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Background. The spectrum of neurologic disease attributable to Mycoplasma pneumoniae in children is incompletely understood in part because of limitations of microbiologic diagnostic methods. Our objective was to characterize the neurologic complications of M. pneumoniae in children using stringent diagnostic criteria. Methods. All children admitted to the Hospital for Sick Children over a 16-year period with acute neurologic manifestations and polymerase chain reaction (PCR)-confirmed M. pneumoniae infection were eligible for inclusion. Cases were categorized as definite, probable, or possible according to strength of evidence implicating M. pneumoniae. Children with underlying noninfectious neurologic conditions or an alternative infectious cause were excluded. Results. A total of 365 children had M. pneumoniae detected in the cerebrospinal fluid (CSF) or respiratory tract by PCR, 42 (11.5%) of whom had neurologic disease attributable to M. pneumoniae. The most common clinical syndromes were encephalitis (52%), acute disseminated encephalomyelitis (12%), transverse myelitis (12%), and cerebellar ataxia (10%). Two distinct disease patterns were observed, one with a prolonged prodrome (=7 days), respiratory manifestations, an immunoglobulin M (IgM) response in peripheral blood, and detection of M. pneumoniae in the respiratory tract, but not the CSF, and one with a brief (<7 days) or no prodrome, less frequent respiratory manifestations and IgM response, and detection of M. pneumoniae in the CSF, but not the respiratory tract. Conclusions. Our findings support the hypothesis of two separate pathogenetic mechanisms for M. pneumoniae- associated neurologic disease, one related to direct infection of the central nervous system and one indirect, likely immunologically mediated. [ABSTRACT FROM AUTHOR]

Abstract: We present the clinical and cytogenetic findings in an 8 year old girl with mental retardation, acquired microcephaly, delayed motor skills and stereotypical hand movements. Array comparative genomic hybridization identified a mosaic de novo deletion of approximately 7.505 Mb in chromosome region 18q21.1q21.31, resulting in the loss of one copy of the TCF4 gene as well as 29 other RefSeq genes. The deletion likely occurred early in development as this child has clinical symptoms affecting multiple organ systems, reminiscent of those observed in Pitt–Hopkins syndrome (PHS; OMIM 610954). This case represents the second known example of a mosaic deletion resulting in clinical symptoms consistent with Pitt-Hopkins syndrome, and illustrates the utility of genomic microarray analysis in detecting large mosaic imbalances that may otherwise be missed by G-band analysis. [ABSTRACT FROM AUTHOR]

Objective. There are not enough pediatric neurologists to meet the many needs of pediatric neurology patients. The Hospital for Sick Children has responded by expanding the nursing role in the pediatric neurology outpatient clinic. The objective of this study was to examine the use of a telephone nursing line in this hospital-based pediatric neurology clinic. Methods. A cross-sectional study was performed on all telephone call records collected during a 2-week study period. Each initial incoming call concerning a patient was counted as an index call. Associations between clinic type or diagnosis and length of telephone calls were assessed using the X² test. Results. A total of 208 index calls were received, generating a total of 597 incoming and outgoing calls. The most common clinic types were Epilepsy clinic (35.6%) and General Neurology clinic (32.7%), and the most common patient diagnoses were epilepsy (63.5%) and developmental delay (45.2%). Most patients were between the ages of 1 and <7 years (33.9%) and 12 and <18 years (32.8%) and male (55.2%). Most calls were made by mothers (57.2%) to ask about medical administrative issues (28.4%) and/or symptoms (27.9%). Physicians were notified for 47.1% of calls; nurses were twice as likely to notify physicians for calls concerning new symptoms (relative risk: 2.1; 95% confidence interval: 1.6-2.7). Most calls required between 1 and 5 minutes (49.0%). Long telephone calls (>10 minutes) were strongly associated with a diagnosis of epilepsy. Conclusions. There is a high demand for the neurology nursing line in our clinic. Most telephone calls and most long telephone calls concerned patients with epilepsy.... [ABSTRACT FROM AUTHOR]

Evaluates the effect of corticosteroids in the treatment of pediatric Bell's palsy. Natural history of Bell's palsy in children; Lack of positive evidence for the beneficial effects of corticosteroids in pediatric Bell's palsy; Non-recommendation on the routine use of corticosteroids.

Aim Method Results Interpretation 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.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.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.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. [ABSTRACT FROM AUTHOR]

The author reflects on the study "Shaken Baby Syndrome: The Quest for Evidence," which discussed non-accidental head injury. She offers a summary of the issues and accepted factual information about head injury in children and adolescents. She states that whether or not the term "shaken baby syndrome" is accurate, there has been an opportunity to research societal and parenting risk factors with construction of educational programs on the theme of shaken baby with significant effect on prevention. She cites some areas where research should focus in.

