Vascularites du système nerveux central Chabrier, S.; Darteyre, S.; Mazzola, L. ...
Archives de pédiatrie : organe officiel de la Société française de pédiatrie,
August 2014, Letnik:
21, Številka:
8
Journal Article
Recenzirano
Les vascularites du système nerveux central sont liées à l’infiltration de la paroi des vaisseaux encéphaliques par des cellules inflammatoires circulantes. Chez l’enfant, elles sont caractérisées ...par leur origine (infectieuse versus (vs) non infectieuse), leur évolutivité (transitoires vs chroniques) et par la taille du vaisseau prioritairement affecté. On distingue aussi les affections confinées au système nerveux central de celles dont l’atteinte cérébro-vasculaire est la manifestation neurologique d’une maladie systémique. Les vascularites des grosses artères cérébrales se présentent par un déficit neurologique focal soudain secondaire à un accident vasculaire, ischémique plus fréquemment qu’hémorragique. Elles sont souvent post-infectieuses et monophasiques. Le diagnostic repose sur les antécédents de l’enfant et l’angiographie cérébrale. Les vascularites des petits vaisseaux, plus rares, se manifestent par des céphalées, une épilepsie, des déficits multifocaux, une détérioration cognitive et des troubles du comportement. Ces symptômes peuvent survenir insidieusement sur quelques semaines à quelques mois. Le tableau clinico-biologique systémique associé aux formes secondaires et la biopsie cérébro-méningée dans les formes primitives permettent de porter le diagnostic étiologique. Le traitement des vascularites secondaires est celui de leur cause. La vascularite primitive du système nerveux central est une maladie diffuse et évolutive justifiant un traitement immunosuppresseur prolongé. À l’inverse, les artériopathies cérébrales post-infectieuses, en particulier post-varicelle, ont une évolution favorable sous monothérapie par aspirine. La prise en charge des symptômes associés, des séquelles et des complications du traitement doit reposer sur une équipe pluridisciplinaire.
Central nervous system vasculitides are defined as the invasion of the vascular wall by blood-borne inflammatory cells. In childhood, they may be classified according to their trigger event (infectious vs. non-infectious), their temporal course (time-limited vs. chronic), and the size of the affected vessel. Diseases apparently confined to the central nervous system are also distinguished from secondary forms, associated with infection or rheumatic or systemic inflammatory disorders. Large-vessel vasculitis, the most frequent form, causes stroke and presents with acute focal deficits. MR, or more seldom contrast angiography is required for the positive diagnosis, while the child's medical history conveys the etiological diagnosis. The clinical manifestations of small-vessel vasculitis include headaches, seizures, focal deficits, cognitive decline, and behavior changes that can occur insidiously over a few weeks or a few months. The diagnosis is based on the associated clinical and biological symptoms in secondary forms and on cerebromeningeal biopsy in primary forms. Secondary forms of vasculitides are treated according to the etiology. The injury of large basal arteries is often observed after infection, especially varicella, and is also called transient focal cerebral arteriopathy (TCA) or post-varicella arteriopathy (PVA). This focal, monophasic, and time-limited entity is highly specific of childhood. There are no arguments in the current literature supporting the hypothesis that an aggressive immunomodulatory treatment would be more effective, in terms of recurrence rate or functional outcome, than aspirin alone. In contrast, the diffuse, prolonged, and aggressive course of the rare primary vasculitis of the central nervous system requires a prolonged immunosuppressive treatment. The management of associated symptoms, treatment-related adverse effects, and sequelae is based on a multidisciplinary approach.
