5,336 results match your criteria Pediatric Neurology[Journal]


Tuberous Sclerosis Complex Genotypes and Developmental Phenotype.

Pediatr Neurol 2019 Mar 13. Epub 2019 Mar 13.

Department of Pediatrics, Division of Medical Genetics, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, Texas.

Background: Children with tuberous sclerosis complex (TSC), caused by pathogenic variants in TSC1/TSC2, are at risk for intellectual disability. TSC2 pathogenic variants appear to increase the risk, compared with TSC1. However, the effect of TSC2 pathogenic variants on early and specific domains of development hasn't been studied. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.03.003DOI Listing

White Matter Lesions Detected by Magnetic Resonance Imaging in Neonates and Children With Congenital Myotonic Dystrophy.

Pediatr Neurol 2019 Mar 13. Epub 2019 Mar 13.

Department of Neurology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio; University of Cincinnati, College of Medicine, Cincinnati, Ohio. Electronic address:

Background: Congenital myotonic dystrophy (CDM1) is an autosomal dominant genetic disorder caused by abnormal cytosine-thymine-guanine trinucleotide repeat expansion that results in weakness and cognitive deficits. Studies detailing brain magnetic resonance imaging (MRI) findings in neonates and children with this condition are limited.

Objective: We evaluated the brain MRI findings in children, including neonates with CDM1, to assess the nature of central nervous system involvement and progression of MRI lesions over time. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08878994183114
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http://dx.doi.org/10.1016/j.pediatrneurol.2019.03.004DOI Listing
March 2019
1 Read

"No-No" Head Tremor-A Nod to the Diagnosis.

Pediatr Neurol 2019 Mar 19. Epub 2019 Mar 19.

Department of Neurology and Neurophysiology, Temple Street Children's University Hospital, Dublin, Ireland; Academic Centre on Rare Diseases, School of Medicine and Medical Science, University College Dublin, Ireland.

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https://linkinghub.elsevier.com/retrieve/pii/S08878994193002
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http://dx.doi.org/10.1016/j.pediatrneurol.2019.03.007DOI Listing
March 2019
1 Read

Tolosa-Hunt Syndrome: Clinical Manifestations in Children.

Pediatr Neurol 2019 Feb 20. Epub 2019 Feb 20.

Pediatric Department, University Hospital Of Heraklion, Medical School, University of Crete, Heraklion, Crete, Greece. Electronic address:

Background: Tolosa-Hunt syndrome (THS) is a rare condition in children characterized by painful ophthalmoplegia caused by inflammation of unknown etiology in the cavernous sinus, superior orbital fissure, or orbital apex. Our main purpose was to report two pediatric cases of THS, a typical one and another extremely rare one preceded by facial palsy.

Methods: Both cases were diagnosed with THS based on the 2013 International Classification of Headache Disorders (ICHD-3 beta) criteria. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08878994183072
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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.013DOI Listing
February 2019
5 Reads

Dysmaturation of Premature Brain: Importance, Cellular Mechanisms, and Potential Interventions.

Authors:
Joseph J Volpe

Pediatr Neurol 2019 Feb 27. Epub 2019 Feb 27.

Department of Neurology, Harvard Medical School, Boston, Massachusetts; Department of Pediatric Newborn Medicine, Harvard Medical School, Boston, Massachusetts. Electronic address:

Prematurity, especially preterm birth (less than 32 weeks' gestation), is common and associated with high rates of both survival and neurodevelopmental disability, especially apparent in cognitive spheres. The neuropathological substrate of this disability is now recognized to be related to a variety of dysmaturational disturbances of the brain. These disturbances follow initial brain injury, particularly cerebral white matter injury, and involve many of the extraordinary array of developmental events active in cerebral white and gray matter structures during the premature period. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08878994193010
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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.016DOI Listing
February 2019
2 Reads

Craniocervical Arterial Dissection in Children: Pathophysiology and Management.

Pediatr Neurol 2019 Feb 2. Epub 2019 Feb 2.

Section of Pediatric Neurology, Department of Pediatrics and Child Health, University of Manitoba, Manitoba, Canada; Children's Hospital Research Institute of Manitoba, Winnipeg, Manitoba, Canada. Electronic address:

Background: Craniocervical arterial dissection is a commonly reported arteriopathy associated with stroke in children. It is characterized by a high stroke recurrence rate and variable outcomes. Here we review the pathophysiology, clinical presentation, and diagnostic neuroimaging approaches that are helpful in accurate diagnosis and follow-up of children with arterial dissection. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08878994183130
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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.020DOI Listing
February 2019
3 Reads

Proband-Only Clinical Exome Sequencing for Neurodevelopmental Disabilities.