Background: Moyamoya is a progressive, non-atherosclerotic cerebral arteriopathy that may present in childhood and currently has no cure. Early diagnosis is critical to prevent a lifelong risk of neurological morbidity. Blood-oxygen-level-dependent (BOLD) MRI cerebrovascular reactivity (CVR) imaging provides a non-invasive, in vivo measure of autoregulatory capacity and cerebrovascular reserve. However, non-compliant or younger children require general anesthesia to achieve BOLD-CVR imaging.To determine the same-day repeatability of BOLD-CVR imaging under general anesthesia in children with moyamoya.Twenty-eight examination pairs were included (mean patient age = 7.3 ± 4.0 years). Positive and negatively reacting voxels were averaged over signals and counted over brain tissue and vascular territory. The intraclass correlation coefficient (ICC), Wilcoxon signed-rank test, and Bland–Altman plots were used to assess the variability between the scans.There was excellent-to-good (≥ 0.59) within-day repeatability in 18 out of 28 paired studies (64.3%). Wilcoxon signed-rank tests demonstrated no significant difference in the grey and white matter CVR estimates, between repeat scans (all p-values > 0.05). Bland–Altman plots of differences in mean magnitude of positive and negative and fractional positive and negative CVR estimates illustrated a reasonable degree of agreement between repeat scans and no systematic bias.BOLD-CVR imaging provides repeatable assessment of cerebrovascular reserve in children with moyamoya imaged under general anesthesia.Objective: Moyamoya is a progressive, non-atherosclerotic cerebral arteriopathy that may present in childhood and currently has no cure. Early diagnosis is critical to prevent a lifelong risk of neurological morbidity. Blood-oxygen-level-dependent (BOLD) MRI cerebrovascular reactivity (CVR) imaging provides a non-invasive, in vivo measure of autoregulatory capacity and cerebrovascular reserve. However, non-compliant or younger children require general anesthesia to achieve BOLD-CVR imaging.To determine the same-day repeatability of BOLD-CVR imaging under general anesthesia in children with moyamoya.Twenty-eight examination pairs were included (mean patient age = 7.3 ± 4.0 years). Positive and negatively reacting voxels were averaged over signals and counted over brain tissue and vascular territory. The intraclass correlation coefficient (ICC), Wilcoxon signed-rank test, and Bland–Altman plots were used to assess the variability between the scans.There was excellent-to-good (≥ 0.59) within-day repeatability in 18 out of 28 paired studies (64.3%). Wilcoxon signed-rank tests demonstrated no significant difference in the grey and white matter CVR estimates, between repeat scans (all p-values > 0.05). Bland–Altman plots of differences in mean magnitude of positive and negative and fractional positive and negative CVR estimates illustrated a reasonable degree of agreement between repeat scans and no systematic bias.BOLD-CVR imaging provides repeatable assessment of cerebrovascular reserve in children with moyamoya imaged under general anesthesia.Materials and methods: Moyamoya is a progressive, non-atherosclerotic cerebral arteriopathy that may present in childhood and currently has no cure. Early diagnosis is critical to prevent a lifelong risk of neurological morbidity. Blood-oxygen-level-dependent (BOLD) MRI cerebrovascular reactivity (CVR) imaging provides a non-invasive, in vivo measure of autoregulatory capacity and cerebrovascular reserve. However, non-compliant or younger children require general anesthesia to achieve BOLD-CVR imaging.To determine the same-day repeatability of BOLD-CVR imaging under general anesthesia in children with moyamoya.Twenty-eight examination pairs were included (mean patient age = 7.3 ± 4.0 years). Positive and negatively reacting voxels were averaged over signals and counted over brain tissue and vascular territory. The intraclass correlation coefficient (ICC), Wilcoxon signed-rank test, and Bland–Altman plots were used to assess the variability between the scans.There was excellent-to-good (≥ 0.59) within-day repeatability in 18 out of 28 paired studies (64.3%). Wilcoxon signed-rank tests demonstrated no significant difference in the grey and white matter CVR estimates, between repeat scans (all p-values > 0.05). Bland–Altman plots of differences in mean magnitude of positive and negative and fractional positive and negative CVR estimates illustrated a reasonable degree of agreement between repeat scans and no systematic bias.BOLD-CVR imaging provides repeatable assessment of cerebrovascular reserve in children with moyamoya imaged under general anesthesia.Results: Moyamoya is a progressive, non-atherosclerotic cerebral arteriopathy that may present in childhood and currently has no cure. Early diagnosis is critical to prevent a lifelong risk of neurological morbidity. Blood-oxygen-level-dependent (BOLD) MRI cerebrovascular reactivity (CVR) imaging provides a non-invasive, in vivo measure of autoregulatory capacity and cerebrovascular reserve. However, non-compliant or younger children require general anesthesia to achieve BOLD-CVR imaging.To determine the same-day repeatability of BOLD-CVR imaging under general anesthesia in children with moyamoya.Twenty-eight examination pairs were included (mean patient age = 7.3 ± 4.0 years). Positive and negatively reacting voxels were averaged over signals and counted over brain tissue and vascular territory. The intraclass correlation coefficient (ICC), Wilcoxon signed-rank test, and Bland–Altman plots were used to assess the variability between the scans.There was excellent-to-good (≥ 0.59) within-day repeatability in 18 out of 28 paired studies (64.3%). Wilcoxon signed-rank tests demonstrated no significant difference in the grey and white matter CVR estimates, between repeat scans (all p-values > 0.05). Bland–Altman plots of differences in mean magnitude of positive and negative and fractional positive and negative CVR estimates illustrated a reasonable degree of agreement between repeat scans and no systematic bias.BOLD-CVR imaging provides repeatable assessment of cerebrovascular reserve in children with moyamoya imaged under general anesthesia.Conclusion: Moyamoya is a progressive, non-atherosclerotic cerebral arteriopathy that may present in childhood and currently has no cure. Early diagnosis is critical to prevent a lifelong risk of neurological morbidity. Blood-oxygen-level-dependent (BOLD) MRI cerebrovascular reactivity (CVR) imaging provides a non-invasive, in vivo measure of autoregulatory capacity and cerebrovascular reserve. However, non-compliant or younger children require general anesthesia to achieve BOLD-CVR imaging.To determine the same-day repeatability of BOLD-CVR imaging under general anesthesia in children with moyamoya.Twenty-eight examination pairs were included (mean patient age = 7.3 ± 4.0 years). Positive and negatively reacting voxels were averaged over signals and counted over brain tissue and vascular territory. The intraclass correlation coefficient (ICC), Wilcoxon signed-rank test, and Bland–Altman plots were used to assess the variability between the scans.There was excellent-to-good (≥ 0.59) within-day repeatability in 18 out of 28 paired studies (64.3%). Wilcoxon signed-rank tests demonstrated no significant difference in the grey and white matter CVR estimates, between repeat scans (all p-values > 0.05). Bland–Altman plots of differences in mean magnitude of positive and negative and fractional positive and negative CVR estimates illustrated a reasonable degree of agreement between repeat scans and no systematic bias.BOLD-CVR imaging provides repeatable assessment of cerebrovascular reserve in children with moyamoya imaged under general anesthesia. [ABSTRACT FROM AUTHOR]

Background Alternating hemiplegia of childhood and rapid-onset dystonia parkinsonism are two separate movement disorders with different dominant mutations in the same sodium-potassium transporter ATPase subunit gene, ATP1A3. Patient We present a child with topiramate-responsive alternating hemiplegia of childhood who was tested for an ATP1A3 gene mutation. Results Gene sequencing revealed an identical ATP1A3 mutation as in three typical adult-onset rapid-onset dystonia parkinsonism cases but never previously described in an alternating hemiplegia of childhood case. Conclusion The discordance of these phenotypes suggests that there are other undiscovered environmental, genetic, or epigenetic factors influencing the development of alternating hemiplegia of childhood or rapid-onset dystonia parkinsonism. [ABSTRACT FROM AUTHOR]

Background Thoracic outlet syndrome is rare in children but may have serious consequences. Compression of the neurovascular structures at the thoracic outlet by anomalous soft tissues or cervical ribs may cause neurological deficits in the upper limb and venous or arterial insufficiency. Patients The symptoms and signs of this condition are well documented, but we describe two patients with an atypical presentation. We review similar published cases where delay in diagnosis resulted in cerebrovascular catastrophe. Our patients presented with relatively nonspecific central nervous system symptoms and were found to have thoracic outlet compression. Both were treated by surgical decompression of the thoracic outlet, and the symptoms completely resolved with no long-lasting neurological consequences. Conclusions We highlight the importance of these rare cases because of the risk of stroke and discuss the theory behind the pathological process. [ABSTRACT FROM AUTHOR]

Predictors of quality of life can define potentially modifiable factors to increase favorable outcomes after pediatric stroke. Quality of life was measured using the Centre for Health Promotion’s Quality of Life Profile (CHP-QOL) in 112 children surviving arterial ischemic stroke or cerebral sinovenous thrombosis at mean 3 years after stroke. Overall quality of life was poor in 17.8% children despite mean scores (3.52) in the “adequate” range. Quality of life related to school and play was most problematic and that related to physical and home environment was least problematic. Female gender, cerebral sinovenous thrombosis stroke, and older age at testing predicted reduced overall and domain-specific quality of life (P < .05), whereas neurological outcome and family socioeconomic status did not. Cognitive/behavioral deficit and low Verbal IQ adversely affected socialization and quality of life, especially among older children and females. Altered cognition/behavior has a major impact on quality of life after pediatric stroke. Implementation of ameliorative strategies warrants further study. [ABSTRACT FROM AUTHOR]

