Guillaume Sébire

Guillaume Sébire
McGill University | McGill · Department of Pediatrics and Department of Neurology and Neurosurgery

MD, PhD

About

225
Publications
30,833
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Introduction
G Sébire’s research focuses on stroke and infectious/inflammatory aggressions occurring in the developing brain and responsible for severe handicaps, ie cerebral palsy, learning disabilities and behaviourial disorders such as autism. Through a combination of clinical and experimental approaches, Sebire’s team has shown that prenatal exposure to bacterial components induces cerebral lesions, through cytokines'release, and predisposes to circulatory defects which occur immediately after birth.
Additional affiliations
February 2014 - August 2020
McGill University
Position
  • Managing Director
February 2014 - present
McGill University
Position
  • Professor (Full)
February 2012 - January 2014
Université de Sherbrooke
Position
  • Member of Child Neurology Division, Professor

Publications

Publications (225)
Article
Objective To understand the impact of pediatric multiple sclerosis (MS), health-related quality of life (HRQL) measures have been used. However, specific areas of concern of youths with MS are not known. The objective of this study was to contribute content for inclusion in a new condition-specific measure of the life impact of MS for children and...
Article
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Group B Streptococcus (GBS) is one of the most common bacteria isolated in human chorioamnionitis. Placental infection due to GBS is a major risk factor for fetal organ injuries, preterm birth, perinatal morbidity and mortality, and life-long multiorgan morbidities. Preclinical and clinical studies have shown that GBS-induced infection drives polym...
Article
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Group B Streptococcus (GBS) is a leading cause of placental infection, termed chorioamnionitis. Chorioamnionitis is associated with an increased risk of neurobehavioral impairments, such as autism spectrum disorders, which are more prominent in males than in female offspring. In a pre-clinical model of chorioamnionitis, a greater inflammatory respo...
Article
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Preterm birth is defined as any birth occurring before 37 completed weeks of gestation by the World Health Organization. Preterm birth is responsible for perinatal mortality and long-term neurological morbidity. Acute chorioamnionitis is observed in 70% of premature labor and is associated with a heavy burden of multiorgan morbidities in the offspr...
Article
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Group B Streptococcus (GBS) infection during pregnancy is involved in maternal sepsis, chorioamnionitis, prematurity, fetal infection, neonatal sepsis, and neurodevelopmental alterations. The GBS-induced chorioamnionitis leads to a plethora of immune and trophoblast cells alterations that could influence endothelial cells to respond differently to...
Article
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Background: Hirayama Disease (HD) is a rare disorder consisting of insidious onset of unilateral weakness and atrophy of the forearm and intrinsic hand muscles. Vein of Galen aneurysmal malformations (VGAMs) are rare congenital cerebral vascular malformations, consisting of high-flow arteriovenous shunting between a persistent median prosencephalic...
Article
Introduction: The purpose of this report is to present a rare case of Hirayama disease (HD) in a patient with a history of late-onset symptomatic vein of Galen aneurysmal malformation (VGAM). This report may provide new insights into the pathophysiology of HD, a rare disorder consisting of insidious onset of unilateral weakness and atrophy of the...
Article
Background Identifying antepartum versus intrapartum timing and the cause of neonatal encephalopathy (NE) often remains elusive due to our limited understanding of the underlying pathophysiological processes and lack of appropriate biomarkers. Objectives This retrospective observational study describes a case series of term newborns with NE who di...
Article
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Context: 4H or POLR3-related leukodystrophy is an autosomal recessive disorder typically characterized by hypomyelination, hypodontia, and hypogonadotropic hypogonadism, caused by biallelic pathogenic variants in POLR3A, POLR3B, POLR1C, and POLR3K. The endocrine and growth abnormalities associated with this disorder have not been thoroughly investi...
Article
Necroptosis is emerging among possible mechanisms underlying cell death in neurodegenerative diseases. In this line, we hypothesize that necroptosis might be implicated in neuronal cell death in amyotrophic lateral sclerosis (ALS). To support this hypothesis, we hereby provide pilot data as well as some findings from the literature about the expres...