Introduction Perinatal ischemic stroke is one of the most common type of stroke in children and the most important cause of unilateral Cerebral Palsy. The object of the study was to find the factors ...related to lesser functional independence at 3.5 years old, in a population of children with neonatal arterial ischemic stroke. Patients and methods It was a French multicentric cohort study (AVCnn cohort), in a population of term born children with neonatal arterial ischemic stroke. 100 newborns were included between November 2003 and October 2006, in 39 French hospital centers. At 3.5 years old, their functional independence was assessed by the Wee-FIM scale. The Wee-FIM stars were compared to healthy children of same age in general population and with the following factors: cerebral palsy, epilepsy, stroke side and mother studies level. Results 80 children fulfilled the Wee-FIM scale at 3.5 years old. The motor condition at 7 years old was known in 69 children (42 boys and 27 girls): 23 had cerebral palsy and 7 were epileptic. The AVC was in the right hemisphere in 26% of cases and in the left hemisphere in 74% of cases. 70% of the mothers were graduated more than Bachelor Degree. Functional independence was weaker in the AVCnn cohort than in healthy children of same age in general population, except for alimentation. The most affected fields were bladder control, bath and shower transfers, expression and comprehension. Epilepsy seems to be the most pejorative factor on independence, and even more if associated to cerebral palsy. The most impacted fields were then: dressing, toilet use, sphincters control, and transfers. Stroke side and mother studies level were not associated to significant variation of functional independence. Conclusion There is a delay in all functional independence fields in children with neonatal arterial ischemic stroke. Functional independence, assessed by the Wee-FIM scale, seems to be most of all impacted by the presence of epilepsy.
Le virus varicella-zoster (VZV) occupe une place prépondérante parmi les facteurs infectieux à l’origine de vasculopathies cérébrales et d’accidents vasculaires cérébraux (AVC) pédiatriques. Une ...atteinte virale directe de la paroi vasculaire a été démontrée dans de rares observations neuropathologiques ainsi que la présence de marqueurs viraux dans le LCR. Cela témoigne d’un processus infectieux localisé probablement associé à des phénomènes inflammatoires indirects. Cependant, l’utilité d’un bilan biologique (ponction lombaire PL) et d’un traitement antiviral ou anti-inflammatoire reste incertaine au vu de l’évolution souvent monophasique de l’artériopathie cérébrale post-varicelleuse chez l’enfant, expliquant les divergences d’attitude thérapeutique observées. Ce travail présente un état des lieux des modalités pédiatriques de prise en charge diagnostique et thérapeutique des AVC post-varicelleux à partir de l’analyse de 26 observations de la littérature depuis l’année 2000, auxquelles s’ajoutent 3 observations personnelles. L’AVC post-varicelleux est classiquement due à une artériopathie du segment initial de l’artère cérébrale moyenne (ACM) entraînant un infarctus du territoire lenticulo-strié et touche de jeunes enfants immunocompétents. La recherche d’une thrombophilie est en général négative. Une PL a été réalisée dans 17/29 cas. La présence de marqueurs viraux n’a été cherchée que dans 14 cas et ne s’est avérée positive que chez 8 enfants. Un traitement antiviral a été administré dans 11 cas. Sous réserve d’un échantillonnage rétrospectif et de petite taille, les enfants traités n’ont ni une meilleure évolution de la vasculopathie ni un moindre risque des séquelles neurologiques, comparativement à l’évolution spontanée.
Among infectious factors, varicella-zoster virus (VZV) is a leading cause of central nervous system vasculopathy and stroke in childhood. Not only have viral markers been detected in the cerebrospinal fluid of affected patients, but also direct evidence of viral particles in the wall of cerebral arteries has been demonstrated in rare pathological specimens. This certainly reflects a localized infectious process likely associated with variable indirect inflammatory responses. Yet the usefulness in this setting of a lumbar puncture as well as of subsequent targeted antiviral and/or anti-inflammatory therapies is uncertain. Indeed, in the majority of cases, the so-called post-varicella angiopathy has a monophasic evolution with spontaneous resolution or stabilization, explaining diverging diagnostic and treatment approaches. In this paper, we have addressed this problematic area by reviewing 26 published cases from the year 2000 and three unpublished cases. Post-varicella stroke is typically associated with angiopathy most often involving the initial portion of the middle cerebral artery, causing a basal ganglia stroke. It tends to occur in young immunocompetent children. Thrombophilia work-up is in general negative. Lumbar puncture was performed in 17 out of 29 cases. Viral markers were examined in 14 cases, but were positive in only eight cases. Antiviral therapy was administrated in 11 children. In this small retrospective study, the treated children's vasculopathy did not progress more favorably nor was there a better outcome compared with untreated subjects.