Pediatr Neurol 2019 Feb 27. Epub 2019 Feb 27.

Division of Pediatric Neurology, Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, Epilepsy Research Institute, Seoul, Korea. Electronic address:

Background: Whole exome sequencing on family trios gives the highest diagnostic yield, but high cost limits its application. Here, we performed proband-only clinical exome sequencing in a population of patients with neurodevelopmental disabilities and tested the diagnostic yield.

Methods: This observational, retrospective study included 108 unrelated patients with neurodevelopmental disabilities who underwent clinical exome sequencing at the outpatient clinics of the Severance Children's Hospital, Seoul, South Korea, between March 2017 and May 2018. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.017DOI Listing
February 2019
2 Reads

Stroke Recurrence in Nigerian Children With Sickle Cell Disease: Evidence for a Secondary Stroke Prevention Trial.

Pediatr Neurol 2019 Jan 17. Epub 2019 Jan 17.

Department of Hematology and Blood Transfusion, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria. Electronic address:

Background: To improve the quality of care for children with sickle cell anemia in Kano, Nigeria, we initiated a standard care protocol in 2014 to manage children with strokes at Aminu Kano Teaching Hospital.

Methods: The standard care protocol requires that children with acute strokes be treated with hydroxyurea at a fixed dose of 20 mg/kg/day within two months of the stroke.

Results: Twenty-nine children with sickle cell anemia and initial stroke were identified based on clinical World Health Organization criteria from 2014 to 2017. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08878994183089
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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.008DOI Listing
January 2019
4 Reads

Sickle Cell Disease and Stroke.

Pediatr Neurol 2019 Feb 27. Epub 2019 Feb 27.

Developmental Neurosciences Section and Biomedical Research Unit, Clinical and Experimental Sciences, University of Southampton, UCL Great Ormond Street Institute of Child Health, London, UK. Electronic address:

Cerebral infarction is a common complication of sickle cell disease and may manifest as overt stroke or cognitive impairment associated with "silent" cerebral infarction on magnetic resonance imaging. Vasculopathy may be diagnosed on transcranial Doppler or magnetic resonance angiography. The risk factors in sickle cell disease for cognitive impairment, overt ischemic stroke, silent cerebral infarction, overt hemorrhagic stroke, and vasculopathy defined by transcranial Doppler or magnetic resonance angiography overlap, with severe acute and chronic anemia, acute chest crisis, reticulocytosis, and low oxygen saturation reported with the majority. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.018DOI Listing
February 2019
2 Reads

Comparative Effectiveness of Phenobarbital versus Levetiracetam for Infantile Epilepsy.

Pediatr Neurol 2019 Mar 1. Epub 2019 Mar 1.

Ann & Robert H. Lurie Children's Hospital of Chicago, Feinberg School of Medicine, Northwestern University, Chicago, Illinois.

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.010DOI Listing
March 2019
1 Read

Cerebrospinal Fluid Neopterin in Children With Enterovirus-Related Brainstem Encephalitis.

Pediatr Neurol 2019 Feb 7. Epub 2019 Feb 7.

Department of Pediatrics, Hospital Sant Joan de Deu (University of Barcelona), Barcelona, Spain; Pediatric Infectious Diseases Research Group, Institut de Recerca Sant Joan de Deu, Barcelona, Spain; CIBER en Epidemiología y Salud Pública (CIBERESP), Madrid, Spain. Electronic address:

Background: Enterovirus-A71 causes outbreaks of brainstem encephalitis, ranging from self-limited disease to acute flaccid paralysis. The aim of this study was to assess the role of cerebrospinal fluid (CSF) neopterin as a biomarker of disease severity in children with enterovirus-related brainstem encephalitis.

Methods: A descriptive, prospective cohort study was conducted from April 2016 to March 2017 in a tertiary hospital. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.024DOI Listing
February 2019
4 Reads

Cyclin-Dependent Kinase-Like 5 Deficiency Disorder: Clinical Review.

Pediatr Neurol 2019 Feb 23. Epub 2019 Feb 23.

