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Pediatric neurology

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https://www.readbyqxmd.com/read/29145335/clavicle-fracture-associated-with-atlantoaxial-rotatory-displacement-type-ii-in-an-8-year-old-girl-a-case-report
#1
Jacek Karski, Łukasz Matuszewski, Paweł Jakubowski, Klaudia Karska, Grzegorz Kandzierski
RATIONALE: Fracture of the clavicle is a very common injury in children. However, association between clavicle fracture and atlantoaxial rotatory displacement is rarely observed. PATIENT CONCERNS: We present a case of an 8-year-old girl, who suffered a right clavicle fracture as a result of a sledge accident. Six weeks after figure of 8 casting for a right clavicle fracture, an 8-year-old girl was brought to the Pediatric Orthopedic Department due to torticollis...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29143272/preliminary-results-of-immune-modulating-antibody-mdv9300-pidilizumab-treatment-in-children-with-diffuse-intrinsic-pontine-glioma
#2
Iris Fried, Alex Lossos, Tal Ben Ami, Rina Dvir, Helen Toledano, Myriam Weil Ben Arush, Sergei Postovski, Abed Abu Kuidar, Michal Yalon, Michael Weintraub, Mony Benifla
Diffuse intrinsic pontine glioma (DIPG) is an incurable disease with a median overall survival of 10 months. Immune modulating antibodies have recently emerged as a highly promising treatment modality in multiple cancer types. We present results from the first study to evaluate the immune modulating antibody MDV9300 (pidilizumab) in pediatric patients with DIPG. All patients aged 3 years and older, diagnosed with DIPG between February 2014 and June 2015 in Israel, were offered to participate in the study...
November 15, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29141312/-clinical-manifestation-and-gene-analyses-of-15-patients-with-intellectual-disability-or-developmental-delay-complicated-with-congenital-nystagmus
#3
Z J Gao, Q Jiang, Q Chen, K M Xu, Z Q Liu, X B Chen, X L Chen
Objective: To analyze the clinical and genetic features of 15 cases with intellectual disability or developmental delay (ID/DD) complicated with congenital nystagmus. Method: The clinical characteristics and the results of laboratory tests, images and genetics of 15 patients with ID/DD complicated with congenital nystagmus, confirmed by gene diagnosis in the Department of Neurology, Children's Hospital Affiliated to Capital Institute of Pediatrics from March 2015 to October 2016, were retrospectively analyzed...
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29140931/hemispheric-cerebral-oximetry-monitoring-during-pediatric-seizure-activity-in-a-pediatric-emergency-department
#4
Thomas J Abramo, Shane McKinney, James Moore, Richard Jacobs, Gregory Albert, Mark Meredith, Nicholas Hobart Porter, Elizabeth Storm, Errin Willis, Cruz Velasco Gonzalez, Hailey Hargrave, Brad Schneider, Gergory Sharp
BACKGROUND: Sustained neuronal activity during seizures causes cellular perturbations, alterations in cerebral physiology, and potentially neurological injury, a neurological emergency. With variable clinical manifestations of seizures, frequent failure of seizure recognition by providers in pediatric and developmentally challenged patients can increase seizure complications. Neuroresuscitation should include rapid cerebral physiology assessment for increased seizure recognition and optimal neurological outcomes...
November 15, 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/29140276/clinical-profile-associated-with-adverse-childhood-experiences-the-advent-of-nervous-system-dysregulation
#5
Jorina Elbers, Cynthia R Rovnaghi, Brenda Golianu, Kanwaljeet J S Anand
BACKGROUND: We report the prevalence of children with multiple medical symptoms in a pediatric neurology clinic, describe their symptom profiles, and explore their association with adverse childhood experiences (ACEs). METHODS: We retrospectively reviewed 100 consecutive patients from an outpatient pediatric neurology clinic. Patients were included if they were ≥5 years old and reported ≥4 symptoms that were unexplained for ≥3-months. Symptom profiles across six functional domains were recorded: (1) executive dysfunction, (2) sleep disturbances, (3) autonomic dysregulation, (4) somatization, (5) digestive symptoms, and (6) emotional dysregulation...