Neonatal arterial ischemic stroke (NAIS) is a rare event that occurs in approximately one in 5000 term or close-to-term infants. Most affected infants will present with seizures. Although a ...well-recognized clinical entity, many questions remain regarding diagnosis, risk factors, treatment, and follow-up modalities. In the absence of a known pathophysiological mechanism and lack of evidence-based guidelines, only supportive care is currently provided. To address these issues, a French national committee set up by the French Neonatal Society (Société française de néonatologie) and the national referral center (Centre national de référence) for arterial ischemic stroke in children drew up guidelines based on an HAS (Haute Autorité de santé HAS; French national authority for health) methodology. The main findings and recommendations established by the study group are: (1) among the risk factors, male sex, primiparity, caesarean section, perinatal hypoxia, and fetal/neonatal infection (mainly bacterial meningitis) seem to be the most frequent. As for guidelines, the study group recommends the following: (1) the transfer of neonates with suspected NAIS to a neonatal intensive care unit with available equipment to establish a reliable diagnosis with MRI imaging and neurophysiological monitoring, preferably by continuous video EEG; (2) acute treatment of suspected infection or other life-threatening processes should be addressed immediately by the primary medical team. Persistent seizures should be treated with a loading dose of phenobarbital 20mg/kg i.v.; (3) MRI of the brain is considered optimal for the diagnosis of NAIS. Diffusion-weighted imaging with apparent diffusion coefficient is considered the most sensitive measure for identifying infarct in the neonatal brain. The location and extent of the lesions are best assessed between 2 and 4 days after the onset of stroke; (4) routine testing for thrombophilia (AT, PC PS deficiency, FV Leiden or FII20210A) or for detecting other biological risk factors such as antiphospholipid antibodies, high FVIII, homocysteinemia, the Lp(a) test, the MTHFR thermolabile variant should not be considered in neonates with NAIS. Testing for FV Leiden can be performed only in case of a documented family history of venous thromboembolic disease. Testing neonates for the presence of antiphospholipid antibodies should be considered only in case of clinical events arguing in favor of antiphospholipid syndrome in the mother; (5) unlike childhood arterial ischemic stroke, NAIS has a low 5-year recurrence rate (approximately 1 %), except in those children with congenital heart disease or multiple genetic thrombophilia. Therefore, initiation of anticoagulation or antithrombotic agents, including heparin products, is not recommended in the newborn without identifiable risk factors; (6) the study group recommends that in case of delayed motor milestones or early handedness, multidisciplinary rehabilitation is recommended as early as possible. Newborns should have physical therapy evaluation and ongoing outpatient follow-up. Given the risk of later-onset cognitive, language, and behavioral disabilities, neuropsychological testing in preschool and at school age is highly recommended.
Introduction and goal Perinatal arterial ischemic stroke (PAIS) affects one child for 4000 births. The few studies about cognitive development specific to PAIS showed that cognitive performances in ...this population do not follow up a normal development (Westmacott et al., 2010; Ricci et al., 2008). Based on new data about relation between motricity and cognition (Smits-Engelsman et Hill, 2012), and on the theory of the embodied cognition, led us to hypothesize that cognitive performances would be correlated to the motor performances in children with PAIS. Patients and methodology We tested 77 7 years old children meeting the criteria of neonatal AIS, with a diagnosis before the 28th day of life relying on cerebral imagery. After excluding children with seizure and bi-hemispheric lesion, 56 children participated to our study. The cognitive evaluation was performed with the Wechsler Intelligence Scale for Children (WISC-4), the motor evaluation relied on testing of gross motor of the upper arm (Box and Block Test) and fine prehension test (“Nine Hole Peg Test”). The localisation of the lesion, the economic level of parents, the gender, sensory impairments and the presence of hemiplegia were collected. We analyzed these results with simple linear regression. Results The main result of our study is the significative correlation ( P < 0.03) between scores of the WISC4 (except for working memory index) and motor results. In contrast we did not find any correlation between the scores of the WISC4 and the presence of hemiplegia or with lesion localization. Discussion Many brain networks develop during the first year through sensorimotor experiences, which contribute to the emergence of knowledge. This concept of development, supported by the approach embodied cognition, can explain the correlations between cognition and motor found in our work and in several studies with children with other early neurological damage.
