scholarly journals Focal Cerebral Arteriopathy of Childhood: Clinical and Imaging Correlates

Stroke ◽  
2021 ◽  
Author(s):  
Gabriela Oesch ◽  
Francisco A. Perez ◽  
Mark S. Wainwright ◽  
Dennis W.W. Shaw ◽  
Catherine Amlie-Lefond

Background and Purpose: Focal cerebral arteriopathy (FCA) of childhood with unilateral stenosis of the anterior circulation is reported to account for up to one-quarter of childhood arterial ischemic stroke, with stroke recurrence in 25% of cases. Limited knowledge regarding pathophysiology and outcome results in inconsistent treatment of FCA. Methods: Children with arterial ischemic stroke due to FCA between January 1, 2009, and January 1, 2019, were retrospectively identified at our institution which serves the US Pacific Northwest region. Electronic health record data, including neuroimaging studies, were reviewed, and the Pediatric Stroke Outcome Measure at 1 year was determined as the primary clinical end point. Results: Fifteen children were diagnosed with FCA, accounting for 19% of children with cerebral arteriopathies (n=77). Among children with FCA, the median age at the time of stroke was 6.8 years (Q1–Q3, 1.9–14.0 years). Four (20%) patients had worsening stroke, 3 of whom had concurrent infection. Three (20%) FCA cases were treated with steroids, one of whom had worsening stroke. Median Pediatric Stroke Outcome Measure at 1 year was 1.0 (Q1–Q3, 0.6–2.0). Variability in arteriopathy severity was observed within many patients. Patients with more severe arteriopathy using the Focal Cerebral Arteriopathy Severity Score had larger strokes and were more likely to have worsening stroke. The most common long-term neurological deficit was hemiparesis, which was present in 11 (73%) patients and associated with middle cerebral artery arteriopathy and infarcts. Conclusions: FCA may be less common than previously reported. Neuroimaging in FCA can help identify patients at greater risk for worsening stroke.

2018 ◽  
Vol 13 (8) ◽  
pp. 820-823 ◽  
Author(s):  
Christine K Fox ◽  
Lori C Jordan ◽  
Lauren A Beslow ◽  
Jennifer Armstrong ◽  
Mark T Mackay ◽  
...  

Background Epilepsy is a common complication of pediatric stroke. Aim In this study, we aim to measure the association between epilepsy and neurologic outcome after childhood arterial ischemic stroke. Methods Prospective cohort study of children (29 days–19 years) enrolled after an acute arterial ischemic stroke at 21 international pediatric stroke centers and followed to identify epilepsy. One year post-stroke, outcomes were scored using the examination-based Pediatric Stroke Outcome Measure (range = 0–10); higher values reflect greater disability. Ordinal logistic regression was used to measure the association of Pediatric Stroke Outcome Measure scores (categorized as 0–1, 1.5–3, 3.5–6, 6.5–10) with epilepsy. Results Investigators enrolled 86 children (median age = 6.1 years, interquartile range (IQR) = 1.4–12.2 years) with acute stroke. At 1 year, 18/80 (23%) remained on an anticonvulsant including 8/80 (10%) with epilepsy. Among the 70 with Pediatric Stroke Outcome Measure scored, the median was 0.5 (IQR = 0–1.5) for children without epilepsy ( n = 63), and 6 (IQR = 0.5–10) for children with epilepsy ( n = 7). In univariable analyses, poorer 1-year outcome was associated with middle cerebral artery stroke, cortical infarcts, hemorrhagic transformation, hospital disposition not to home, and epilepsy. In multivariable analysis, middle cerebral artery stroke (odds ratio (OR) = 4.9, 95% confidence intervals (CI) = 1.1–21.3) and epilepsy (OR = 24.1, CI = 1.5–380) remained associated with poorer outcome. Conclusions Children who developed epilepsy during the first year post-stroke had poorer neurologic outcomes than those without epilepsy.


Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
Lori L Billinghurst ◽  
Adam Kirton ◽  
Steven Pavlakis ◽  
Jo Ellen Lee ◽  
Luigi Titomanlio ◽  
...  

