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The goal of this study was to examine executive functioning, math performance, and visuospatial processing skills of children with perinatal stroke, which have not been well explored in this population. Participants included 18 children with perinatal stroke (aged 6-16 years old) and their primary caregiver. Each child completed standardized tests of executive function and visuospatial processing skills, Intelligence Quotient (IQ), and math achievement. Performance on executive function, IQ, math, and visuospatial processing tests was significantly lower in children with perinatal stroke when compared to normative means. Poorer inhibitory control was associated with worse math performance. Increased age at testing was associated with better performance on visuospatial ability (using standardized scores), and females performed better than males on a test of inhibitory control. Children with perinatal stroke displayed a range of neuropsychological impairments, and difficulties with executive function (inhibition) may contribute to math difficulties in this population. [ABSTRACT FROM AUTHOR]

Background: Perinatal stroke is a leading cause of hemiparetic cerebral palsy and lifelong disability. Neurodevelopmental outcomes are difficult to predict and markers of long-term poor outcome continue to be investigated. Deceleration in growth of head circumference has been associated with worse developmental outcomes in neonatal brain injury. We hypothesized that perinatal stroke would result in decreased rates of head growth during childhood that would be associated with worse developmental outcomes. Methods: Patients with magnetic resonance imaging (MRI)–confirmed neonatal arterial ischemic stroke and arterial presumed perinatal ischemic stroke were identified from a population-based research cohort (Alberta Perinatal Stroke Project). Demographics and occipital-frontal circumference data were collected from medical records. Head growth was compared to typically developing control charts using a 2-tailed t test. The Fisher exact test was used to examine associations between Pediatric Stroke Outcome Measures (PSOM) scores and occipital-frontal head circumference. Results: Three hundred fifteen occipital-frontal head circumference measurements were collected from 102 patients (48 female, 54 male), over a median of 3.2 years (standard deviation = 5.18, range = 0-18.3). After 3 months for female patients and 1 year for male patients, occipital-frontal head circumference deviated and remained below normal growth trajectories (P <.05) with a large effect size (Cohen d >0.8). Poor outcome (PSOM ≥ 1) was associated with smaller occipital-frontal head circumference (P <.05). Conclusion: Head growth deceleration is observed in children with perinatal arterial ischemic stroke and is associated with poor outcome. Head circumference may be a tool to alert clinicians to the potential of abnormal neurologic outcome. [ABSTRACT FROM AUTHOR]

The Canadian Stroke Best Practice Consensus Statement: Secondary Stroke Prevention during Pregnancy, is the first of a two-part series devoted to stroke in pregnancy. This document focuses on unique aspects of secondary stroke prevention in a woman with a prior history of stroke or transient ischemic attack who is, or is planning to become, pregnant. Although stroke is relatively rare in this cohort, several aspects of pregnancy can increase stroke risk during or immediately after pregnancy. The rationale for the development of this consensus statement is based on the premise that stroke in this group requires a specifically-tailored management approach. No other broad-based, stroke-specific guidelines or consensus statements exist currently. Underpinning the development of this document was the concept that maternal health is vital for fetal wellbeing; therefore, management decisions should be based on the confluence of two clinical considerations: (a) decisions that would be made if the patient was not pregnant and (b) decisions that would be made if the patient had not had a stroke. While empirical research in this area is limited, this consensus document is based on the best available literature and guided by expert consensus. Issues addressed in this document include general management considerations for secondary stroke prevention, the use of antithrombotics, blood pressure management, lipid management, diabetes care, and management for specific ischemic stroke etiologies in pregnancy. The focus is on maternal and fetal health while minimizing risks of a recurrent stroke, through counseling, monitoring, and the safety of select pharmacotherapy. These statements are appropriate for health care professionals across all disciplines. [ABSTRACT FROM AUTHOR]

