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Pediatric neurology 15건

  1. [해외논문]   Short Takes  

    Pavlakis, Steven G.
    Pediatric neurology v.84 ,pp. 1 - 2 , 2018 , 0887-8994 ,

    초록

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    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

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  2. [해외논문]   Neuroplasticity: The Other Side of the Coin  

    Pal, Ria (Corresponding author. (J. Elbers)) , Elbers, Jorina
    Pediatric neurology v.84 ,pp. 3 - 4 , 2018 , 0887-8994 ,

    초록

    원문보기

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    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

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  3. [해외논문]   Xenon Combined With Hypothermia in Perinatal Hypoxic-Ischemic Encephalopathy: A Noble Gas, a Noble Mission  

    Amer, Ashraf R. (Corresponding author. (D.E. Oorschot)) , Oorschot, Dorothy E.
    Pediatric neurology v.84 ,pp. 5 - 10 , 2018 , 0887-8994 ,

    초록

    Abstract Perinatal hypoxia-ischemia is a major cause of neonatal morbidity. It generates primary neuronal damage of the neonatal brain and later secondary damage when reperfusion of the ischemic brain tissue causes a surge of oxygen free radicals and inflammation. This post–hypoxic-ischemic brain damage is a leading cause of motor and intellectual disabilities in survivors. Research worldwide has focused on mitigating this injury. Mild or moderate hypothermia is the standard treatment in many centers. However, its benefit is modest and the search for combinatorial effective neuroprotectants continues. This review focuses on xenon as one such agent. The use of mild to moderate hypothermia is reviewed first. Then promising results on the use of xenon to potentiate the effect of hypothermia in in vitro and in vivo animal experiments are discussed. In the first feasibility study on human neonates, researchers found a significant benefit of using 50% xenon for 18 hours in addition to 72 hours of hypothermia. Yet, this additional benefit of xenon was lacking in a larger cohort study, potentially because xenon was used beyond six hours of birth. The future of using xenon is promising, but further clinical studies are awaited to confirm the feasibility of its routine use and its optimal timing, concentration, and duration, for human neonatal hypoxia-ischemia.

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    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

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  4. [해외논문]   A Multidisciplinary Consensus for Clinical Care and Research Needs for Sturge-Weber Syndrome  

    De la Torre, Alejandro J. (Corresponding author. (J.A. Loeb)) , Luat, Aimee F. , Juhá , sz, Csaba , Ho, Mai Lan , Argersinger, Davis P. , Cavuoto, Kara M. , Enriquez-Algeciras, Mabel , Tikkanen, Stephanie , North, Paula , Burkhart, Craig N. , Chugani, Harry T. , Ball, Karen L. , Pinto, Anna Lecticia , Loeb, Jeffrey A.
    Pediatric neurology v.84 ,pp. 11 - 20 , 2018 , 0887-8994 ,

    초록

    Abstract Background Sturge-Weber syndrome is a neurocutaneous disorder associated with port-wine birthmark, leptomeningeal capillary malformations, and glaucoma. It is associated with an unpredictable clinical course. Because of its rarity and complexity, many physicians are unaware of the disease and its complications. A major focus moving ahead will be to turn knowledge gaps and unmet needs into new research directions. Methods On October 1-3, 2017, the Sturge-Weber Foundation assembled clinicians from the Clinical Care Network with patients from the Patient Engagement Network of the Sturge-Weber Foundation to identify our current state of knowledge, knowledge gaps, and unmet needs. Results One clear unmet need is a need for consensus guidelines on care and surveillance. It was strongly recommended that patients be followed by multidisciplinary clinical teams with life-long follow-up for children and adults to monitor disease progression in the skin, eye, and brain. Standardized neuroimaging modalities at specified time points are needed together with a stronger clinicopathologic understanding. Uniform tissue banking and clinical data acquisition strategies are needed with cross-center, longitudinal studies that will set the stage for new clinical trials. A better understanding of the pathogenic roles of cerebral calcifications and stroke-like symptoms is a clear unmet need with potentially devastating consequences. Conclusions Biomarkers capable of predicting disease progression will be needed to advance new therapeutic strategies. Importantly, how to deal with the emotional and psychological effects of Sturge-Weber syndrome and its impact on quality of life is a clear unmet need.