Children's Hospital Colorado, University of Colorado, School of Medicine, Aurora, Colorado; Department of Pediatrics, University of Colorado, School of Medicine, Aurora, Colorado; Department of Pharmacology, University of Colorado, School of Medicine, Aurora, Colorado; Department of Neurology, University of Colorado, School of Medicine, Aurora, Colorado; Department of Otolaryngology, University of Colorado, School of Medicine, Aurora, Colorado.

Cyclin-dependent kinase-like 5 (CDKL5) deficiency disorder (CDD) is a developmental encephalopathy caused by pathogenic variants in the gene CDKL5. This unique disorder includes early infantile onset refractory epilepsy, hypotonia, developmental intellectual and motor disabilities, and cortical visual impairment. We review the clinical presentations and genetic variations in CDD based on a systematic literature review and experience in the CDKL5 Centers of Excellence. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.015DOI Listing
February 2019
1 Read

Physical Growth of the Contralateral Cerebrum is Preserved After Hemispherotomy in Childhood.

Pediatr Neurol 2019 Feb 13. Epub 2019 Feb 13.

Department of Radiology, UCLA Medical Center, David Geffen School of Medicine at UCLA, Los Angeles, California. Electronic address:

Background: Hemispherotomy can be an effective treatment for refractory childhood epilepsy. However, the extent of postoperative brain development after hemispherotomy remains incompletely understood. This study aims to provide an anatomic foundation in assessing development of the contralateral hemisphere, by measuring volumetric growth after hemispherotomy. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.007DOI Listing
February 2019

Correspondence Reply to Madaan et al.

Pediatr Neurol 2019 Feb 2. Epub 2019 Feb 2.

Child and Adolescent Neurology and Epilepsy, Mayo Clinic, Rochester, Minnesota. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.004DOI Listing
February 2019

6-Pyruvoyltetrahydropterin Synthase Deficiency: Review and Report of 28 Arab Subjects.

Pediatr Neurol 2019 Feb 18. Epub 2019 Feb 18.

Division of Genetics, Department of Pediatrics, King Abdulaziz Medical City, Ministry of National Guard-Health Affairs (MNGHA), Riyadh, Saudi Arabia; King Abdullah International Medical Research Center (KAIMRC), Riyadh, Saudi Arabia; College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia. Electronic address:

Background: Tetrahydrobiopterin is an essential cofactor for the hydroxylation of aromatic amino acids phenylalanine, tyrosine, and tryptophan. Therefore, tetrahydrobiopterin deficiency results in hyperphenylalaninemia as well as dopamine and serotonin depletion in the central nervous system. The enzyme 6-pyruvoyltetrahydropterin synthase catalyzes the second step of de novo synthesis of tetrahydrobiopterin, and its deficiency is the most frequent cause of tetrahydrobiopterin metabolism disorders. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.008DOI Listing
February 2019
1.504 Impact Factor

Focal Status Epilepticus With Unilateral Brain Edema: An Expanding Spectrum.

Pediatr Neurol 2019 Feb 8. Epub 2019 Feb 8.

Pediatric Neurology Division, Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.002DOI Listing
February 2019
1 Read

Hemidystonia in a Neonate.

Pediatr Neurol 2019 Feb 27. Epub 2019 Feb 27.

Division of Child Neurology, Department of Neurology, Albany Medical College, Albany, New York. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.009DOI Listing
February 2019
1.504 Impact Factor

Chronic Encephalopathy, Startle, and Intracranial Calcification: Think Beyond Intrauterine Infections.

Pediatr Neurol 2019 Jan 30. Epub 2019 Jan 30.

Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.014DOI Listing
January 2019
1.504 Impact Factor

Unilateral Bowing of Legs - Do not Forget to Examine the Skin.

Pediatr Neurol 2019 Feb 7. Epub 2019 Feb 7.

Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.022DOI Listing
February 2019

Gabapentin Use for Hospitalized Neonates.

Pediatr Neurol 2019 Feb 21. Epub 2019 Feb 21.

Center for Perinatal Research, Nationwide Children's Hospital, Columbus, Ohio; Department of Pediatrics, The Ohio State University, Columbus, Ohio. Electronic address:

Background: Despite some clinician advocacy for the use of gabapentin to treat neonatal irritability of presumed neurological origin, the extent of gabapentin administration to hospitalized neonates is unknown. We aimed to identify trends in gabapentin utilization among infants hospitalized in neonatal intensive care units (NICUs) across the United States and to evaluate the associations between clinical diagnoses and gabapentin treatment.