November 15, 2017: Children
https://www.readbyqxmd.com/read/29138020/moyamoya-syndrome-in-a-pediatric-patient-with-congenital-human-immunodeficiency-virus-type-1-infection-resulting-in-intracranial-hemorrhage
#6
Junko Yamanaka, Ikuma Nozaki, Mizue Tanaka, Hideko Uryuu, Noriko Sato, Takeji Matsushita, Hiroyuki Shichino
In the era of Antiretroviral Therapy (ART) in which human immunodeficiency virus type 1 (HIV-1) infection affected children can expect a better prognosis, the importance of careful follow up of pediatric HIV-1 cases for neurological complications has been growing. We present a case of hemorrhagic Moyamoya syndrome in a child with congenital HIV-1 infection. A 10-year-old girl was referred to our hospital for the treatment of Pneumocystis Jirovecii Pneumonia (PCP: Pneumocystis pneumonia). Her HIV-1 control was poor and Moyamoya syndrome was found during the opportunistic infection screening at admission...
November 11, 2017: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
https://www.readbyqxmd.com/read/29135901/clinical-factors-associated-with-pediatric-brain-neoplasms-versus-primary-headache-a-case-control-analysis
#7
David C Sheridan, Bethany Waites, Bradley Lezak, Robert J Coryell, Kellie J Nazemi, Amber L Lin, Rongwei Fu, Matthew L Hansen
OBJECTIVE: Pediatric headaches are a common presentation to emergency departments accounting for almost half a million annual visits. Providers are left with the difficult task of deciding who has a secondary headache etiology that warrants neuroimaging. METHODS: We conducted a retrospective case-control study. Patients from a pediatric neuro-oncology clinic database with brain cancer and a headache at presentation were identified as cases. Controls were patients from 2 local pediatric tertiary care emergency departments with a final diagnosis of headache after negative neuroimaging...
November 14, 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/29132771/-neonatal-herpes-epidemiology-clinical-manifestations-and-management-guidelines-for-clinical-practice-from-the-french-college-of-gynecologists-and-obstetricians-cngof
#8
L Renesme
OBJECTIVES: To describe the epidemiology of neonatal herpes and its risk factors, clinical and paraclinic manifestations, propose guidelines for a newborn at risk of neonatal herpes, describe treatment modalities, describe post-natal transmission and its prevention. METHODS: Bibliographic search from Medline, Cochrane Library databases and research of international clinical practice guidelines. RESULTS: Neonatal herpes is rare (about 20 cases per year in France) and mainly due to HSV 1 (level of evidence LE3)...
November 10, 2017: Gynecologie, Obstetrique, Fertilite & Senologie
https://www.readbyqxmd.com/read/29131920/congenital-hyperinsulinism
#9
Elena Minakova, Alison Chu
Congenital hyperinsulinism is a rare disorder that commonly presents in the immediate postnatal period as persistent hypoglycemia. The condition is frequently resistant to medical therapies, and the genetic mutations implicated in the disorder can be predictive of response to therapy. Evaluation of hypoglycemia in the illustrative case presented in this article led to genetic testing identifying recessive mutations in the potassium channel subunits of the beta-islet pancreatic cells. Potassium channel defects are often refractory to medical therapies, so near-total pancreatectomy is usually indicated; however, genetic mutations leading to metabolic dysregulation within the beta-islet pancreatic cells are usually responsive to medical therapy...
November 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/29128319/a-proteomic-investigation-into-mechanisms-underpinning-corticosteroid-effects-on-neural-stem-cells
#10
Rawaa S Al-Mayyahi, Luke D Sterio, Joanne B Connolly, Christopher F Adams, Wa'il A Al-Tumah, Jon Sen, Richard D Emes, Sarah R Hart, Divya M Chari
Corticosteroids (CSs) are widely used clinically, for example in pediatric respiratory distress syndrome, and immunosuppression to prevent rejection of stem cell transplant populations in neural cell therapy. However, such treatment can be associated with adverse effects such as impaired neurogenesis and myelination, and increased risk of cerebral palsy. There is increasing evidence that CSs can adversely influence key biological properties of neural stem cells (NSCs) but the molecular mechanisms underpinning such effects are largely unknown...
