levels using latent class analysis (LCA). We validated a severity classification scheme (PSOM-SCS) in: (a) children who had Pediatric Evaluation of Disability Inventory (PEDI; n = 63) and/or the Pediatric Quality-of-Life Inventory (PedsQL; n = 97) scored; and (b) an external cohort (AIS; n = 102) with concurrently scored modified Rankin Scale (mRS), King's Outcome Scale for Childhood Head-Injury (KOSCHI) and PSOM. RESULTS: Within the Derivation Cohort, LCA identified three severity levels: "normal/mild," "moderate," and "severe" (83.7%, 13.3%, and 3%, respectively). We developed severity classification based on PSOM subscale scores: "normal/mild"-normal function in all domains or slowing in one domain, "moderate"-slowing in ≥2 domains or missing function in one domain, and "severe"-missing function in ≥2 domains or slowing in ≥1 plus missing in one domain. PEDI and PedsQL both differed significantly across the severity groups. PSOM-SCS displayed high concordance with mRS (agreement coefficient [AC2] = 0.88) and KOSCHI (AC2 = 0.79). CONCLUSION: The PSOM-SCS constitutes a valid tool for classifying overall neurological severity emphasizing function and encompassing the full range of severity in pediatric stroke. IMPACT: Arithmetic summing of the PSOM subscales scores to assess severity classification is inadequate.The prior severity classification using PSOM overestimates poor outcomes.Three distinct severity profiles using PSOM subscales are identified.The PSOM-SCS is in moderate to excellent agreement with other disability measures.PSOM-SCS offers a valid tool for classifying the overall neurological deficit severity.

Obstructive sleep apnea syndrome and neuropsychological function in pediatric stroke.

OBJECTIVES: To assess the prevalence of obstructive sleep apnea syndrome (OSAS) in children with arterial ischemic stroke (AIS) and to evaluate its association with neuropsychological outcomes. METHODS: We conducted a cross-sectional study of sleep health and neuropsychological outcome in children with AIS. A consecutive cohort of children attending a stroke clinic were assessed using a standardized pediatric sleep questionnaire (PSQ) and standardized measures of pediatric stroke outcome and intellectual, executive and adaptive function. High risk for OSAS was defined as PSQ score ≥0.33. RESULTS: Overall, 102 children were included (55% males, median age: 9 years [interquartile-range [IQR]: 6-14]). The prevalence of OSAS in children with AIS was significantly higher compared to published normative prevalence rate (25.5% vs 5%, p < 0.001). Children with OSAS were more likely to have infarcts affecting both the anterior and posterior circulation (37.5% vs 9.5%, p = 0.021). In addition, children with OSAS had significantly higher median Pediatric Stroke Outcome Measure (PSOM) scores (2 [IQR: 0-2] vs 1 [IQR: 1-3.5], p = 0.01) and were more likely to be prescribed concomitant medications affecting sleep architecture (50% vs 22.4%, p = 0.007). OSAS was associated with significantly lower scores on intellectual, memory, cognitive, behavioral, attention, executive and adaptive function scales. The association between PSQ and intellectual ability and working memory remained statistically significant upon controlling for potential confounding factors including stroke related characteristics (neurologic impairment and arterial territory). CONCLUSIONS: The prevalence of OSAS in children with AIS compared to healthy controls is significantly elevated and is associated with poor neuropsychological outcomes. We highlight the importance of regular screening for OSAS - a modifiable risk factor - in children with AIS. The specific risk factors for OSAS and the potential benefits of therapeutic interventions in this patient population warrant further investigation.

Headache Hygiene in Pediatrics: Brushing Up on the Basics.

Headache hygiene refers to self-management behaviors and practices aimed at reducing headache-related disability and improving self-efficacy. Although self-management interventions have an established place in the management of a wide range of chronic conditions, there is still not a standardized approach to this in pediatric headache. In this article, we focus on headache hygiene approaches including education, lifestyle interventions, and psychologic interventions. We also present our center's resource compilation, made available to patients by quick response code technology, as an example of a structured approach to headache hygiene. Further work should explore a standardized approach to headache hygiene and strategies to support adherence, including the use of technology as an innovative health care delivery pathway.

Recurrent stroke: the role of thrombophilia in a large international pediatric stroke population.

Risk factors for arterial ischemic stroke in children include vasculopathy and prothrombotic risk factors but their relative importance to recurrent stroke is uncertain. Data on recurrent stroke from databases held in Canada (Toronto), Germany (Kiel-Lübeck/Münster), and the UK (London/Southampton) were pooled. Data were available from 894 patients aged 1 month to 18 years at first stroke (median age, 6 years) with a median follow-up of 35 months. Among these 894 patients, 160 (17.9%) had a recurrence between 1 day and 136 months after the first stroke (median, 3.1 months). Among 288 children with vasculopathy, recurrence was significantly more common [hazard ratio (HR) 2.5, 95% confidence interval (95% CI) 1.92-3.5] compared to the rate in children without vasculopathy. Adjusting for vasculopathy, isolated antithrombin deficiency (HR 3.9; 95% CI: 1.4-10.9), isolated elevated lipoprotein (a) (HR 2.3; 95% CI: 1.3-4.1), and the presence of more than one prothrombotic risk factor (HR 1.9; 95% CI: 1.12-3.2) were independently associated with an increased risk of recurrence. Recurrence rates calculated per 100 person-years were 10 (95% CI: 3-24) for antithrombin deficiency, 6 (95% CI: 4-9) for elevated lipoprotein (a), and 13 (95% CI: 7-20) for the presence of more than one prothrombotic risk factor. Identifying children at increased risk of a second stroke is important in order to intensify measures aimed at preventing such recurrences.

Anatomical Venous Variants in Children With Cerebral Sinovenous Thrombosis.