Methods: We analyzed neonates admitted to the NICU using the Pediatric Health Information System (2005 to 2016) to measure treatment timing, duration, and frequency. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.012DOI Listing
February 2019

Atypical Prodromal Symptoms Help to Distinguish Patients With Psychogenic Nonsyncopal Collapse Among Youth Referred for Fainting.

Authors:
Geoffrey L Heyer

Pediatr Neurol 2019 Feb 13. Epub 2019 Feb 13.

Department of Neurology, Dell Medical School, Austin, Texas. Electronic address:

Background: Distinguishing patients with psychogenic nonsyncopal collapse (PNSC), a conversion disorder that resembles syncope, can pose a difficult clinical challenge. Using the open-ended question "what does it feel like to faint?," the present study aimed to characterize how patients with PNSC perceive and communicate the prodromal symptoms associated with their attacks by comparing narratives between patients with PNSC and those with syncope.

Methods: During a 42-month database-type study of tilt-table diagnoses, all patients with a history of fainting were asked the open-ended question. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08878994193000
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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.006DOI Listing
February 2019
5 Reads
1.504 Impact Factor

A Rare Cause of Childhood Stroke.

Pediatr Neurol 2019 Feb 8. Epub 2019 Feb 8.

Department of Radiology, Jubilee Mission Medical College Hospital and Research Institute Thrissur, Kerala, India.

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.003DOI Listing
February 2019

Hemorrhagic Transformation of Arterial Ischemic and Venous Stroke in Children.

Pediatr Neurol 2019 Feb 8. Epub 2019 Feb 8.

Division of Neurology, Children's Hospital of Philadelphia, Departments of Neurology and Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address:

Hemorrhagic transformation can complicate both arterial ischemic stroke and cerebral sinus venous thrombosis. Risk factors for hemorrhagic transformation after adult arterial ischemic stroke include larger infarct volume, cardioembolic stroke, and anticoagulation in the acute period. Large hemorrhagic transformation in adults is associated with poor outcome. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.023DOI Listing
February 2019
1 Read

Unexpected Neuroimaging Findings in an Adolescent With First Seizure Presentation.

Pediatr Neurol 2019 Feb 2. Epub 2019 Feb 2.

Department of Medical Imaging, Gold Coast University Hospital, Southport, Queensland, Australia. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.019DOI Listing
February 2019

Multiple Autoimmune Disorders in Aicardi-Goutières Syndrome.

Pediatr Neurol 2019 Feb 2. Epub 2019 Feb 2.

Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas.

Background: Aicardi-Goutières syndrome is an early-onset encephalopathy with presumed immune pathogenesis caused by inherited defects in nucleic acid metabolism. It is a model disease to study systemic autoimmunity, and there are many clinical, genetic, and basic science considerations that underline a possible overlap between Aicardi-Goutières syndrome and systemic lupus erythematosus.

Results: We describe a 15-year-old girl with Aicardi-Goutières syndrome due to compound heterozygous pathogenic variants in SAMHD1 (sterile alpha motif domain and HD domain-containing protein 1). Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.017DOI Listing
February 2019
2 Reads

Short Takes.

Pediatr Neurol 2019 Feb 2. Epub 2019 Feb 2.

Department of Neurology, SUNY Downstate, Clarkson Ave, Brooklyn, NY. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.021DOI Listing
February 2019

TBCK Encephaloneuropathy With Abnormal Lysosomal Storage: Use of a Structural Variant Bioinformatics Pipeline on Whole-Genome Sequencing Data Unravels a 20-Year-Old Clinical Mystery.

Pediatr Neurol 2019 Feb 13. Epub 2019 Feb 13.

Department of Medical Genetics, Oslo University Hospital and University of Oslo, Oslo, Norway. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.001DOI Listing
February 2019
1 Read

Herculean Boy With Facial Myokymia.

Pediatr Neurol 2019 Feb 20. Epub 2019 Feb 20.

Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.011DOI Listing
February 2019
1.504 Impact Factor

So Dramatic They Could Never Be Overlooked: History of Pediatric Neuromyelitis Optica.

Authors:
Alison Christy

Pediatr Neurol 2019 Feb 10. Epub 2019 Feb 10.

Pediatric Neurology, Providence Health & Services, Portland, Oregon. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.004DOI Listing
February 2019

Chondrodysplasia Punctata: A Clue to the Zellweger Spectrum Disorders.