Anticoagulation is recommended in the acute phase of cerebral venous thrombosis in adults, then for 3-12 months. In children, 2 consensus reports published in 2008 also recommend use of ...anticoagulants, whereas conclusions diverge for newborns. These consensus reports are based on observational studies, authors' experience, and comparisons with adult pathology. In view of the original studies published since then, the French Society of Pediatric Neurology (Société française de neurologie pédiatrique SFNP) wished to update the level of evidence and the knowledge in this domain. The results from the analysis of the literature show that anticoagulation is widely used in pediatrics. It is well-tolerated in children (class I, level of evidence B) and probably in the newborn (class IIa, level of evidence B). In the acute phase of cerebral venous thrombosis, anticoagulation is probably effective in reducing the risk of death in children (class IIa, level of evidence B). It is not possible to draw a conclusion on newborns (class IIb). Over the longer term, anticoagulation is effective in reducing the risk of recurrence (class I, level of evidence B). Since this risk is highly dependent on a number of individual factors (the main ones being the child's age, the cause of the thrombosis, and the kinetics of the sinus recanalization), the duration of anticoagulation should be analyzed individually (class I, level of evidence B). All in all, the convergence of the results, the physiopathologic arguments, and the concordance with the data on adult patients has led to the following recommendations: in the absence of a contra-indication, it is reasonable to propose anticoagulation in the acute phase of cerebral venous thrombosis in children. Prolonging this treatment for 3-6 months is indicated depending on the number of individual factors. In the absence of a contra-indication, anticoagulation may be considered individually in the acute phase of cerebral venous thrombosis in newborns for 6-12 weeks.
Many works in the literature focus on the definition of evaluation metrics and criteria that enable to quantify the performance of an image processing algorithm. These evaluation criteria can be used ...to define new image processing algorithms by optimizing them. In this paper, we propose a general scheme to segment images by a genetic algorithm. The developed method uses an evaluation criterion which quantifies the quality of an image segmentation result. The proposed segmentation method can integrate a local ground truth when it is available in order to set the desired level of precision of the final result. A genetic algorithm is then used in order to determine the best combination of information extracted by the selected criterion. Then, we show that this approach can either be applied for gray-levels or multicomponents images in a supervised context or in an unsupervised one. Last, we show the efficiency of the proposed method through some experimental results on several gray-levels and multicomponents images.
Questions about care practices and the role of palliative care in pediatric neurodegenerative diseases have led the Neuromuscular Committee of the French Society of Neurology to conduct a ...retrospective study in spinal muscular atrophy type 1, a genetic disease most often leading to death before the age of 1 year.
A retrospective multicenter study from pediatricians included in the reference centers of pediatric neuromuscular diseases was carried out on two 10-year periods (1989-1998 and 1999-2009).
The 1989-1998 period included 12 centers with 106 patients, the 1999-2009 period 13 centers with 116 children. The mean age of onset of clinical signs was 2.1 months (range, 0-5.5 months), the median age at diagnosis was 4 months (range, 0-9 months) vs 3 months. The median age of death was 7.5 months (range, 0-24 months) vs 6 months. The care modalities included physiotherapy (90 %), motor support (61 % vs 26 % for the previous period), enteral nutrition by nasogastric tube (52 % vs 24 %), and 3.4 % of children had a gastrostomy (vs 1.8 %). At home, pharyngeal aspiration was used in 64 % (vs 41 %), oxygen therapy in 8 %, noninvasive ventilatory support in 7 %. The mean age at death was 8.1 months (range, 0-24 months) vs 7 months, the time from diagnosis to death was 4 months vs 3 months. Death occurred at home in 23 % vs 17 %, in a pediatric unit in 62 % vs 41 %. The use of analgesics and sedative drugs was reported in 60 % of cases: 40 % morphine (vs 18 %) and benzodiazepines in 48 % (vs 29 %). Respiratory support was limited mostly to oxygen by nasal tube (55 % vs 54 %), noninvasive ventilation in 9 % of the cases, and intubation and assisted mechanical ventilation (2 %).
These results confirm a change in practices and the development of palliative care in children with a French consensus of practices quite different from the standard care in North-America and closer to the thinking of English medical teams. A prospective study within the 2011 national hospital clinical research program (PHRC 2011) is beginning in order to evaluate practices and the role of families and caregivers.
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