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    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

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  5. [해외논문]   Health-Related Quality of Life for Patients With Genetically Determined Leukoencephalopathy  

    Mirchi, Amytice (Corresponding author. (G. Bernard)) , Pelletier, Fé , lixe , Tran, Luan T. , Keller, Stephanie , Braverman, Nancy , Tonduti, Davide , Vanderver, Adeline , Pizzino, Amy , Dilenge, Marie-Emmanuelle , Poulin, Chantal , Shevell, Michael , Majnemer, Annette , Sé , bire, Guillaume , Srour, Myriam , Osterman, Bradley , Boucher, René , e-Myriam , Vanasse, Michel , Rossignol, Elsa , Mitchell, John , Venkateswaran, Sunita , Pohl, Daniela , Kauffman, Marcelo , Schiffmann, Raphael , Goizet, Cyril , Moutton, Sebastien , Roncarolo, Federico , Bernard, Geneviè , ve
    Pediatric neurology v.84 ,pp. 21 - 26 , 2018 , 0887-8994 ,

    초록

    Abstract Background We attempted to characterize the health-related quality of life in patients with genetically determined leukoencephalopathies as it relates to the severity of clinical features and the presence or absence of a precise molecular diagnosis. Methods Health-related quality of life was assessed using the Pediatric Quality of Life Inventory model (Pediatric Quality of Life Inventory 4.0 Self- and Proxy-reports) on 59 patients diagnosed with genetically determined leukoencephalopathies. In total, 38 male and 21 female patients ranging from one to 32 years of age (mean nine years), as well as their parents, completed the Pediatric Quality of Life Inventory health-related quality of life measures. In addition, participants completed detailed standardized clinical assessments or questionnaires. The correlation between health-related quality of life results and the severity of the clinical features, as well as the presence or absence of a molecular diagnosis, were analyzed. Results Patients with more severe clinical features showed statistically significant lower total Pediatric Quality of Life Inventory scores. More specifically, lower health-related quality of life was noted in children with sialorrhea, gastrostomy, and dystonia and in children who use a wheelchair. Conclusions Patients with more severe clinical features experience a lower quality of life. Our study further highlights the importance of addressing both physical and psychosocial issues and discussing perception of quality of life with both parents and children. A larger multicenter prospective study will be needed to further define the burden of these diseases and to identify modifiable factors.

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    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

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  6. [해외논문]   Narcolepsy and Hypothalamic Region Tumors: Presentation and Evolution  

    Weil, Alexander G. (Corresponding author. (S. Perreault)) , Muir, Katherine , Hukin, Juliette , Desautels, Alex , Martel, Vé , ronique , Perreault, Sé , bastien
    Pediatric neurology v.84 ,pp. 27 - 31 , 2018 , 0887-8994 ,

    초록

    Abstract Objective Because most cases of brain tumor-associated narcolepsy have been published in the form of case reports or small series, the clinical presentation and evolution have not been well described. We sought to better define the epidemiology, etiology, and outcome of brain tumor-related narcolepsy. Methods We conducted an extensive review of the literature to identify cases of narcolepsy associated with brain tumors. Only cases of brain tumors involving the hypothalamic region including the suprasellar, sellar, and thalamus were included in this study. Results We report a child with possible narcolepsy in a child with a brain tumor. Through our literature review, we identified 25 additional cases of narcolepsy associated with brain tumors affecting the hypothalamic area. Most symptomatic narcolepsy cases were reported in children (70%). Half of the patients (13 of 25, 52%) developed narcolepsy after surgery, whereas 11 patients (44%) were symptomatic at the time of the tumor diagnosis. Ten patients had narcolepsy without cataplexy. Most cases were associated with craniopharyngioma (38%), adenoma (24%), and glioma (14%). Three, including our patient, experienced a complete resolution of symptoms. All patients underwent biopsy and were treated with adjuvant therapy. For patients with persistent symptoms, most (60%) improved following medical management of narcolepsy. Conclusion This study represents the largest compilation of patients with this association. Our study allows us to better understand the etiology and outcome of patients with narcolepsy-related brain tumors.