Introduction: Headache at stroke onset occurs in up to a quarter of adults and is associated with younger age, female gender, right hemisphere and cerebellar infarcts. Little is known about headache at stroke onset in children. Methods: Children (29 days-18 years) with clinical and radiographic confirmation of arterial ischemic stroke were prospectively enrolled in the International Pediatric Stroke Study from 2003-2014. Details regarding demographics, stroke presentation and infarct location were obtained from the multi-center, pediatric stroke registry. Headache at stroke presentation was classified and annotated in the registry by the individual site investigators as present, absent or unclear. Results: We analyzed 2103 children. Half of all subjects ≥ 6 yo reported headache at stroke onset (N=509/1047, 49%; Figure). Headache was less prevalent in children < 6 yo (N=112/1056, 11%; p<0.001), though headache presentation was more commonly classified as unclear (10% vs 32%; p<0.001). In children ≥ 6 yo, headache was significantly associated with papilledema (p = 0.03) and vertigo (p = 0.01), but not with hemiparesis (p = 0.11), visual field deficit (p = 0.90), aphasia (p = 0.35), dysarthria (p = 0.44), or ataxia (p = 0.50). Headache was more common in posterior than anterior circulation infarcts (p<0.001). There was a significant association between headache and right or bilateral hemisphere infarcts (p = 0.04) but not with gender (p = 0.76). Conclusion: Headache is more prevalent in children than adults at stroke ictus and shares similar associations, including infarcts involving the posterior circulation and right hemisphere. Headache may be under-reported in young infants and children due to pre-verbal stages of development. These findings have implications for early identification and treatment of pediatric stroke and warrant further investigation in prospective studies to distinguish stroke from more common benign mimics, including migraine.


Stroke ◽  
2012 ◽  
Vol 43 (suppl_1) ◽  
Author(s):  
Warren Lo ◽  
Anne Gordon ◽  
Mardee Greenham ◽  
Alison Gomes ◽  
Christine Hajek ◽  
...  

BACKGROUND: The Pediatric Stroke Outcome Measure (PSOM), a 10 point scale based on 5 domains, is one of the few measures of outcome in pediatric stroke. The PSOM has been used to estimate global deficit for studies of stroke outcome and post-stroke quality of life, but its sensitivity to cognitive and functional impairments has received little attention. To address this question, we examined the ability of the PSOM to predict cognitive functioning and behavior after childhood stroke. METHODS: Children ages 6-15 years who had neonatal or childhood onset arterial ischemic stroke were identified by chart review at the Nationwide Children’s Hospital (Columbus, OH, USA) and Royal Children’s Hospital (Melbourne, Australia). Subjects were included if they could complete the test battery. Age- and gender-matched children who had asthma were selected as controls. Stroke cases were assessed with the PSOM and had an MRI brain scan after the acute phase of stroke. Cases and controls underwent functional assessments at least 1 year after the incident stroke. The battery of tests and ratings included the Child Behavior Checklist, Adaptive Behavior Assessment System, Child and Adolescent Scales of Participation, the Wechsler Abbreviated Scales of Intelligence (WASI), and the WISC-4 Processing Speed Index. Infarct volumes were measured by manual segmentation. RESULTS: The sample included 36 children with perinatal or childhood stroke, and 15 controls. Median age at assessment was 8.7 yrs (IQR 6.9-11.9) and median time after presentation was 5.1 yrs (IQR 2.8-7.4). The total PSOM was significantly and inversely related to all measures of cognitive functioning (p range for WISC and WASI 0.001-0.048), multiple measures of adaptive behavior (p range for ABAS scales 0.000-0.031), and all measures of social participation (p range for CASP scales 0.000-0.007). The cognitive/behavioral (C/B) scale of the PSOM was significantly and inversely related to all measures of social participation (p values for CASP 0.000) and multiple measures of adaptive behavior (p range for ABAS scales 0.001-0.057), and positively related to multiple measures of behavior problems (p range for CBCL scales 0.001-0.037). Conclusions: The total PSOM can predict multiple areas of intellectual impairment, and also predicts impairment in adaptive behavior and social participation. In addition the C/B scale can predict behavior problems. The C/B scale depends upon parental report and the clinician's impression, so it is noteworthy that abnormalities on this four-point scale can suggest impairments in a broad range of domains. In addition to a quantitative measure of stroke outcome, the PSOM can serve as a useful screening tool for the identification of intellectual or functional deficits that warrant further investigation for appropriate intervention.