Background and Purpose- Literature is sparse on the frequency and significance of anatomical venous variants (AVVs) in pediatric cerebral sinovenous thrombosis (CSVT). Methods- We retrospectively reviewed children with CSVT and controls undergoing computed tomography/magnetic resonance venography from January 2008 to 2014. Clinical features examined included raised intracranial pressure, risk factors, and treatment. Radiological features examined included CSVT location, presence and type of AVVs, hemorrhagic venous infarction, and venous collateralization. Clinical outcome was measured by the pediatric stroke outcome measure and radiological outcome by thrombus recanalization. Results- Fifty-one children with CSVT were identified. Twenty-two (43%) had AVVs at presentation. Nineteen (86%) had hypoplasia/absence of major dural sinus, 5 (23%) had persistent fetal structures, 3 (14%) had duplications/fenestrations, and 1 (5%) had disconnected superficial and deep venous systems. Controls had a slightly higher but nonsignificant prevalence 26 (51%) of AVVs. No significant clinical and radiological differences were observed between children with CSVT and AVVs compared with those with typical venous anatomy. Conclusions- AVVs are seen in many children with and without CSVT and do not seem to alter the presentation or clinical course. The influence of these variations on the brain's ability to tolerate venous congestion because of thrombosis merits further study.

Adenovirus-Associated Central Nervous System Disease in Children.

OBJECTIVE: To characterize the spectrum and salient clinical features of adenovirus-associated neurologic disease in immunocompetent children. STUDY DESIGN: Previously healthy children (aged 1 month-18 years) with central nervous system (CNS) disease associated with adenovirus infection were identified via the Encephalitis Registry (1996-2016) and Microbiology Database (2000-2016) at The Hospital for Sick Children, Toronto, and by systematic review of the literature. The data were pooled and analyzed to identify the spectrum of illness, clinical outcome, and risk factors for death or neurologic impairment. RESULTS: Neurologic complications associated with adenovirus infection in our institution included febrile seizures, encephalitis, acute disseminated encephalomyelitis, and aseptic meningitis. A total of 48 immunocompetent children with adenovirus-associated CNS disease were included in the pooled analysis-38 from the literature and 10 from our institution. In 85% of cases, the virus was detected in the respiratory or gastrointestinal tract, but not the cerebrospinal fluid. Eighteen of the 48 (38%) patients either died or suffered permanent neurologic sequelae. Predictors of adverse outcome included younger age, coagulopathy, the absence of meningismus, serotype 2 virus, and the presence of seizures. After multivariable adjustment, only seizures remained a significant risk factor. CONCLUSION: Adenovirus is a rare cause of CNS disease in immunocompetent children. Disease spectrum is variable, ranging from mild aspetic meningitis and fully reversible encephalopathy to severe, potentially fatal, acute necrotizing encephalopathy.

Predictors of Cognitive and Academic Outcome following Childhood Subcortical Stroke.

Childhood arterial ischemic stroke often involves basal ganglia and thalamus but little is known about neuropsychological outcomes in this group. We examined intellectual ability, academics, attention, executive function, and psychological diagnoses in children and adolescents (6-20 years of age) with childhood stroke involving the basal ganglia (n = 32) or thalamus (n = 12). Intellectual ability was age-appropriate but working memory was significantly lower than expected. Compared to the normative mean, the stroke group exhibited significantly weaker performance in reading comprehension, math fluency, attention, and greater challenges with executive function. Children with basal ganglia stroke had weaker working memory and were more likely to receive diagnoses of Attention Deficit Hyperactivity Disorder and Anxiety Disorder than those with thalamic stroke. Lesion size was most important in predicting working memory ability, whereas age at stroke and age at test were important in predicting academic ability.

Arterial Wall Imaging in Pediatric Stroke.

BACKGROUND AND PURPOSE: Arteriopathy is common in childhood arterial ischemic stroke (AIS) and predicts stroke recurrence. Currently available vascular imaging techniques mainly image the arterial lumen rather than the vessel wall and have a limited ability to differentiate among common arteriopathies. We aimed to investigate the value of a magnetic resonance imaging-based technique, namely noninvasive arterial wall imaging (AWI), for distinguishing among arteriopathy subtypes in a consecutive cohort of children presenting with AIS. METHODS: Children with confirmed AIS and magnetic resonance angiography underwent 3-Tesla AWI including T1-weighted 2-dimensional fluid-attenuated inversion recovery fast spin echo sequences pre- and post-gadolinium contrast. AWI characteristics, including wall enhancement, wall thickening, and luminal stenosis, were documented for all. RESULTS: Twenty-six children with AIS had AWI. Of these, 9 (35%) had AWI enhancement. AWI enhancement was associated with anterior circulation magnetic resonance angiography abnormality and cortical infarction in 8 of 9 (89%) children and normal magnetic resonance angiography with posterior circulation subcortical infarction in 1 (1 of 9; 11%) child. AWI enhancement was not seen in 17 (65%), 10 (59%) of whom had an abnormal magnetic resonance angiography. Distinct patterns of pre- and postcontrast signal abnormality were demonstrated in the vessel wall in the region of interest in children with transient cerebral arteriopathy, arterial dissection, primary central nervous system angiitis, dissecting aneurysm, and cardioembolic stroke. CONCLUSIONS: AWI is a noninvasive, high-resolution magnetic resonance AWI technique, which can be successfully used in children presenting with AIS. Patterns of AWI enhancement are recognizable and associated with specific AIS pathogeneses. Further studies are required to assess the additional diagnostic utility of AWI over routine vascular imaging techniques, in childhood AIS.

[Formula: see text]Neurocognitive outcomes in children with unilateral basal ganglia arterial ischemic stroke and secondary hemidystonia.

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.