This study aimed to describe children with moyamoya disease from an international multicenter stroke database, and explore risk factors for stroke recurrence. We reviewed data of children >28-days old with moyamoya disease enrolled in the International Pediatric Stroke Study from January 2003 to March 2013. A total of 174 children from 32 sites and 14 countries had moyamoya disease; median age 7.4 years, 49% male. Of these, 90% presented with ischemic stroke, 7.5% with transient ischemic attack, and 2.5% with hemorrhagic stroke. One-third of patients had moyamoya syndrome. Stroke recurrence was 20% over median follow-up of 13 months; 9% had multiple recurrences. Children treated with surgical revascularization were less likely to have stroke recurrence (P = .046). Moyamoya disease accounted for 8% of arterial strokes in this international pediatric stroke registry. One-third of pediatric patients with moyamoya disease have an underlying syndromic condition. Surgical revascularization is effective at reducing the incidence of stroke recurrence. [ABSTRACT FROM AUTHOR]

Perinatal stroke injures developing motor systems, resulting in hemiparetic cerebral palsy. Diffusion tensor imaging can explore structural connectivity. We used diffusion tensor imaging to assess corticospinal tract diffusion in hemiparetic children with perinatal stroke. Twenty-eight children (6-18 years) with unilateral stroke underwent diffusion tensor imaging. Four corticospinal tract assessments included full tract, partial tract, minitract and region of interest. Diffusion characteristics (fractional anisotropy, mean, axial, and radial diffusivity) were calculated. Ratios (lesioned/nonlesioned) were compared across segments and to validated long-term motor outcomes (Pediatric Stroke Outcome Measure, Assisting Hand Assessment, Melbourne Assessment). Fractional anisotropy and radial diffusivity ratios decreased as tract size decreased, while mean diffusivity showed consistent symmetry. Poor motor outcomes were associated with lower fractional anisotropy in all segments and radial diffusivity correlated with both Assisting Hand Assessment and Melbourne Assessment. Diffusion imaging of segmented corticospinal tracts is feasible in hemiparetic children with perinatal stroke. Correlations with disability support clinical relevance and utility in model development for personalized rehabilitation. [ABSTRACT FROM AUTHOR]

Background: Diffusion-weighted imaging magnetic resonance imaging may detect changes in brain structures remote but connected to stroke consistent with neuropathological descriptions of diaschisis. Early diffusion-weighted imaging demonstrates restriction in corticospinal pathways after arterial ischemic stroke of all ages that correlates with motor outcome. Aim/hypothesis: We hypothesized that cerebral diaschisis is measurable in childhood arterial ischemic stroke and explored associations with outcome. Methods: This sub-study of the validation of the Pediatric NIH Stroke Scale study prospectively enrolled children with acute arterial ischemic stroke and both acute and early follow-up (5-14 days) diffusion-weighted imaging. Inclusion criteria were (1) unilateral middle cerebral artery arterial ischemic stroke, (2) acute and subacute diffusion-weighted imaging (b=1000), and (3) 12 month neurological follow-up (Pediatric Stroke Outcome Measure). A validated method using ImageJ software quantified diffusion-weighted imaging diaschisis in anatomically connected structures. Diaschisis measures were corrected for infarct volume, compared to age, imaging timing, and outcomes (Chi square/Fisher, Mann-Whitney test). Results: Nineteen children (53% male, median 8.1 years) had magnetic resonance imaging at medians of 21 and 168 h post-stroke onset. Diaschisis was common and evolved over time, observed in one (5%) on acute but eight (42%) by follow-up diffusion-weighted imaging. Thalamic and callosal diaschisis were most common (5, 26%). Estimates of perilesional diaschisis varied (54±18% of infarct volume). Children with diaschisis tended to be younger (7.02±5.4 vs. 11.82±4.3 years, p=0.08). Total diaschisis score was associated with poor cognitive outcomes (p=0.03). Corticospinal tract diaschisis was associated with motor outcome (p=0.004). Method reliability was excellent. Conclusions: Diffusion-weighted imaging diaschisis occurs in childhood arterial ischemic stroke. Mistaking diaschisis for new areas of infarction carries important clinical implications. Improved recognition and study are required to establish clinical relevance. [ABSTRACT FROM AUTHOR]