Author(s):  
Maria Gladkikh ◽  
Hugh J. McMillan ◽  
Andrea Andrade ◽  
Cyrus Boelman ◽  
Ishvinder Bhathal ◽  
...  

ABSTRACT: Background: Childhood acute arterial ischemic stroke (AIS) is diagnosed at a median of 23 hours post-symptom onset, delaying treatment. Pediatric stroke pathways can expedite diagnosis. Our goal was to understand the similarities and differences between Canadian pediatric stroke protocols with the aim of optimizing AIS management. Methods: We contacted neurologists at all 16 Canadian pediatric hospitals regarding AIS management. Established protocols were analyzed for similarities and differences in eight domains. Results: Response rate was 100%. Seven (44%) centers have an established AIS protocol and two (13%) have a protocol under development. Seven centers do not have a protocol; two redirect patients to adult neurology, five rely on a case-by-case approach for management. Analysis of the seven protocols revealed differences in: 1) IV-tPA dosage: age-dependent 0.75–0.9 mg/kg (N = 1) versus age-independent 0.9 mg/kg (N = 6), with maximum doses of 75 mg (N = 1) or 90 mg (N = 6); 2) IV-tPA lower age cut-off: 2 years (N = 5) versus 3 or 10 years (each N = 1); 3) IV-tPA exclusion criteria: PedNIHSS score <4 (N = 3), <5 (N = 1), <6 (N = 3); 4) first choice of pre-treatment neuroimaging: computed tomography (CT) (N = 3), magnetic resonance imaging (MRI) (N = 2) or either (N = 2); 5) intra-arterial tPA use (N = 3) and; 6) mechanical thrombectomy timeframe: <6 hour (N = 3), <24 hour (N = 2), unspecified (N = 2). Conclusions: Although 44% of Canadian pediatric hospitals have established AIS management pathways, several differences remain among centers. Some criteria (dosage, imaging) reflect adult AIS literature. Canadian expert consensus regarding IV-tPA and endovascular treatment should be established to standardize and implement AIS protocols across Canada.


Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
Adam de Havenon ◽  
Nabeel Chauhan ◽  
Jennifer Majersik ◽  
David Tirschwell ◽  
Ka-Ho Wong ◽  
...  

Introduction: Enhancing intracranial atherosclerotic plaque on high-resolution vessel wall MRI (vwMRI) is a reliable marker of recent thromboembolism, and confers a recurrent stroke risk of up to 30% a year. Post-contrast plaque enhancement (PPE) on vwMRI is thought to represent inflammation, but studies have not fully examined the clinical, serologic or radiologic factors that contribute to PPE. Methods: Inpatients with acute ischemic stroke due to intracranial atherosclerosis were prospectively enrolled at a single center from 2015-16. vwMRI was performed on a 3T Siemens Verio and included 3D DANTE pulse sequences, pre- and post-contrast (for PPE identification). Three experienced neuroradiologists interpreted vwMRI using a validated multicontrast technique. The Chi-squared, Fisher’s Exact, and Student’s t-test were used for intergroup differences, and logistic regression was fitted to the primary outcome of PPE. Results: Inclusion criteria were met by 35 patients. Atherosclerotic plaques were in the anterior circulation in 21/35 (60%) and PPE was diagnosed in 20/35 (57%) of stroke parent arteries. PPE predictors are shown in Table 1 with logistic regression in Table 2 . Conclusion: PPE is associated with stenosis, which was expected, but the association with HgbA1c is novel. All patients with HgbA1c >8 had PPE and a one point HgbA1c rise increased the odds of PPE 3-fold. Hyperglycemia induces vascular oxidative stress by generating reactive oxygen species, quenching nitric oxide, and triggering an inflammatory cascade. Given the high rate of stroke recurrence in PPE patients, aggressive HgbA1c reduction may be a viable treatment target and warrants additional study.