Authors:
Nigel S Bamford

Pediatr Neurol 2019 Feb 2. Epub 2019 Feb 2.

Departments of Pediatrics, Neurology, Cellular and Molecular Physiology, Yale University, New Haven, Connecticut. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.013DOI Listing
February 2019

Benign External Hydrocephalus in Children: A Condition in Search of an Etiology.

Pediatr Neurol 2019 Feb 23. Epub 2019 Feb 23.

Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.014DOI Listing
February 2019
1.504 Impact Factor

Physical and Family History Variables Associated With Neurological and Cognitive Development in Sturge-Weber Syndrome.

Pediatr Neurol 2018 Dec 20. Epub 2018 Dec 20.

Department of Neurology, Kennedy Krieger Institute, Baltimore, Maryland; Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland.

Background: Sturge-Weber syndrome (SWS) is caused by a somatic mutation in GNAQ leading to capillary venous malformations in the brain presenting with various neurological, ophthalmic, and cognitive symptoms of variable severity. This clinical variability makes accurate prognosis difficult. We hypothesized that the greater extent of physical factors (extent of skin, eye, and brain involvement), presence of possible genetic factors (gender and family history), and age of seizure onset may be associated with greater symptom severity and need for surgery in patients with SWS. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08878994183069
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http://dx.doi.org/10.1016/j.pediatrneurol.2018.12.002DOI Listing
December 2018
4 Reads

Pediatric Multiple Sclerosis in Rio de Janeiro: Secondary Progression and Disability.

Pediatr Neurol 2019 May 31;94:48-54. Epub 2018 Dec 31.

Background: The onset of multiple sclerosis (MS) in 2% to 10% of cases occurs prior to 18 years of age. Early age onset appears to affect some aspects of multiple sclerosis. The objective of our study was to evaluate the prevalence, the clinical and demographic characteristics, and the disease progression in a sample of pediatric multiple sclerosis patients from a mixed population. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2018.12.015DOI Listing
May 2019
1 Read

Analysis for the Association Between Corpus Callosum Thickness and Corpus Callosotomy Outcomes for Patients With Epileptic Spasms or Tonic Spasms.

Pediatr Neurol 2019 Jan 30. Epub 2019 Jan 30.

Epilepsy Center, Seirei Hamamatsu General Hospital, Shizuoka, Japan.

Background: This retrospective study is designed to determine whether the thickness of the corpus callosum can predict corpus callosotomy outcome in pediatric patients with epileptic or tonic spasms.

Methods: We retrospectively studied 25 patients (18 boys) with intractable childhood-onset epileptic or tonic spasms who underwent corpus callosotomy between March 2008 and January 2017. Seizure outcomes were classified as favorable (class I and II of Engel's outcome classification) or unfavorable (class III and IV of Engel's outcome classification) at 12 months postoperatively. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.012DOI Listing
January 2019
3 Reads

Girl With Progressive Head Enlargement and Gait Disturbance: Clinicoradiological Clues.

Pediatr Neurol 2019 Jan 30. Epub 2019 Jan 30.

Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.011DOI Listing
January 2019
2 Reads
1.504 Impact Factor

Clinical, Radiological, and Demographic Details of Benign External Hydrocephalus: A Population-Based Study.

Pediatr Neurol 2019 Jan 30. Epub 2019 Jan 30.

Department of Clinical Medicine K1, University of Bergen, Norway; Department of Neurosurgery, Haukeland University Hospital, Bergen, Norway.

Background: Benign external hydrocephalus has an incidence of about 0.4 per 1000 live births. It affects infants and is characterized by an increasing head circumference and typical neuroimaging findings. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.015DOI Listing
January 2019
3 Reads

The Ketogenic and Modified Atkins Diet Therapy for Children With Refractory Epilepsy of Genetic Etiology.

Pediatr Neurol 2019 May 29;94:32-37. Epub 2018 Dec 29.

Mayo Clinic, Rochester.

Background: The ketogenic diet is an accepted treatment modality in refractory childhood epilepsy. In this study, we analyzed the efficacy and tolerability of the ketogenic and modified Atkins diets in children with refractory epilepsy of genetic etiology and studied the effect of the diet on seizure frequency.