Stroke rehabilitation is a progressive, dynamic, goal-orientated process aimed at enabling a person with impairment to reach their optimal physical, cognitive, emotional, communicative, social and/or functional activity level. After a stroke, patients often continue to require rehabilitation for persistent deficits related to spasticity, upper and lower extremity dysfunction, shoulder and central pain, mobility/gait, dysphagia, vision, and communication. Each year in Canada 62,000 people experience a stroke. Among stroke survivors, over 6500 individuals access in-patient stroke rehabilitation and stay a median of 30 days (inter-quartile range 19 to 45 days). The 2015 update of the Canadian Stroke Best Practice Recommendations: Stroke Rehabilitation Practice Guidelines is a comprehensive summary of current evidence-based recommendations for all members of multidisciplinary teams working in a range of settings, who provide care to patients following stroke. These recommendations have been developed to address both the organization of stroke rehabilitation within a system of care (i.e., Initial Rehabilitation Assessment; Stroke Rehabilitation Units; Stroke Rehabilitation Teams; Delivery; Outpatient and Community-Based Rehabilitation), and specific interventions and management in stroke recovery and direct clinical care (i.e., Upper Extremity Dysfunction; Lower Extremity Dysfunction; Dysphagia and Malnutrition; Visual-Perceptual Deficits; Central Pain; Communication; Life Roles). In addition, stroke happens at any age, and therefore a new section has been added to the 2015 update to highlight components of stroke rehabilitation for children who have experienced a stroke, either prenatally, as a newborn, or during childhood. All recommendations have been assigned a level of evidence which reflects the strength and quality of current research evidence available to support the recommendation. The updated Rehabilitation Clinical Practice Guidelines feature several additions that reflect new research areas and stronger evidence for already existing recommendations. It is anticipated that these guidelines will provide direction and standardization for patients, families/caregiver(s), and clinicians within Canada and internationally. [ABSTRACT FROM AUTHOR]

Every year, approximately 62 000 people with stroke and transient ischemic attack are treated in Canadian hospitals, and the evidence suggests one-third or more will experience vascular-cognitive impairment, and/or intractable fatigue, either alone or in combination. The 2015 update of the Canadian Stroke Best Practice Recommendations: Mood, Cognition and Fatigue Module guideline is a comprehensive summary of current evidence-based recommendations for clinicians in a range of settings, who provide care to patients following stroke. The three consequences of stroke that are the focus of the this guideline (poststroke depression, vascular cognitive impairment, and fatigue) have high incidence rates and significant impact on the lives of people who have had a stroke, impede recovery, and result in worse long-term outcomes. Significant practice variations and gaps in the research evidence have been reported for initial screening and in-depth assessment of stroke patients for these conditions. Also of concern, an increased number of family members and informal caregivers may also experience depressive symptoms in the poststroke recovery phase which further impact patient recovery. These factors emphasize the need for a system of care that ensures screening occurs as a standard and consistent component of clinical practice across settings as stroke patients transition from acute care to active rehabilitation and reintegration into their community. Additionally, building system capacity to ensure access to appropriate specialists for treatment and ongoing management of stroke survivors with these conditions is another great challenge. [ABSTRACT FROM AUTHOR]