Aim Plasticity in the developing brain is a controversial issue. Although language and motor function often recover remarkably well following early brain injury, recent evidence suggests that damage to the developing brain results in significant long-term neuropsychological impairment. Our aim was to investigate the relationship among age at injury, lesion location and intellectual outcome. Method Using age-appropriate Wechsler scales of intellectual ability, we explored this issue by evaluating a large group ( n=145) of children (89 males, 56 females) who experienced unilateral arterial ischaemic stroke during the perinatal period (diagnosed mean 73d, SD 29d), between the ages of 1 month and 5 years (mean 2y 10mo, SD 1y 9mo), or between the ages of 6 and 16 years (mean 11y 1mo SD 3y 6mo). The mean age at assessment was 8 years (SD 3y 10mo) in the perinatal group, 7 years 5 months (SD 2y 9mo) in the 1 month to 5 years group, and 12 years 5 months (SD 3y 9mo) in the 6 to 16 years group. The mean time interval between stroke and assessment was 8 years (SD 18d) for perinatal, 4 years 6 months (SD 1y 5mo) for 1 month to 5 years, and 1 year 4 months (SD 2y 9mo) for 6 to 16 years. The relationship between age at stroke and lesion location (subcortical, cortical, or combined) as it pertains to cognitive outcome was also examined. Results Measures of overall intelligence, verbal ability, working memory, and processing speed were significantly lower in children who had had a stroke than in the normative sample (all z>2.5, all p<0.01). The perinatal group performed more poorly than the other two groups on most cognitive measures, regardless of lesion location. The combined lesion location group performed more poorly than those with damage to either cortical or subcortical areas alone. Further investigation revealed different periods of peak vulnerability for subcortical lesions (perinatal) and cortical lesions (1mo–5y). Interpretation Lesion location modulates the relationship between age at stroke and cognitive outcome. [ABSTRACT FROM AUTHOR]

Craniocervical arterial dissection is an important cause of childhood arterial ischemic stroke, accounting for 7.5% to 20% of cases. Significant neurologic morbidity and mortality may result and recurrence risk may be higher than in adults. However, the natural history and long-term outcome of pediatric dissection are poorly studied. We report 3 cases of extracranial vertebral artery dissection with complications including pseudoaneurysm formation, recurrent stroke, and late spontaneous thrombosis of the dissected artery. These cases illustrate the dynamic processes involved in vascular injury and healing of vertebral artery dissection in children over years, with potential implications for long-term management and prevention of recurrence. [ABSTRACT FROM AUTHOR]

We identified neurological admissions temporally associated with influenza vaccine to determine the likelihood of causality using World Health Organization (WHO) criteria. Although all cases were categorized as possibly related to the vaccine, most had a compelling alternative explanation. This observation suggests that the current WHO criteria may not be sufficient in determining if an adverse event is truly vaccine related. [ABSTRACT FROM AUTHOR]

Am J Neuroradiol 2009; 30: 232 - 52. 6 Toelle SP, Avetisyan T, Kuyumjyan N, Sukhudyan B, Boltshauser E, Hackenberg A. Infantile basal ganglia stroke after mild head trauma associated with mineralizing angiopathy of lenticulostriate arteries: an under recognized entity. Abbreviation AIS Arterial ischemic stroke In 2014, Lingappa et al.1 described a distinct clinicoradiological stroke syndrome in children, which they called "mineralizing angiopathy". We propose that a subgroup of children with isolated idiopathic basal ganglia AIS after minor head injury have mineralizing angiopathy and warrant consideration of a non-contrast CT to demonstrate the calcifications. Three had silent basal ganglia infarcts detected at the time of acute presentation, four had basal ganglia hemorrhage, and three had parietal or thalamic calcifications (Fig. [Extracted from the article]

The use of alternative therapies in immigrant children with stroke and cerebrovascular diseases has not been reported. We identified immigrant and first-generation Chinese-Canadian children with stroke and cerebrovascular diseases in a pediatric stroke clinic and used standardized questionnaires to interview their parents about alternative therapy use. The chi-square test was used to assess the associations of parental educational level and foreign birth in the child with alternative therapy use. We identified 17 children. 9 boys and 8 girls. Six were immigrants to Canada and 11 were Canadian born. Nine of 17 families (53%) reported using alternative therapies. All 9 used Chinese herbs, but only 3 were able to report the names of specific preparations. Four families were using medications that had the potential to affect coagulation. Documentation of alternative therapy use was found in the medical charts of 3 of the 9 patients. Three families reported feeling uncomfortable discussing alternative therapy use with their physicians. We did not see an association between alternative therapy use and parental educational level or foreign birth in the child. Physicians should be aware that parents of children with stroke may be using alternative therapies to treat their children and may be unwilling or unable to report all therapies used. (J Child Neural 2003;18:714-717). [ABSTRACT FROM AUTHOR]

Cognitive dysfunction has been demonstrated in multiple sclerosis but has not been extensively studied after acute disseminated encephalomyelitis (ADEM). Because ADEM often presents with widespread demyelination, which may not completely resolve, these patients may be at risk for persistent cognitive dysfunction. The study objective was to explore the profile and severity of neurocognitive sequelae in pediatric ADEM. Children aged 6-15 years diagnosed with ADEM were invited to participate in a structured neurologic assessment, neuropsychological evaluation, and a follow-up magnetic resonance imaging. Nine of 15 children diagnosed with ADEM met the age criteria and six participated in the study. The mean age at presentation was 7.7 years; the mean duration of follow-up was 3.5 years. As a group, these children with prior ADEM performed within the average range on cognitive testing. However, a variety of mild cognitive deficits were demonstrated in each of the children, even in those whose magnetic resonance imaging studies had completely normalized. Four children demonstrated a cognitive profile of relatively poorer visuospatial/visuomotor function. The cognitive deficits observed in these children are similar but less severe than those previously reported in adults and children with multiple sclerosis, which may reflect the monophasic nature of ADEM, compared with the chronic, recurrent demyelination characteristic of multiple sclerosis. [Copyright &y& Elsevier]

Few studies have examined walking after neonatal arterial ischemic stroke and sinovenous thrombosis. We looked at the development of walking in a retrospective and consecutive cohort study of 88 term and near-term neonates. We used Kaplan-Meier survival curves and Cox proportional hazards models to assess (1) sex, (2) stroke type (arterial ischemic stroke or sinovenous thrombosis), (3) number of cerebral hemispheres with infarction, and (4) presence of neonatal comorbidity as predictors of the probability over time of starting to walk independently. These variables were assessed as predictors of parent-reported gait normality using the chi-square test on 2 X 2 contingency tables. Seventy-five of 83 survivors (90.4%, 95% confidence interval = 81.9-95.7) walked with a median time of first steps at 13 months of age (95% confidence interval = 12-14). Only bilateral strokes were associated with a lower probability over time of initiating independent walking (hazard ratio = 0.41, P = .04). Parents reported normal gait for 58 of 75 walkers (77.3%, 95% confidence interval = 67.8-86.8). No variables predicted parent-reported gait, normality. Our findings suggest that most survivors of neonatal arterial ischemic stroke and sinovenous thrombosis walk with a gait that appears normal to parents, but bilateral infarctions decrease the probability over time of starting to walk independently. (J Child Neurol 2003; 18:530-536). [ABSTRACT FROM AUTHOR]