November 8, 2017: Molecular and Cellular Neurosciences
https://www.readbyqxmd.com/read/29127224/clinical-prediction-rule-for-neurological-sequelae-due-to-acute-encephalopathy-a-medical-community-based-validation-study-in-harima-japan
#11
Kaori Sasaki, Hiroaki Nagase, Azusa Maruyama, Kyoko Fujita, Masahiro Nishiyama, Tsukasa Tanaka, Sadayuki Nukina, Toru Takumi, Kanae Takenaka, Yoshinobu Oyazato, Atsushi Nishiyama, Tomoko Kawata, Keisuke Saeki, Yuichi Takami, Eriko Satake-Inoue, Kazumoto Iijima, Ichiro Morioka, Yoshiyuki Uetani
OBJECTIVES: This study aimed to verify the screening performance of our clinical prediction rule for neurological sequelae due to acute encephalopathy (NSAE-CPR), which previously identified the following three variables as predictive of poor outcomes: (1) refractory status epilepticus; (2) consciousness disturbance and/or hemiplegia at 6 hours from onset and (3) aspartate aminotransferase >90 IU/L within 6 hours of onset. DESIGN: Medical community-based multicentre retrospective cohort study...
November 9, 2017: BMJ Open
https://www.readbyqxmd.com/read/29126579/chest-pain-as-a-manifestation-of-hypokalemia-in-a-pediatric-patient
#12
Jonathan S Schiffman
Patients presenting to the emergency department with chest pain are common and a cause of significant concern to patients and families and physicians alike. The causes of chest pain are myriad. These causes span diverse categories including cardiovascular, respiratory, abdominal and gastrointestinal, musculoskeletal, psychiatric, hematologic and oncologic, and neurologic Thull-Freedman (2010) [1]. These diverse etiologies present a diagnostic and management challenge to the ER physician who is tasked to minimize unnecessary diagnostics while not missing any significant disease...
November 7, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29125443/neurosurgical-management-in-children-with-bleeding-diathesis-auditing-neurological-outcome
#13
Zaitun Zakaria, Chandrasekaran Kaliaperumal, Darach Crimmins, John Caird
OBJECTIVE The aim of this study was to assess the outcome of neurosurgical treatment in children with bleeding diathesis and also to evaluate the current management plan applied in the authors' service. METHODS The authors retrospectively analyzed all cases in which neurosurgical procedures were performed in pediatric patients presenting with intracranial hematoma due to an underlying bleeding tendency over a 5-year period at their institution. They evaluated the patients' neurological symptoms from the initial referral, hematological abnormalities, surgical treatment, neurological outcome, and scores on the Pediatric Glasgow Outcome Scale-Extended (GOS-E Peds) obtained 1 year after the last operation...
November 10, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29125442/long-term-outcomes-after-surgical-treatment-of-pediatric-neurogenic-thoracic-outlet-syndrome
#14
Jennifer Hong, Jared M Pisapia, Zarina S Ali, Austin J Heuer, Erin Alexander, Gregory G Heuer, Eric L Zager
OBJECTIVE Neurogenic thoracic outlet syndrome (nTOS) is an uncommon compression syndrome of the brachial plexus that presents with pain, sensory changes, and motor weakness in the affected limb. The authors reviewed the clinical presentations and outcomes in their series of pediatric patients with surgically treated nTOS over a 6-year period. METHODS Cases of nTOS in patients age 18 years or younger were extracted for analysis from a prospective database of peripheral nerve operations. Baseline patient characteristics, imaging and neurophysiological data, operative findings, and outcomes and complications were assessed...
November 10, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29124391/implementing-a-digital-real-time-hydrocephalus-and-shunt-registry-to-evaluate-contemporary-pattern-of-care-and-surgical-outcome-in-pediatric-hydrocephalus
#15
Hans Christoph Bock, Maximilian Kanzler, Ulrich-Wilhelm Thomale, Hans Christoph Ludwig
OBJECTIVE: Treatment monitoring and outcome evaluation in pediatric hydrocephalus require gapless documentation regarding surgical and clinical follow-up data beginning from day 1 of treatment in order to apply high quality of care. Endoscopic procedures, shunt insertion and revision surgeries, and individual modifications of valve hardware or pressure settings during follow-up as well as established outcome measurements are highly relevant for complete illustration of the patient's hydrocephalus histories...