National organizations recommend cholesterol screening in children to prevent vascular disease in adulthood. There are currently no recommendations for cholesterol and lipoprotein (a) testing in children who experience an arterial ischemic stroke. While dyslipidemia and elevated lipoprotein (a) are associated with ischemic stroke in adults, the role of atherosclerotic risk factors in childhood arterial ischemic stroke is not known. A review of the literature was performed from 1966 to April 2012 to evaluate the association between childhood arterial ischemic stroke and dyslipidemia or elevated lipoprotein (a). Of 239 citations, there were 16 original observational studies in children (with or without neonates) with imaging-confirmed arterial ischemic stroke and data on cholesterol or lipoprotein (a) values. Three pairs of studies reported overlapping subjects, and two were eliminated. Among 14 studies, there were data on cholesterol in 7 and lipoprotein (a) in 10. After stroke, testing was performed at >three-months in nine studies, at ≤three-months in four studies, and not specified in one study. There were five case-control studies: four compared elevated lipoprotein (a) and one compared abnormal cholesterol in children with arterial ischemic stroke to controls. A consistent positive association between elevated lipoprotein (a) and stroke was found [ Mantel- Haenszel OR 4·24 (2·94-6·11)]. There was no association in one study on total cholesterol, and a positive association in one study on triglycerides. The literature suggests that elevated lipoprotein (a) may be more likely in children with arterial ischemic stroke than in control children. The absence of confirmatory study on dyslipidemia should be addressed with future research. [ABSTRACT FROM AUTHOR]

Amanda Leong, BSc I Calgary Pediatric Stroke Program, Alberta Children's Hospital, Calgary, Alberta, Canada i Amalia Floer I Calgary Pediatric Stroke Program, Alberta Children's Hospital, Calgary, Alberta, Canada i Adam Kirton, MD I Calgary Pediatric Stroke Program, Alberta Children's Hospital, Calgary, Alberta, Canada, Department of Pediatrics and Clinical Neurosciences, Alberta Children's Hospital, Calgary, Alberta, Canada i Aleksandra Mineyko, MD I Calgary Pediatric Stroke Program, Alberta Children's Hospital, Calgary, Alberta, Canada, Department of Pediatrics and Clinical Neurosciences, Alberta Children's Hospital, Calgary, Alberta, Canada i References 1 Chabrier S, Peyric E, Drutel L; et al. Thank you for forwarding to us the correspondence from Garel et al in response to our article "Head circumference trajectory in children with perinatal stroke.". [Extracted from the article]

Occipital bone compression of the superior sagittal sinus occurs in supine neonates. The authors previously showed that this compression is associated with neonatal cerebral sinovenous thrombosis, an important neonatal neurological condition, presumably via increased venous stasis. They hypothesized that a pillow alleviating occipital compression could improve cerebral venous flow. Neonates without cerebral sinovenous thrombosis requiring brain magnetic resonance imaging/venography were prospectively enrolled. Demographics, imaging indications, head position, and superior sagittal sinus compression were recorded. Flow velocities in the anterior and posterior superior sagittal sinus and sigmoid sinus before and after pillow placement were quantified using Doppler ultrasound. A total of 10 neonates (6 female, median postconceptional age 40.6 weeks) were enrolled. Flow velocities increased beyond the superior sagittal sinus compression point following pillow placement: 0.09 ± 0.04 m/sec to 0.15 ± 0.12 m/sec (P = .047) for posterior superior sagittal sinus; 0.10 ± 0.05 m/sec to 0.19 ± 0.15 m/sec (P = .005) for sigmoid sinus. Pillow decompression can improve neonatal cerebral venous flow, representing a potential noninvasive intervention for the prevention and treatment of neonatal cerebral sinovenous thrombosis. [ABSTRACT FROM AUTHOR]

Mitochondrial diseases are increasingly being recognized as causes of encephalopathy and intractable epilepsy. There is no gold-standard test for diagnosing mitochondrial disease, and the current diagnosis relies on establishing a consistent pattern of evidence from clinical data, neuroimaging, tissue biopsy, and biochemical, genetic, and other investigations. Experience in the diagnosis and treatment of patients with certain forms of mitochondrial disease, such as Alpers syndrome, is largely gained from case reports or small case series. The authors describe a case of Alpers syndrome due to POLG1 mutations, including serial neuroimaging and pathological investigations, to illustrate two main points: (1) Unique characteristics evident on serial diffusion-weighted imaging can be a valuable indicator of Alpers syndrome; and (2) abnormal lipid metabolism can be present in Alpers syndrome, which may need to be considered when using a ketogenic diet. [ABSTRACT FROM PUBLISHER]