The aim of this study was to investigate the sensitivity of cranial ultrasonography for detecting acute arterial ischemic stroke in term neonates. Thirty-six neonates with gestational age 36 weeks who had cranial ultrasonography followed by computed tomography (CT) or magnetic resonance imaging (MRI) confirming arterial ischemic stroke were identified from a consecutive cohort study of all children diagnosed with arterial ischemic stroke by CT or MRI and seen at Chedoke McMaster Hospital between January 1992 and December 1998 or at The Hospital for Sick Children between January 1992 and December 2000. Cranial ultrasonography demonstrated focal abnormalities in 11 patients, giving the initial cranial ultrasonography a sensitivity of 30.5% for identifying neonates with infarction (95% CI 15.5-45.5%). The sensitivity of cranial ultrasonography performed in the two pediatric referral centers (Chedoke McMaster Hospital and Hospital for Sick Children; n = 19) was higher than that in community hospitals (n = 17) (47.3% versus 11.7%; P =.031). Neonates with suspected infarction should be evaluated with CT or MRI. [ABSTRACT FROM AUTHOR]

Examines the diagnosis and management of idiopathic intracranial hypertension in London, England. Occurrence of visual loss secondary to idiopathic intracranial hypertension; Symptoms and signs of increased intracranial pressure; Incidence of idiopathic intracranial hypertension.

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Seven patients with hemolytic-uremic syndrome who had major neurological symptoms during the acute illness were neurologically and cognitively evaluated prospectively several years after recovery from the illness. Four patients showed evidence of subtle neurological sequelae, including posturing, clumsiness, poor fine-motor coordination, hyperactivity, and distractibility. Psychoeducational evaluation of all seven subjects revealed mean scores within the average range in cognitive abilities, academic achievement, single word receptive vocabulary, visual/motor planning, overall adaptive functioning, and hyperactivity. The lapse of time (minimum of 7 years) between the acute illness and the psychometric evaluation could have been responsible for our normal results. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Pediatric arterial ischemic stroke remains incompletely understood. Population-based epidemiological data inform clinical trial design but are scant in this condition. We aimed to determine age-specific epidemiological characteristics of arterial ischemic stroke in neonates (birth to 28 days) and older children (29 days to 18 years).Methods: We conducted a 16-year, prospective, national population-based study, the Canadian Pediatric Ischemic Stroke Registry, across all 16 Canadian acute care children's hospitals. We prospectively enrolled children with arterial ischemic stroke from January 1992 to December 2001 and documented disease incidence, presentations, risk factors, and treatments. Study outcomes were assessed throughout 2008, including abnormal clinical outcomes (stroke-related death or neurological deficit) and recurrent arterial ischemic stroke or transient ischemic attack.Results: Among 1129 children enrolled with arterial ischemic stroke, stroke incidence was 1.72/100,000/year, (neonates 10.2/100,000 live births). Detailed clinical and radiological information were available for 933 children (232 neonates and 701 older children, 55% male). The predominant clinical presentations were seizures in neonates (88%), focal deficits in older children (77%), and diffuse neurological signs (54%) in both. Among neonates, 44% had no discernible risk factors. In older children, arteriopathy (49% of patients with vascular imaging), cardiac disorders (28%), and prothrombotic disorders (35% of patients tested) predominated. Antithrombotic treatment increased during the study period (P < 0.001). Stroke-specific mortality was 5%. Outcomes included neurological deficits in 60% of neonates and 70% of older children. Among neonates, deficits emerged during follow-up in 39%. Overall, an initially decreased level of consciousness, a nonspecific systemic presentation, and the presence of stroke risk factors predicted abnormal outcomes. For neonates, predictors were decreased level of consciousness, nonspecific systemic presentation, and basal ganglia infarcts. For older children, predictors were initial seizures, nonspecific systemic presentation, risk factors, and lack of antithrombotic treatment. Recurrent arterial ischemic stroke or transient ischemic attack developed in 12% of older children and was predicted by arteriopathy, presentation without seizures, and lack of antithrombotic treatment. Emerging deficit was predicted by neonatal age at stroke and by cardiac disease.Conclusions: This national data set provides a population-based disease incidence rate and demonstrates the protective effect of antithrombotic treatment in older children, and frequent long-term emerging deficits in neonates and in children with cardiac disorders. Further clinical trials are required to develop effective age-appropriate treatments for children with acute arterial ischemic stroke. [ABSTRACT FROM AUTHOR]

Aim: We aimed to evaluate whether an institutional acute stroke protocol (ASP) could accelerate the diagnosis and secondary treatment of pediatric stroke.Method: We initiated an ASP in 2005. We compared 209 children (125 males, 84 females; median age 4.8y, interquartile range [IQR] 1.2-9.3y, range 0.09-17.7y) diagnosed with arterial ischemic stroke 'pre-protocol' (1992-2004) to 112 children (60 males, 52 females; median age 5.8y, IQR 1.0-11.4y, range 0.08-17.7y) diagnosed 'post-protocol' (2005-2012) for time-to-diagnosis, mode of diagnostic imaging, and time-to-treatment with antithrombotic medication (aspirin or anticoagulants).Results: Overall, the interval from symptom onset to diagnosis was similar post-protocol compared to pre-protocol (20.3 vs 22.7h; p=0.109), although mild strokes (Pediatric National Institute of Health Stroke Scale [PedNIHSS] 0-4), were diagnosed faster post-protocol (12.1 vs 36.3h; p=0.003). Magnetic resonance imaging (MRI) was the initial diagnostic modality more often post-protocol (25% vs 1.4%; p<0.001). Initial MRI was more accurate for diagnosing stroke than initial CT (100% vs 47%; p<0.001) with similar time-to-diagnosis. The proportion of children receiving antithrombotic medication within 24 hours doubled in the post-protocol period (83% vs 36%; p<0.001).Interpretation: A pediatric ASP accelerated time-to-treatment, time-to-diagnosis in children with subtle strokes, and increased MRI as initial imaging, reducing the need for computed tomography. Implementing optimized ASPs can facilitate more timely access to diagnosis and management of children with acute stroke. [ABSTRACT FROM AUTHOR]