Article
Background Acute flaccid myelitis (AFM) is characterised by rapid onset of limb weakness with spinal cord grey-matter abnormalities on MRI scan. We aimed to assess whether detection of enterovirus in respiratory or other specimens can help predict prognosis in children with AFM. Methods In this nationwide, longitudinal study, we evaluated the sign...
Article
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Abstract Context: 4H or POLR3-related leukodystrophy is an autosomal recessive disorder typically characterized by hypomyelination, hypodontia, and hypogonadotropic hypogonadism, caused by biallelic pathogenic variants in POLR3A, POLR3B, POLR1C and POLR3K. The endocrine and growth abnormalities associated with this disorder have not been thoroughl...
Article
Genetically determined leukoencephalopathies comprise a group of rare inherited white matter disorders. The majority are progressive diseases resulting in early death. We performed a cross-sectional pilot study including 55 parents from 36 families to assess the level of stress experienced by parents of patients with genetically determined leukoenc...
Article
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Objective To test the association between exposure to perinatal inflammation – i.e. clinical chorioamnionitis or early-onset neonatal infection – in preterm children without severe neonatal brain injury and neurodevelopmental outcome at 30 months of corrected age (CA). Design Cross-sectional study from a French regional cohort of clinical follow-u...
Article
Background: Antibiotic therapy during preterm labor with intact membranes has been associated with an increased risk of neonatal death. Objectives: Using an established rat model of group B Streptococcus (GBS)-induced chorioamnionitis, we hypothesized that ampicillin treatment increases placental inflammation, as shown in other bacterial infecti...
Article
Background: There is currently no targeted treatment available for neonatal arterial ischemic strokes (NAIS). Epidemiological studies demonstrated that perinatal infection/inflammation, peripartum hypoxia, and occlusion of the internal carotid tree are the main determinants of NAIS. The well-established benefit of therapeutic hypothermia (HT) in n...
Article
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Introduction Aucun traitement n’est actuellement disponible pour l’infarctus cérébral artériel néonatal (NAIS). Les études épidémiologiques ont montré que l’infection/inflammation périnatale, l’hypoxie perpartum associées à l’occlusion d’une artère cervico-encéphalique sont les principaux déterminants du NAIS. L’effet neuroprotecteur de l’hypotherm...
Article
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CDC42BPB encodes MRCKβ (myotonic dystrophy‐related Cdc42‐binding kinase beta), a serine/threonine protein kinase, and a downstream effector of CDC42, which has recently been associated with Takenouchi‐Kosaki syndrome, an autosomal dominant neurodevelopmental disorder. We identified 12 heterozygous predicted deleterious variants in CDC42BPB (9 misse...
Article
Lissencephaly (LIS), denoting a "smooth brain," is characterized by the absence of normal cerebral convolutions with abnormalities of cortical thickness. Pathogenic variants in over 20 genes are associated with LIS. The majority of posterior predominant LIS is caused by pathogenic variants in LIS1 (also known as PAFAH1B1), although a significant fr...
Article
This commentary is on the original article by Sorg et al. on pages 513–520 of this issue.
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Lateral medullary syndrome is rare in pediatrics. It is characterized by neurological deficits due to an ischemic lesion in the lateral medulla. The authors describe a 17-year-old boy who developed lateral medullary syndrome in the context of a hyperflexion neck injury while diving in shallow water with traumatic vascular injury. He had “crossed” n...
Article
Cerebral palsy (CP), an umbrella term for a developmental motor disorder caused by early brain injury (EBI)/interference, remains debated. In this essay, we present a narrative, beginning with the original anatomical-clinical description of the so-called paralysie congéniale (congenial paralysis) by the French psychiatrist Jean-Baptiste Cazauvieilh...
Article
Background: By participating in parent-mediated interventions, parents develop skills to promote their toddler’s development. Knowing that the potential mediating role of a parent’s behavior has been poorly assessed, our objective was to measure the effects of a parent-mediated intervention on the developmental outcome of toddlers (12–30 months) at...
Article
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Group B Streptococcus (GBS) is one of the most common bacteria isolated in human chorioamnionitis, which is a major risk factor for premature birth and brain injuries. Males are at greater risk than females for developing lifelong neurobehavioural disorders, although the origins of this sex bias remain poorly understood. We previously showed that e...