An improved understanding of perinatal stroke epidemiology, classification, neuroimaging, and outcomes has emerged in recent years. Despite this, little is known regarding the pathophysiological mechanisms responsible for most cases. A multitude of possible associations and putative risk factors have been reported, but most lack definitive empirical evidence supporting primary causation. These include obstetrical and maternal factors, perinatal conditions, infectious diseases, prothrombotic abnormalities, cardiac disorders, medications, and many others. The bulk of evidence is weak, dominated by case reports and retrospective case series. Findings from the small number of case-control and cohort studies that exist are limited by heterogeneous populations and methodologies. The single largest barrier to ultimately understanding and potentially improving outcomes from this common and disabling condition is the lack of comprehensive, fully powered risk factor studies required to definitively describe perinatal stroke pathogenesis. This review summarizes current evidence and suggests future directions for research. [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]

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]

Acute necrotizing encephalopathy is a fulminant neurologic disease seen predominantly in Japan and Taiwan. We present two cases diagnosed at a Canadian center within the same year in Caucasian children. Both were previously well, developed an acute viral illness with fever and vomiting, and progressed to brain death within 2 to 4 days. Neuroimaging and postmortem examination demonstrated the unique features of bilateral and severe necrosis of deep gray-and subcortical white-matter structures. The first case was associated with extensive, but transient, hepatic involvement, recent varicella and rotavirus infections, and detailed metabolic studies, including mitochondrial functional analysis, were normal. The second case tested positive for influenza A infection, whereas evidence of liver damage was lacking. Both children demonstrated early lymphopenia and myocardial necrosis, two features not previously associated with acute necrotizing encephalopathy. These cases are unique in their occurrence in non-Japanese children and are among the first published reports in Canada. (J Child Neurol 2005;20:527-532). [ABSTRACT FROM AUTHOR]

The physical manifestations a seizure produces provide critical information. It is assumed that all generalized convulsions are ostensibly the same, regardless of whether they are primary or secondary generalized seizures. We undertook a pilot study to determine if the clinical phenomenology of secondary generalized seizures in children with epilepsy is different from classic descriptions of generalized tonic-clonic convulsions. A data capture sheet was created and applied to the video-electroencephalographic (EEG) records of 64 secondary generalized seizures from 13 children with intractable and/or refractory epilepsy. Many features of secondary generalized seizures were different from traditional descriptions of generalized convulsions. In 100% of cases, the mouth either remained open or repeatedly opened and closed rather than slamming shut. In 77% of cases, a variety of late motor activities were seen to occur after the seizure activity had ceased and the EEG record was quiet. The clinical features of a generalized convulsion in a child, especially mouth opening and late motor events, can be useful in establishing the origin as either focal or primary generalized. (J Child Neurol 2004:19:265-2701 [ABSTRACT FROM AUTHOR]

The incidence of spinal injuries is increased in people with epilepsy although compressive thoracic myelopathy has not been reported. We describe a 15-year-old girl with SCN1A mutation (Dravet syndrome), refractory generalized tonic-clonic seizures, and prior posterior instrumentation and fusion for scoliosis, who presented with progressive lower extremity weakness. Junctional kyphosis with disc herniation and spinal cord compression directly rostral to the instrumentation was apparent on imaging. On history, the patient had suffered a particularly severe convulsive seizure just before developing symptoms. Surgical decompression and stabilization led to a complete neurologic recovery. This unusual presentation of myelopathy illustrates the need to consider this complication in patients with epilepsy and spinal instrumentation. [ABSTRACT FROM AUTHOR]