Background: Malignant middle cerebral artery infarct syndrome is a potentially fatal complication of stroke that is poorly understood in children. We studied the frequency, associated characteristics, and outcomes of this condition in children.Methods: Children, aged two months to 18 years with acute middle cerebral artery infarct diagnosed at our center between January 2005 and December 2012 were studied. Associations with malignant middle cerebral artery infarct syndrome were sought, including age, seizures, neurological deficit severity (Pediatric National Institute of Health Stroke Severity Score), stroke etiology, fever, blood pressure, blood glucose, infarct location, infarct volume (modified pediatric Alberta Stroke Program Early Computed Tomography Score), and arterial occlusion. Death and neurological outcomes were determined.Results: Among 66 children with middle cerebral artery stroke, 12 (18%) developed malignant middle cerebral artery infarct syndrome, fatal in three. Prolonged seizures during the first 24 hours (odds ratio, 25.51; 95% confidence interval, 3.10 to 334.81; P = 0.005) and a higher Pediatric National Institute of Health Stroke Severity Score (odds ratio, 1.22; 95% confidence interval, 1.08 to 1.45; P = 0.006) were independently associated with malignant middle cerebral artery infarct syndrome. All children aged greater than two years with a Pediatric National Institute of Health Stroke Severity Score ≥8 and initial seizures ≥5 minutes duration developed malignant middle cerebral artery infarct syndrome (100%).Conclusions: Malignant middle cerebral artery infarct syndrome affects nearly one in five children with acute middle cerebral artery stroke. Children with higher Pediatric National Institute of Health Stroke Severity Scores and prolonged initial seizures are at greatly increased risk for malignant middle cerebral artery infarct syndrome. Children with middle cerebral artery infarcts warrant intensive neuroprotective management and close monitoring to enable early referral for hemicraniectomy surgery. [ABSTRACT FROM AUTHOR]

A response by Ari Bitnun, Jorina M. Elbers, Daune MacGregor, Raymond Tellier and Susan E. Richardson to a letter to the editor about their article "A 12-Year Prospective Study of Childhood Herpes Simplex Encephalitis: Is There a Broader Spectrum of Disease?" in the 2007 issue is presented.

Background: Descending corticospinal tract diffusion-weighted magnetic resonance imaging (MRI) signal is predictive of poor motor outcome in neonatal and childhood arterial ischemic stroke. However, descending corticospinal tract diffusion-weighted MRI signal has not been documented in the setting of cerebral sinovenous thrombosis, and its role is not understood.Objective: We describe a neonate with cerebral sinovenous thrombosis, extensive diffusion restriction, and bilateral descending corticospinal tract diffusion-weighted MRI signal on MRI of the brain. We discuss the underlying mechanisms and implications of these findings in venous ischemia.Conclusion: The prognostic value of descending corticospinal tract diffusion-weighted MRI signal differs when observed in cerebral sinovenous thrombosis from when observed in arterial ischemic stroke. Consequently, caution should be exercised in using descending corticospinal tract diffusion-weighted MRI signal to predict outcome in children with cerebral sinovenous thrombosis. [ABSTRACT FROM AUTHOR]

Aim: To study neuroradiological features in pediatric patients with corpus callosum abnormalities, using new functional subtyping for the corpus callosum, and to correlate the features with the clinical presentation.Method: We performed a retrospective review of 125 patients with radiologically identified abnormalities of the corpus callosum seen between 1999 and 2012. The study reviewed clinical features, genetic etiology, and chromosomal microarray (CMA) results. We used a new functional classification for callosal abnormalities based on embryological and anatomical correlations with four classes: complete agenesis, anterior agenesis (rostrum, genu, body), posterior agenesis (isthmus, splenium), and complete hypoplasia (thinning). We also studied the presence of extracallosal abnormalities.Results: The new functional callosal subtyping did not reveal significant differences between the various subtypes in association with neurological outcome; however, the presence of cardiac disease was found more frequently in the group with complete agenesis. Thirty-seven per cent (46/125) had identifiable causes: of these, 48% (22/46) had a monogenic disorder, 30% (14/46) had a pathogenic chromosomal copy-number variant detected by CMA or karyotype, and 22% (10/46) had a recognizable clinical syndrome for which no confirmatory genetic test was available (namely Aicardi syndrome/septo-optic dysplasia and Goldenhar syndrome). The diagnostic yield for a significant CMA change was 19%. The presence of Probst bundles was found to be associated with a better neurodevelopmental outcome.Interpretation: The functional classification system alone 'without clinical data' cannot predict the functional outcome. The presence of extracallosal brain abnormalities and an underlying genetic diagnosis predicted a worse neurodevelopmental outcome. This study highlights the importance of CMA testing and cardiac evaluation as part of a routine screen. [ABSTRACT FROM AUTHOR]

Background Moyamoya disease is characterized by progressive cerebrovascular stenosis with recurrent cerebral ischemic events. Transient ischemic attacks are often associated with hyperventilation in children with moyamoya, suggesting hypoperfusion rather than thrombotic vaso-occlusion as a prominent mechanism. The patterns of ischemia and severity of steno-occlusive disease in such children may elucidate these mechanisms. Methods Children, 1 month to 18 years, with moyamoya, observed over 11 years were analyzed. A study neuroradiologist reviewed all presurgical neuroimaging. Ischemic injury was categorized into cortical, subcortical, and watershed infarction. Angiographic findings were staged using a standardized method. Results Twenty children, 15 girls, median age 6.4 years, were included. All children had magnetic resonance imaging and angiography, and in 16, conventional angiography was available. All 40 hemispheres, in 20 children, were evaluated. The initial clinical presentation included neurological deficits in 17, recurrent transient ischemic attacks in 7, headache in 8, seizures in 8, and alteration in consciousness in 4 children. Infarcts were bilateral in 13 (65%) children (ischemia alone in 14, ischemic stroke with hemorrhagic transformation in two, and primary hemorrhage in two). Infarcts were cortical and/or subcortical in 13 (65%), both deep and cortical watershed in 11 (55%), and cortical watershed alone in 5 (25%) children. The predominant vascular territory involved was the middle cerebral artery. The internal carotid arterial system was involved in all, with stage IV being the most frequent angiographic stage. Conclusions Ischemic injury in deep watershed zones is common in childhood moyamoya and may reflect non–vaso-occlusive ischemic mechanisms. Location and severity of vascular involvement may correlate with various patterns of ischemic infarction in moyamoya disease and requires further study. [ABSTRACT FROM AUTHOR]

Aim To describe outcomes and outcome predictors in childhood basilar artery stroke ( BAS). Method We prospectively enrolled children with BAS with or without basilar artery occlusion ( BAO) in the Toronto Children's Stroke Registry from 1992 to 2009. We assessed presenting features and outcomes including Pediatric Stroke Outcome Measure scores. Results Among 578 children with acute arterial ischemic stroke, 27 had BAS (4.6% including neonates, 6% excluding neonates). Twenty-four (14 males, 10 females) children met study criteria (mean age at stroke was 8y 10mo; range 0-17y). Eleven children had BAO. Aspirin or anticoagulation was given to 15 children. None received tissue plasminogen activator or endovascular treatments. At mean follow-up (3y 2mo, range 1mo-11y 8mo), 12 had a 'good outcome' (seven normal, five insignificant deficit) and 12 had 'poor outcome' (10 moderate or severe deficit, two acute deaths). Larger infarct size (≥50% of axial brainstem diameter) independently predicted poor outcome ( p=0.02; odds ratio 21.2, 95% confidence interval 1.6-274.9) but not BAO, altered level of consciousness, or age. Interpretation Compared with adults, in childhood BAS death is rare and survivors frequently have good outcomes. Aggressive endovascular interventions may not be justifiable in this population. [ABSTRACT FROM AUTHOR]