Stroke ◽  
2020 ◽  
Vol 51 (Suppl_1) ◽  
Author(s):  
Ishvinder Bhathal ◽  
Tazeen Qureshi ◽  
Mahendranath Moharir ◽  
Daune MacGregor ◽  
Elizabeth Pulcine ◽  
...  

Background: Existing literature reports an association between recreational drug use and arterial ischemic stroke (AIS) in adults. Due to recent trends in legalization and concerns regarding the impact of drugs on the developing brain, there is an urgent need for increased awareness of recreational drug use as a risk factor for AIS in childhood. Purpose: To increase awareness of an association between AIS and recreational drug use in a pediatric cohort. Methods: We conducted a retrospective chart review of a consecutive cohort of patients at a tertiary care pediatric center diagnosed with AIS in the context of recreational drug use between 2008-2017. Drug use was confirmed using toxicology testing and clinical history. Demographic, clinical and radiological data were collected. Pediatric Stroke Outcome Measure scores (PSOM) were obtained from an institutional Stroke Registry. Results: Three males and one female were included in the study. Mean age at stroke presentation was 16.3 years (range 16-17 years). Three children presented with focal neurologic deficit and one with new onset seizure. Drug use for each patient was described as follows: Patient A - Marijuana; Patient B - Oxybutynin, Fluoxetine and unidentified compound; Patient C - Marijuana and Amphetamine; Patient D - Marijuana and alcohol. MRI demonstrated diffusion restriction in the anterior circulation in two children, anterior and posterior circulation in one child, and bilateral posterior circulation in one child also found to have a remote AIS. Vascular findings included: Patient A - normal; Patient B and C - right anterior circulation arteriopathy; Patient D - posterior circulation arteriopathy and bilateral vessel wall enhancement. ECHO and pro-thrombotic results were non-contributory. However, one patient required PFO closure. PSOM scores indicated mild-moderate disability initially and moderate disability at follow-up for three of four patients. Conclusions: This case series describes an association between recreational drug use and AIS in adolescents. We are unable to comment on the incidence of AIS related to drug use from our cohort. However, our data highlights a need for public health strategies that acknowledge AIS as a potential consequence of recreational drug use in adolescents.


Stroke ◽  
2020 ◽  
Vol 51 (5) ◽  
pp. 1596-1599
Author(s):  
Mary Clare McKenna ◽  
Noel Fanning ◽  
Simon Cronin

Background and Purpose— Focal cerebral arteriopathy is monophasic inflammatory stenosis of the distal internal carotid artery or the proximal segment of the middle cerebral artery. It is one of the most common causes of acute arterial ischemic stroke in young children but is a less familiar entity for adult neurologists. Methods— We retrospectively reviewed stroke service radiology records at a tertiary referral center from January 2013 to December 2014. Focal cerebral arteriopathy was defined as nonprogressive unifocal and unilateral stenosis/irregularity of the distal internal carotid artery or its proximal branches. Only patients aged 16 to 55 years with stroke were included. Results— There were 5 cases of focal cerebral arteriopathy: 2 males and 3 females. Three cases were from the cohort of 123 acute presentations of young stroke, and 2 cases were outpatient referrals. The mean age (range) was 43 (32–55) years. The majority presented with recurrent transient ischemic attacks/minor strokes within a single vascular territory over days to weeks. All cases had characteristic radiological features. Interval imaging demonstrated resolution in 1 case and improvement in 3 cases. Functional outcome was excellent with discharge modified Rankin Scale score ranging from 0 to 1. Recurrence occurred in 1 case. Conclusions— Focal cerebral arteriopathy is a rare cause of arterial ischemic stroke in young adults. Follow-up intracranial imaging is essential to differentiate from progressive arteriopathies. Evidence-based treatment warrants further investigation. Prognosis is favorable.


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