November 9, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29119462/syncope-in-pediatric-practice
#16
REVIEW
Pratibha Singhi, Arushi Gahlot Saini
Syncope is defined as a sudden, self-limited loss of consciousness and postural tone followed by spontaneous and complete recovery without any neurological sequelae. It is one of the most common paroxysmal disorders in children and adolescents. The three major causes of syncope in children are neural, cardiovascular and other non-cardiovascular causes. The common unifying mechanism is transient global hypoperfusion of the brain. The diagnosis is primarily clinical and objective laboratory investigations add little to the diagnosis in children especially in neurocardiogenic subtype...
November 9, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/29118737/a-case-series-of-adult-onset-rasmussen-s-encephalitis-diagnostic-and-therapeutic-challenges
#17
James Francis Castellano, Jenny A Meyer, Fred Alexander Lado
Rasmussen's encephalitis (RE) is a rare neurologic disorder characterized by progressive cerebral hemiatrophy and medically refractory epilepsy. The majority of current literature on this topic is focused on the pediatric population. In this case series, we will review three cases of adult-onset RE, as defined by fulfillment of the 2005 Bien criteria. The diagnostic challenge of characterizing this rare disease will be highlighted by the extensive serum, CSF, and pathologic sampling in all three patients. MR imaging and EEG data will be examined over time to characterize hallmark findings as well as progression...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/29116594/elevated-expression-of-ebv-and-tlrs-in-the-brain-is-associated-with-rasmussen-s-encephalitis
#18
Xin Wang, Yisong Wang, Dong Liu, Peigang Wang, Dongying Fan, Yuguang Guan, Tianfu Li, Guoming Luan, Jing An
Rasmussen's encephalitis (RE) is a rare pediatric neurological disorder, the etiology of which remains unclear. It has been speculated that the immunopathogenesis of RE involves damage to neurons, which eventually leads to the occurrence of RE. Viral infection may be a critical factor in triggering RE immunopathogenesis. In this study, we analyzed the expression of Epstein-Barr virus (EBV) antigens as well as of Toll-like receptor 3 (TLR3), TLR9, and downstream adapter TIRdomain-containing adapter-inducing interferon-β (TRIF) in the brain tissues of 26 patients with RE and 16 control individuals using immunohistochemistry (IHC)...
October 2017: Virologica Sinica
https://www.readbyqxmd.com/read/29108846/predictors-of-pediatric-blunt-cerebrovascular-injury
#19
Avery C Rossidis, Sasha J Tharakan, Sourav K Bose, Karuna V Shekdar, Michael L Nance, Thane A Blinman
BACKGROUND/PURPOSE: Blunt cerebrovascular injury (BCVI) is clinically challenging because these injuries are hard to detect and can have serious neurological consequences, and optimal screening criteria have not been established for children. This study aims to determine risk factors for BCVI in pediatric patients and to evaluate screening practices in a single institutional series. METHODS: A retrospective review of all pediatric blunt trauma patients evaluated over a 10-year period was performed...
October 12, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29106876/early-signs-and-symptoms-of-intracranial-complications-of-otitis-media-in-pediatric-and-adult-patients-a-different-presentation
#20
N A Van der Poel, E van Spronsen, D A Dietz de Loos, F A Ebbens
OBJECTIVES: The aim of this study was to review the clinical presentation and early signs and symptoms of otogenic intracranial complications (OIC) in children and adults. METHODS: retrospective chart review. The medical records of all children and adults admitted in our center with OIC during the period 2008-2017 were reviewed. Data concerning clinical presentation, treatment and outcomes were reviewed and analyzed. RESULTS: We included 47 patients with OIC: 21 children (range 1-13 years) and 26 adults (range 22-71 years)...
November 2017: International Journal of Pediatric Otorhinolaryngology
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