Objectives. To describe our experiences in the management of the second wave of influenza A H1N1 (pH1N1) pandemic in a tertiary-care children’s hospital. Methods. An autoethnographic study of the pandemic planning and management committee members involved in managing the second wave of pH1N1 was conducted. Results. Staffing, surge capacity, communications and emergency operations planning by adding leaders of frontline workers and other key operational roles to the incident management team, and creating a tactical response team emerged as important factors in pandemic management in our hospital. The emergency department visits increased by 50%, necessitating increased staffing of the emergency department. Communications using existing chains of command had to be used to reach frontline staff during the pandemic. Conclusions. Incident management teams managing pandemics and other disasters have to be dynamic and create tactical teams to ensure implementation and facilitate bidirectional communication with frontline workers. [ABSTRACT FROM PUBLISHER]

Aim Wallerian degeneration is a radiological finding thought to reflect corticospinal tract degeneration. This finding on magnetic resonance imaging (MRI) is routinely used as a predictor of poor prognosis in childhood stroke. However, its validity has never been established. Our objective was to correlate Wallerian degeneration seen on MRI with histopathology. Method We searched the databases of the Department of Pathology and Children's Stroke registry at the Hospital for Sick Children, Toronto for autopsy specimens exhibiting focal infarcts from children born at term who underwent MRI after a stroke. The specimens were examined for Wallerian degeneration and then correlated with the pre-mortem MRI findings. Results Seven children (four females, three males) with a median age of 11 years (1-17y) at the time of stroke met the inclusion criteria for this study. Of the seven children included in the study with ischaemic or haemorrhagic infarcts, six had concordant Wallerian degeneration findings on both MRI and post-mortem histopathological examination. The median time between stroke and death was 20 days (3-1825d). Interpretation Our results show for the first time that the radiographic finding of Wallerian degeneration is a valid biomarker of corticospinal tract degeneration in children who have had ischaemic or haemorrhagic stroke. [ABSTRACT FROM AUTHOR]

Objective: Pediatric arterial ischemic stroke (AIS) carries an important morbidity and mortality burden. Congenital heart disease (CHD) is among the most important risk factors for pediatric AIS. Data on stroke recurrence in childhood CHD are lacking, resulting in uncertainty regarding optimal strategies for preventing recurrence. Methods: In the Canadian Pediatric Ischemic Stroke Registry-Toronto site, we identified children (birth to 18 years) with CHD diagnosed with AIS during 1992-2008. Data were abstracted from both stroke and cardiac surgery databases. Time-dependent outcomes (death and recurrent stroke) following sentinel stroke were parametrically modeled in competing risk analysis. Factors predicting stroke recurrence in parametric survival models were sought in parametric survival model analyses using backward variable selection of variables. Results: A total of 135 patients (19 with recurrence, 116 without recurrence) were studied. In competing risk analysis, 10 years following sentinel stoke, 27% had experienced a stroke recurrence, 26% had died, and 47% were alive and free from recurrence. Stroke recurrence risk decreased over time from sentinel stroke. Approximately 50% of patients were receiving anticoagulation at recurrence. Significant factors associated with recurrence included the presence of a mechanical valve, prothrombotic condition, and an acute infection at the time of sentinel stroke. Hazard of mortality after recurrence was similar to mortality after sentinel stroke (hazard ratio, 1.3; p = 0.75). Interpretation: Stroke recurrence was relatively common in neonates and children with CHD. Identified groups of patients at increased risk may require more aggressive secondary prophylaxis, especially in the early poststroke period. ANN NEUROL 2012;72:103-111 [ABSTRACT FROM AUTHOR]

Pediatric arterial ischemic stroke (AIS) is increasingly diagnosed and carries significant risks of recurrence, morbidity, and mortality. Anticoagulant therapy (ACT) is commonly prescribed in childhood AIS. Hemorrhagic complication rates in pediatric stroke are unknown, and adult safety data are of limited applicability. We analyzed a prospectively enrolled cohort of children (aged 1 month-18 years) with acute AIS selected using standardized criteria for protocol-based ACT over 14-year period. We assessed ACT-associated intracranial hemorrhage (ICH), including frequency, clinical and radiologic characteristics, predictors, and outcome. Among 215 children with AIS, 123 received ACT within 7 days after diagnosis. During anticoagulation, 14(11%) children developed new or increased ICH, all within 26 days from diagnosis. ICH was symptomatic in 5 (4%), asymptomatic in 9 (7%), and mild (European Cooperative Acute Stroke Study grades HIl or HI2) in all but 1 child (ECASS PH-2). Long-term neurologic outcomes after ACT-associated ICH in survivors were abnormal in 73% (8/11). Comparably, 12 of 75 (16%) children treated without anticoagulation developed new or increased ICH on follow-up imaging (P = .3507). We conclude that ACT is relatively safe in children with AIS, with a 4% risk of symptomatic ICH. Based on the safety of ACT in our study, clinical trials of ACT in childhood AIS are warranted. (Blood. 2012;119(4):949-956) [ABSTRACT FROM AUTHOR]

Aim Moyamoya vasculopathy is characterized by progressive stenosis of the major arteries of the Circle of Willis, resulting in compromised cerebral blood flow and increased risk of stroke. The objectives of the current study were to examine intellectual and executive functioning of children with moyamoya and to evaluate the impact of moyamoya type, stroke (clinical or silent), vasculopathy laterality, and disease duration on neurocognitive abilities. Method Thirty pediatric participants (mean age 10y 10mo, SD 4y; 18 females, 12 males) completed age-appropriate Wechsler Intelligence Scales before any therapeutic revascularization procedures. Reports of executive function were obtained from parents and teachers using the Behavior Rating Index of Executive Function. Results Children with moyamoya scored significantly lower than the test standardization samples on all indices of intelligence and ratings of executive functioning ( p<0.001). Patients did not differ by type of moyamoya or history of stroke. Patients with bilateral disease and stroke scored significantly lower than those with unilateral disease on measures of overall intellectual function ( p=0.035) and verbal comprehension ( p=0.04). Deficits in metacognitive executive functions were also more pronounced in bilateral patients according to teacher ratings. Interpretation Children with moyamoya are at risk for intellectual and executive problems, exacerbated by bilateral disease and clinical stroke history. [ABSTRACT FROM AUTHOR]