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    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

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  7. [해외논문]   Quantitative Apparent Diffusion Coefficient Mapping May Predict Seizure Onset in Children With Sturge-Weber Syndrome  

    Pinto, Anna L.R. (Corresponding author. (A.L.R. Pinto)) , Ou, Yangming , Sahin, Mustafa , Grant, P. Ellen
    Pediatric neurology v.84 ,pp. 32 - 38 , 2018 , 0887-8994 ,

    초록

    Abstract Background Sturge-Weber syndrome (SWS) is often accompanied by seizures, stroke-like episodes, hemiparesis, and visual field deficits. This study aimed to identify early pathophysiologic changes that exist before the development of clinical symptoms and to evaluate if the apparent diffusion coefficient (ADC) map is a candidate early biomarker of seizure risk in patients with SWS. Methods This is a prospective cross-sectional study using quantitative ADC analysis to predict onset of epilepsy. Inclusion criteria were presence of the port wine birthmark, brain MRI with abnormal leptomeningeal capillary malformation (LCM) and enlarged deep medullary veins, and absence of seizures or other neurological symptoms. We used our recently developed normative, age-specific ADC atlases to quantitatively identify ADC abnormalities, and correlated presymptomatic ADC abnormalities with risks for seizures. Results We identified eight patients (three girls) with SWS, age range of 40 days to nine months. One patient had predominantly LCM, deep venous anomaly, and normal ADC values. This patient did not develop seizures. The remaining seven patients had large regions of abnormal ADC values, and all developed seizures; one of seven patients had late onset seizures. Conclusions Larger regions of decreased ADC values in the affected hemisphere, quantitatively identified by comparison with age-matched normative ADC atlases, are common in young children with SWS and were associated with later onset of seizures in this small study. Our findings suggest that quantitative ADC maps may identify patients at high risk of seizures in SWS, but larger prospective studies are needed to determine sensitivity and specificity.

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    무료다운로드 유료다운로드

    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

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  8. [해외논문]   Outcomes After Acute Symptomatic Seizures in Children Admitted to a Neonatal Neurocritical Care Service  

    Glass, Hannah C. (Corresponding author. (H.C. Glass)) , Numis, Adam L. , Gano, Dawn , Bali, Varun , Rogers, Elizabeth E.
    Pediatric neurology v.84 ,pp. 39 - 45 , 2018 , 0887-8994 ,

    초록

    Abstract Background Neonatal seizures due to acute brain injury are associated with high rates of death, disability, and epilepsy. Our objective was to examine incidence of and risk factors for epilepsy among survivors of acute symptomatic neonatal seizures who were cared for by a neonatal neurocritical care service. Methods Neonates with acute symptomatic seizures who were admitted to UCSF Benioff Children's Hospital Neuro-Intensive Care Nursery from July 2008 to June 2014 were considered for inclusion. Results A total of 144 children with acute symptomatic seizures met study criteria and 37 (26%) died before age one. Eighty-seven children (85% of eligible survivors) were followed up to one year or longer. Epilepsy was diagnosed in eight children at median age 4.9 (interquartile range 1.7, 6.1) years. The cumulative incidence risk of epilepsy at one year was 2% (95% confidence interval 0.6% to 9%) and at five years was 7% (95% confidence interval 3% to 20%). Cerebral palsy was diagnosed in 21%. Bayley-III cognitive subscale less than 85 was present in 13%. Children with epilepsy were more likely to be preterm, have brain injury, and be discharged home on antiseizure medication, although the results were not significant after adjusted analysis. Conclusions The risk of epilepsy was lower and age at onset was older than in previous reports, which may be related to multiple factors including a neurocritical care approach, treatment of hypoxic-ischemic encephalopathy with hypothermia, high rate of neonatal transition to palliative care, and the exclusion of neonatal onset epilepsies. Continuation of antiseizure medications in infancy did not decrease the risk of epilepsy. Long-term, multicenter studies are needed to understand whether neonatal seizure management can alter the risk of epilepsy.