Article
BACKGROUND: Group B streptococcus (GBS) is the main bacteria that infects pregnant women and can cause abortion and chorioamnionitis. The impact of GBS effects on human trophoblast cells remains largely elusive, and actions toward anti-inflammatory strategies in pregnancy are needed. A potent anti-inflammatory molecule, uvaol is a triterpene from...
Article
Aminoacyl-tRNA synthetase-interacting multifunctional protein 1 (AIMP1) is a non-catalytic component of the multi-tRNA synthetase complex which catalyzes the ligation of amino acids to the correct tRNAs. Pathogenic variants in several aminoacyl-tRNA synthetases genes have been linked to various neurological disorders, including leukodystrophies and...
Article
We aimed to describe families with genetic epilepsy with febrile seizures plus (GEFS+) in which individuals suffered sudden unexpected death. The Epilepsy Pharmacogenomics Research Database was reviewed for GEFS + families in which at least one individual had suffered sudden death, and two families were identified. In Family A, five males had febri...
Article
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Chorioamnionitis and intrauterine growth retardation (IUGR) are risk factors for cerebral palsy (CP). Common bacteria isolated in chorioamnionitis include group B Streptococcus (GBS) serotypes Ia and III. Little is known about the impact of placental inflammation induced by different bacteria, including different GBS strains. We aimed to test the i...
Article
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Background: Hypothermia is increasingly tested in several neurological conditions, such as neonatal encephalopathy, stroke, traumatic brain injury, subarachnoid hemorrhage, spinal cord injury, and neurological outcomes of cardiac arrest. Current studies aim to increase benefits of hypothermia with new add-on therapies including immunomodulatory ag...
Article
Introduction Environ 30% des nouveau-nés sont exposés à une antibioprophylaxie intrapartum. La dernière méta-analyse Cochrane évaluant l’impact de l’antibiothérapie intrapartum dans la menace d’accouchement prématuré sans rupture des membranes a mis en évidence une augmentation significative de la mortalité néonatale associée à ce traitement (RR :...
Article
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Group B Streptococcus (GBS) is one the most common bacterium responsible of maternal infections during pregnancy. Offspring in utero-exposed to GBS-induced placental inflammation displayed sex-specific forebrain injuries. Sex differences have been reported in several neuropsychiatric disorders. Hence, we hypothesized that female rats in utero-expos...
Article
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Background: Limbic encephalitis (LE) is a rare autoimmune syndrome affecting limbic system structures and causing variety of manifestations including memory changes, temporal epilepsies, and psychiatric symptoms. It is a rare disease in children but with a well-recognizable combination of clinical, neuroimaging and/or histological signature. Beyond...
Article
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Background: Wallenberg’s syndrome (WS), or lateral medullary syndrome is rare in pediatrics, but is not uncommon in adults. It is characterized by neurological deficits due to an ischemic lesion in the lateral medulla. Methods: Case report Results: We describe a 17-year-old boy who developed WS in the context of hyperflexion injury to the neck whil...
Article
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p>OBJECTIVES: Cerebrovascular disease is among the top 10 causes of death in US children, but risk factors for mortality are poorly understood. Within an international registry, we identify predictors of in-hospital mortality after pediatric arterial ischemic stroke (AIS). METHODS: Neonates (0-28 days) and children (29 days-<19 years) with AIS were...
Article
AIMS To characterize health-related quality of life (HRQOL) in patients with genetically determined leukoencephalopathies as it relates to the severity of clinical features and the presence/absence of a precise molecular diagnosis. METHOD HRQOL was assessed using the Pediatric Quality of Life Inventory (PedsQL) model (Pediatric Quality of Life Inve...
Article
Full-text available
Background Acute hemorrhagic encephalomyelitis (AHEM) is considered as a rare form of acute disseminated encephalomyelitis characterized by fulminant encephalopathy with hemorrhagic necrosis and most often fatal outcome.Objective To report the association with Ran Binding Protein (RANBP2) gene variant and the response to decompressive craniectomy a...
Article
Background MRI and laboratory features have been incorporated into international diagnostic criteria for multiple sclerosis. We assessed the pattern of MRI lesions and contributions of cerebrospinal fluid (CSF) and serum antibody findings that best identifies children with multiple sclerosis, and the applicability of international diagnostic criter...