Neonatal cerebral sinovenous thrombosis is a frequent contributor to neonatal mortality and morbidity. Treatment is controversial, and reported clinical outcomes vary widely. Newborns with radiologically confirmed neonatal cerebral sinovenous thrombosis from 1992 to 2009 were prospectively followed in our Children’s Stroke Clinic for standardized outcomes, including the Pediatric Stroke Outcome Measure. Outcomes were available in 90 of 104 (87%) neonates. Early outcomes included cerebral sinovenous thrombosis-associated death (5) and thrombus propagation (15 [6 associated with new venous infarcts]). Lack of anticoagulation predicted propagation (RR = 13; P = .0007). Complete thrombus recanalization occurred in 90% by 3 months. Late outcomes (median, 2.5 years) were epilepsy (15) and neurological disability (50), which included moderate-severe language (43), sensorimotor (38), and cognitive/behavioral (24) deficits. Overall, 61% had poor outcome (death/any deficit). Concurrent neurological comorbidity at diagnosis (odds ratio = 2.8; P = .029) predicted poor outcome. Clinical trials are urgently needed to establish more effective treatment strategies. [ABSTRACT FROM AUTHOR]

Objective Clinical trials are lacking in pediatric cerebral sinovenous thrombosis (CSVT). Neonates and children increasingly receive anticoagulant therapy (ACT) based on adult studies. Safety data for ACT in pediatric CSVT are scant and urgently needed. The objective was to assess the safety and outcome of ACT in pediatric CSVT. Methods In a single-center prospective study, neonates and children with CSVT received ACT (standard/low molecular weight heparin, warfarin) by standardized protocol. A study neuroradiologist (M.S.) assessed all initial and follow-up neuroimaging for intracranial hemorrhage (ICH), thrombus propagation, and recanalization. Clinical outcome was assessed with the Pediatric Stroke Outcome Measure. Results Among 162 pediatric patients, 85 received ACT at diagnosis, including 29/83 (35%) neonates and 56/79 (71%) children. Major hemorrhage occurred in 6% (6/99) of treated patients, including 14% (3/21 neonates, 2/15 children) with and 2% (0/17 neonates, 1/46 children) without pretreatment ICH. ACT-associated bleeds were all nonfatal, and clinical outcome was favorable in 50%, similar to the remaining patients (53%). Early follow-up imaging demonstrated thrombus propagation in 11/57 neonates (10/35 [28%] without and 1/22 [4%] with ACT [ p = 0.037]) and 10/63 children (7/19 [37%] without and 3/44 [7%] with ACT [ p = 0.006]). Propagation was associated with new venous infarcts in 10% neonates and 40% children and worse clinical outcome in children ( p = 0.053). Recanalization occurred earlier and more completely in neonates ( p = 0.002). Clinical outcome was unfavorable in 47%. Interpretation In pediatric CSVT, ACT appears safe. Nontreatment with ACT is associated with thrombus propagation, observed in ¼ of untreated neonates and over ⅓ of children. Anticoagulants merit strong consideration in pediatric CSVT. ANN NEUROL 2010;67:590-599 [ABSTRACT FROM AUTHOR]

To investigate upper limb cerebellar motor function in children with spina bifida myelomeningocele (SBM) and in typically developing controls. Participants with SBM, who had either upper level spinal lesions ( n = 23) or lower level spinal lesions ( n = 65), and controls ( n = 37) completed four upper limb motor function tasks (posture, rebound, limb dysmetria, and diadochokinesis) under four different physical and cognitive challenge conditions. Functional independence was assessed by parental questionnaire. Fewer SBM participants were able to complete the posture task, and they were less likely than controls to obtain a perfect rebound score. Participants with SBM showed impaired performance in either time, accuracy, or both, on the limb dysmetria and diadochokinesis tasks but responded like controls to physical and cognitive challenges. Because upper limb motor performance predicted aspects of functional independence, we conclude that upper limb impairments in children with SBM are significant and have direct implications for the level of independent functioning in children with SBM. [ABSTRACT FROM AUTHOR]

Craniocervical arterial dissection is a frequent cause of childhood arterial ischemic stroke requiring prompt diagnosis and treatment. However, there is no universal guideline for diagnostic cerebrovascular imaging in children. We assessed the role of time-of-flight magnetic resonance angiography in diagnosing craniocervical arterial dissection. We included children (1 month to 18 years) with craniocervical arterial dissection and ischemic stroke from January 1998 to June 2007. Institutional protocol required magnetic resonance imaging (MRI)/ magnetic resonance angiography for all ischemic stroke cases and conventional cerebral angiography if magnetic resonance angiography showed any arteriopathy. Among 233 ischemic stroke cases, craniocervical arterial dissection was diagnosed in 13 patients (5.6%; 10 males), with a mean age of 7.5 years. Initial time-of-flight magnetic resonance angiography missed dissection in 8 patients (61.5%) because the neck vessels were not scanned (n = 2), of suboptimal technique (n = 3), and of diagnostic error (n = 3). T1 fat-saturated MRI and contrast-enhanced magnetic resonance angiography were not performed in 12 patients. If suspicion for arterial dissection is high, T1 fat-saturated neck imaging and contrast-enhanced magnetic resonance angiography should be considered to maximize detection. [ABSTRACT FROM AUTHOR]

Thrombolysis is considered to be contraindicated in acute ischemic stroke secondary to infective endocarditis (IE). We report a 12-year-old female who presented with acute dense right hemiparesis and aphasia. Cranial magnetic resonance imaging and angiography showed multiple diffusion-restricted lesions in the left hemisphere and absence of flow in the left internal carotid artery. She was treated with intra-arterial tissue plasminogen activator within 6 hours of her presentation. Subsequently she was diagnosed with pneumococcal endocarditis and underwent debridement of vegetations and patch repair of the mitral valve. The patient did not have hemorrhagic complications following thrombolytic therapy or surgery. Pathological analysis of the mitral valve vegetations revealed mostly fibrin thrombus. Follow-up imaging showed complete recanalization of the left internal carotid artery, and the patient had a remarkable neurological recovery. This is the first case report of successful intra-arterial thrombolytic therapy in childhood IE-related stroke. We believe that thrombolytic therapy contributed to a favorable outcome in our patient and may be safe in selected patients with childhood IE-related acute ischemic stroke. [ABSTRACT FROM AUTHOR]

OBJECTIVE. Our aim was to define the frequency, predictors, and outcomes of stroke associated with cardiac surgery in children with congenital heart disease. METHODS. We performed a case-control study of children (term birth to 18 years) with congenital heart disease who underwent cardiac surgery at the Hospital for Sick Children between January 1, 1992, and March 1, 2001. Case subjects experienced stroke within 72 hours after cardiac surgery, and control subjects (2 for each case subjects) had cardiac surgery and no stroke. The frequency of arterial ischemic stroke/cerebral sinovenous thrombosis was calculated among children who underwent cardiac surgery during the study period. Predictors for stroke, including age, gender, simple versus complex procedure, reoperation, bypass duration, circulatory arrest, postoperative hematocrit level, and intraoperative activated clotting time, were tested. The presence of clinical and radiologically defined stroke was the main outcome. Neurologic outcomes were assessed in case subjects with the Pediatric Stroke Outcome Measure. RESULTS. During the study period, 30 children with stroke (28 with arterial ischemic stroke and 2 with cerebral sinovenous thrombosis) were identified among 5526 children undergoing cardiac surgery. This yielded a risk for arterial ischemic stroke/ cerebral sinovenous thrombosis of 5.4 strokes per 1000 children undergoing a cardiac operation. Univariate analysis revealed that older age at the time of the procedure, longer duration of cardiopulmonary bypass, number of days in the hospital postoperatively, and reoperation were associated with stroke. In multivariate analyses, only reoperation was associated with stroke. CONCLUSIONS. The frequency of vaso-occiusive stroke in children with congenital heart disease undergoing cardiac surgery was 5.4 cases per 1000 children. Age, duration of bypass, and reoperation may be associated with stroke risk. [ABSTRACT FROM AUTHOR]