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    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

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  9. [해외논문]   Effect of Risperidone on the Motor and Functional Disability in Children With Choreoathetoid Cerebral Palsy  

    Kamate, Mahesh (Corresponding author. (M. Kamate)) , Mittal, Nishant , Metgud, Deepa
    Pediatric neurology v.84 ,pp. 46 - 48 , 2018 , 0887-8994 ,

    초록

    Abstract Background Therapeutic options for management of choreoathetoid cerebral palsy, which is a permanent disorder, are limited. Available medications either have significant side effects or are unsuitable for long-term use. Risperidone has shown promise in the management of chorea and has been found to be safe in children less than five years. Methods Children with choreoathetoid cerebral palsy were enrolled after parental consent and given risperidone for six-month period along with standard care. The choreoathetoid movements were assessed using Abnormal Involuntary Movement Scale, the upper-limb functions were assessed using Quality of upper extremity skill tests, and the quality of life using Cerebral palsy-Quality of life. Side effects were monitored clinically, by biochemical tests and electrocardiogram. Results Of 42 children with choreoathetoid cerebral palsy who were screened over a period of one year, 35 subjects meeting the study criteria were enrolled. Thirty children completed six months of risperidone therapy, the remaining five subjects were excluded because of time missed due to intercurrent unrelated illnesses. Data of these 30 children were analyzed as per per-protocol analysis. Their mean age was 6.35 ± 3.17 years. Abnormal movements showed statistically significant decline after risperidone (19.7 vs 14.7, P P P Conclusion Risperidone is a promising drug to manage children with choreoathetoid cerebral palsy and is well tolerated in children.

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    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

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  10. [해외논문]   Diffusion-Weighted Imaging Changes in a Child With Posterior Ischemic Optic Neuropathy  

    Harrar, Dana B. (Corresponding author. (D.B. Harrar)) , Solomon, Jessica , Shah, Ankoor S. , Vaughn, Jennifer , Durbin, Adam D. , Rivkin, Michael J.
    Pediatric neurology v.84 ,pp. 49 - 52 , 2018 , 0887-8994 ,

    초록

    Abstract Background Posterior ischemic optic neuropathy results from ischemia of the retrobulbar aspect of the optic nerve. It presents as acute loss of vision without optic disc swelling. This is rare in children, with only seven cases reported to date. Neuroimaging is frequently used to aid in the diagnosis of acute visual complaints in children; however, none of the cases described to date delineate the neuroimaging findings of this entity in children. Methods We retrospectively reviewed the electronic medical record. Results We describe the MRI findings in a 10-month-old boy with posterior ischemic optic neuropathy after intraophthalmic artery injection of chemotherapy for retinoblastoma. Conclusions As targeted therapies for retinoblastoma and other diseases amenable to intravascular treatment delivery are more frequently used, the risk of grave vision-related side effects increases. Posterior ischemic optic neuropathy should be considered in the differential diagnosis of any child presenting with acute loss of vision. Dedicated imaging of the orbits can elucidate specific findings that may aid in the diagnosis of this entity in children.

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    회원님의 원문열람 권한에 따라 열람이 불가능 할 수 있으며 권한이 없는 경우 해당 사이트의 정책에 따라 회원가입 및 유료구매가 필요할 수 있습니다.이동하는 사이트에서의 모든 정보이용은 NDSL과 무관합니다.

    NDSL에서는 해당 원문을 복사서비스하고 있습니다. 아래의 원문복사신청 또는 장바구니담기를 통하여 원문복사서비스 이용이 가능합니다.

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