Article
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Based on the review of the literature, perinatal inflammation often induced by infection is the only consistent independent risk factor of neonatal arterial ischemic stroke (NAIS). Preclinical studies show that acute inflammatory processes take place in placenta, cerebral arterial wall of NAIS-susceptible arteries and neonatal brain. A top research...
Article
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Nonparaneoplastic opsoclonus–myoclonus ataxia syndrome is a rare neuroinflammatory condition featured by opsoclonus, myoclonus, ataxia, and cognitive behavioral disturbance. The authors report an observation of enterovirus 71-associated opsoclonus–myoclonus ataxia syndrome evolving toward full recovery on intravenous intravenous immunoglobulin (IG)...
Article
Objective The observation of the dramatic response to intravenous immunoglobulin (IVIG) of a child from our center with intractable epilepsy (IE) due to focal cortical dysplasia (FCD) prompted us to perform a meta-analysis on the efficacy of IVIG in this condition. Background FCD is a common cause of IE. Microglial activation and up-regulation of...
Article
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Background: AHEM is a rare form of acute disseminated encephalomyelitis (ADEM) characterized by fulminant encephalopathy with hemorrhagic necrosis and most often fatal outcome. Methods: A case report and review of literature. Results: A 6-year-old girl known SCD presented an acute demyelinating syndrome (ADS) with diplopia due to unilateral fourth...
Article
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The medium-size intra-cranial arteries arising from the carotid bifurcation are prone to perinatal arterial ischemic strokes (PAIS). PAIS' physiopathology needs to be better understood to develop preventive and therapeutic interventions that are currently missing. We hypothesized that materno-fetal inflammation leads to a vasculitis affecting selec...
Article
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See related article, p 2443. Like many disorders, the incidence of arterial ischemic stroke (AIS) throughout life is represented by a U-shaped curve. The peripartum period carries the highest risk, whereas people from 29 days to 18 years have the lowest risk. The incidence then gradually increases from adolescence to old age. Furthermore, the mec...
Article
Background: Despite the recent introduction of hypothermia as a mandatory standard of care, the incidence of neonatal encephalopathy in full-term newborns and its devastating neuro-behavioral outcomes continues to be a major individual, familial and social issue. Neonatal encephalopathy is mainly due to the compounding and interacting effects of h...
Article
Infection or inflammation during pregnancy is known to lead to maternal immune activation triggering a fetal inflammatory response syndrome associated with deleterious effects, such as brain injury and neurodevelopmental disabilities. Group B Streptococcus (GBS) - one of the most common bacterium colonizing pregnant women - can be responsible for c...
Poster
Full-text available
Poster presented at the International Society for Developmental Neuroscience (ISDN) meeting, May 11-14, 2016.
Article
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Group B Streptococcus (GBS) is a commensal bacterium present in the lower genital tract of 15-30% of healthy pregnant women. GBS is the leading cause of chorioamnionitis and cerebral injuries in newborns, occurring most often in the absence of maternofetal pathogen translocation. Despite GBS being the most frequent bacterium colonizing pregnant wom...
Article
Recent studies reported over-expression of a cytokine (Interleukin (IL)-18) in the serum of sporadic amyotrophic lateral sclerosis (sALS) patients. Here, we report on the first-time detection of in-situ expression of activated IL-18 in the human brain in sALS patients. We also detected cerebral in-situ expression of key-molecules known to be closel...
Article
Problem: Prenatal exposure to inflammation increases the incidence of neonatal brain injury. This raise the question whether maternally produced cytokines, especially interleukin (IL)-1 elevated in pathological pregnancies and known to alter fetal development, can cross the placental barrier and affect the fetus directly. Method of study: We add...
Chapter
It is known that gestational and/or perinatal inflammation combined or not with hypoxia-ischemia (HI) is a risk factor for brain injuries, but the mechanisms underlying are still unclear. This chapter discusses about animal models mimicking those conditions, allowing scientists to uncover mechanisms involved and to study the adverse effects on the...
Research
Full-text available
Canadian National Perinatal Research Meeting, february 2015. Poster session