OBJECTIVES. Hypoglycemia is a significant problem in neonates, and a pattern of parietooccipital diffusion restriction on MRI scans has been reported. The purpose of this study was to determine whether hypoglycemic injury, as indicated by diffusion restriction in the occipital lobes, correlated with visual evoked potentials and long-term cortical visual dysfunction. METHODS. A cohort of 45 neonates from 2000-2005 with diffusion-weighted MRI studies after hypoglycemia was studied retrospectively. Perinatal history and follow-up data were analyzed, and results were correlated with diffusion-weighted imaging findings. The presence of occipital diffusion restriction was assessed qualitatively, and the mean apparent diffusion coefficients of mesial occipital lobes were calculated. RESULTS. Among 25 patients who underwent diffusion-weighted imaging within 6 days after the onset of hypoglycemia, restricted diffusion in the occipital lobes was found in 8 (50%) of 16 term infants but not in preterm infants. For the remaining 20 patients, who had diffusion-weighted imaging performed >6 days after the initial onset of hypoglycemia, occipital diffusion restriction was not seen, even if hypoglycemia was ongoing. Restricted diffusion was associated with abnormal visual evoked potentials detected within 1 week after birth. Cortical visual deficits were seen in a significant proportion of patients with recurrent hypoglycemia and were correlated significantly with low mesial occipital apparent diffusion coefficient values. CONCLUSIONS. Diffusion-weighted imaging studies performed within 6 days after initial hypoglycemia were sensitive in term but not preterm neonates. Diffusion restriction, with low apparent diffusion coefficient values, in the mesial occipital poles may indicate the prognosis for visual outcomes in acute settings after neonatal hypoglycemia. [ABSTRACT FROM AUTHOR]

Objective Perinatal stroke commonly causes childhood neurological morbidity. Presumed perinatal ischemic stroke (PPIS) defines children presenting outside a normal perinatal period with chronic, focal infarction on neuroimaging. Infarcts are assumed to represent arterial strokes, but recent evidence suggests the periventricular venous infarction (PVI) of infants born preterm may also occur in utero and present as PPIS. Using the largest published cohort, we aimed to define arterial and PVI PPIS syndromes and their outcomes. Methods A PPIS consecutive cohort was identified (SickKids Children's Stroke Program, 1992-2006). Systematic neuroradiological scoring executed by blinded investigators included previously defined arterial stroke syndromes. PVI criteria included unilateral injury with at least four of the following conditions: (1) focal periventricular encephalomalacia, (2) internal capsule T2 prolongation, (3) cortical and (4) relative basal ganglia sparing, and (5) remote hemorrhage. Arterial and PVI classifications were validated and correlated with neurological outcomes (Pediatric Stroke Outcome Measure). Results In 59 PPISs (64% male), 94% of lesions fell within potential middle cerebral artery territories. Although arterial proximal M1 infarction was most common (n = 19; 35%), venous PVI was second (n = 12; 22%) and accounted for 75% of subcortical injuries. Motor outcomes (mean follow-up, 5.3 years) were predicted by basal ganglia involvement including leg hemiparesis, spasticity, and need for assistive devices ( p < 0.01). Nonmotor outcomes were associated with cortical involvement, including cognitive/behavioral outcomes, visual deficits, and epilepsy ( p < 0.01). Classification interrater reliability was excellent (correlation coefficients > 0.975). Interpretation Recognizable PPIS patterns predict long-term morbidity and may guide surveillance, therapy, and counseling. PVI is an underrecognized cause of PPIS and congenital hemiplegia. Ann Neurol 2008 [ABSTRACT FROM AUTHOR]

Many neurologic manifestations of Epstein-Barr virus (EBV) infection have been documented, including encephalitis, aseptic meningitis, transverse myelitis, and Guillain-Barré syndrome. These manifestations can occur alone or coincidentally with the clinical picture of infectious mononucleosis. Since 1994, The Hospital for Sick Children has maintained a prospective registry of all children admitted with acute encephalitis. This report summarizes all cases of Epstein-Barr virus-associated encephalitis compiled from 1994 to 2003. Twenty-one (6%) of 216 children, median age 13 years (range 3-17 years), in the Encephalitis Registry were identified as having evidence of Epstein-Barr virus infection. This evidence consisted of convincing Epstein-Barr virus serology and/or positive cerebrospinal fluid polymerase chain reaction (PCR). One patient had symptoms of classic infectious mononucleosis; all others had a nonspecific prodrome, including fever (n = 17; 81%) and headache (n = 14; 66%). Slightly less than half (n = 10; 48%) had seizures and often had electroencephalograms showing a slow background (n = 12; 57%). Many demonstrated cerebrospinal fluid pleocytosis (n = 17; 81%), and 71% (n = 15) had abnormal magnetic resonance imaging findings. Two patients died, 2 suffered mild deficits, and 16 were neurologically normal at follow-up. Most patients with Epstein-Barr virus encephalitis do not show typical symptoms of infectious mononucleosis. Establishing a diagnosis of Epstein-Barr virus encephalitis can be difficult, and, consequently, a combination of serologic and molecular techniques should be used when investigating a child with acute encephalitis. Most children make full recoveries, but residual neurologic sequelae and even death can and do occur. [ABSTRACT FROM AUTHOR]

Craniocervical arterial dissection is a recognized cause of arterial ischemic stroke in children. Whether children with craniocervical arterial dissection have dissection characteristics different from those of adults is unclear. A retrospective review of children, 1 month to 18 years of age, with dissection from two Canadian pediatric ischemic stroke registry centers was conducted. From 213 patients with arterial ischemic stroke, 16 (7.5%) were identified with dissection, 37.5% had warning symptoms, and 50% had a history of head or neck trauma. The clinical presentation included headache (44%), altered consciousness (25%), seizures (12.5%), and focal deficits (87.5%). Dissection involved extracranial vessels in 75% and anterior circulation in 56%. Follow-up included complete recovery in 43%, mild to moderate deficits in 44%, and severe deficits in 13%. Fourteen (87.5%) children received antithrombotic treatment. Follow-up angiography showed resolution of abnormalities in 60% of vessels. Total occlusion had the worst outcome for recanalization. In conclusion, the etiology of arterial dissection in the majority of children appears to be either trauma or idiopathic. Long-term angiography shows variable outcomes, depending on the initial findings. The relationship of angiographic outcomes with recurrent strokes requires further study in pediatric dissection. [ABSTRACT FROM AUTHOR]

Presents a study which demonstrated evidence of Mycoplasma pneumoniae infection in children with acute encephalitis. Patients and methods; Results and discussion.