Methods: The records of children with a genetic etiology for refractory epilepsy treated with ketogenic and modified Atkins diet between September 2005 and July 2016 were reviewed. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2018.12.012DOI Listing
May 2019
2 Reads

Area Postrema: Fetal Maturation, Tumors, Vomiting Center, Growth, Role in Neuromyelitis Optica.

Pediatr Neurol 2019 May 25;94:21-31. Epub 2018 Dec 25.

Department of Paediatric Neurology, Children's University Hospital, Zürich, Switzerland.

Introduction: The area postrema in the caudal fourth ventricular floor is highly vascular without blood-brain or blood-cerebrospinal fluid barrier. In addition to its function as vomiting center, several others are part of the circumventricular organs for vasomotor/angiotensin II regulation, role in neuromyelitis optica related to aquaporin-4, and somatic growth and appetite regulation. Functions are immature at birth. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2018.12.006DOI Listing
May 2019
1 Read

What is the Role of Mechanical Thrombectomy in Childhood Stroke?

Pediatr Neurol 2019 Jan 17. Epub 2019 Jan 17.

Department of Neurology, University of Washington, Seattle, Washington. Electronic address:

Background: Like adults, most children have lifelong morbidity after stroke. Revascularization therapies such as intravenous tissue plasminogen activator and mechanical thrombectomy may be options to decrease this morbidity in selected children, although currently there are no evidence-based recommendations to guide treatment. The utility and safety of mechanical thrombectomy in childhood stroke is unknown because of the lack of safety trials, case-controlled trials, and comprehensive retrospective studies. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.009DOI Listing
January 2019
1 Read

Highly Selective Eating in Autism Spectrum Disorder Leading to Scurvy: A Series of Three Patients.

Pediatr Neurol 2019 May 28;94:61-63. Epub 2018 Dec 28.

Department of Pediatrics, Ruth Rappaport Children's Hospital, Rambam Medical Center, Haifa, Israel; Pediatric Rheumatology Service, Ruth Rappaport Children's Hospital, Rambam Medical Center, Haifa, Israel.

Background: Some children with autism spectrum disorder (ASD) have highly specific food selectivity and therefore are prone to nutritional deficiencies of different kinds.

Patients: We document three children with ASD who presented with refusal to walk and gingivitis who underwent comprehensive evaluations before establishing the diagnosis of vitamin C deficiency (scurvy). The symptoms resolved after treatment with vitamin C. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08878994183088
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http://dx.doi.org/10.1016/j.pediatrneurol.2018.12.011DOI Listing
May 2019
17 Reads

Functional Connectivity in Term Neonates With Hypoxic-Ischemic Encephalopathy Undergoing Therapeutic Hypothermia.

Pediatr Neurol 2019 May 9;94:74-79. Epub 2019 Jan 9.

Department of Pediatrics, Larner College of Medicine, University of Vermont, Burlington, Vermont.

Background: We investigated whether therapeutic hypothermia and rewarming impact functional connectivity using electroencephalography (EEG) as a measure in neonates with hypoxic-ischemic encephalopathy. We hypothesized that EEG coherence and voltage correlations would be lower and phase lag greater in infants with hypoxic-ischemic encephalopathy than control subjects and that functional connectivity would evolve during therapeutic hypothermia with the greatest improvement occurring during rewarming.

Methods: This study was a retrospective study of 14 term neonates (greater than 37 weeks) with moderate hypoxic-ischemic encephalopathy who underwent therapeutic hypothermia and rewarming. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.006DOI Listing
May 2019
2 Reads

Selective Eating: A Common Fuss in Neurodevelopmental Disorders.

Pediatr Neurol 2019 Jan 19. Epub 2019 Jan 19.

Child Neurology Division, Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.007DOI Listing
January 2019
1 Read

Arrest of Fetal Brain Development in ALG11-Congenital Disorder of Glycosylation.

Pediatr Neurol 2019 May 24;94:64-69. Epub 2018 Dec 24.

National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland.

Background: Arrest of fetal brain development and the fetal brain disruption sequence describe a severe phenotype involving microcephaly, occipital bone prominence, and scalp rugae. Congenital disorders of glycosylation are a heterogeneous group of inherited disorders involved in glycoprotein and glycolipid biosynthesis, which can cause microcephaly and severe neurodevelopmental disability.

Methods: We report an example of fetal microcephaly diagnosed at 36 weeks' gestation with a history of normal fetal biometry at 20 weeks' gestation. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08878994183115
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http://dx.doi.org/10.1016/j.pediatrneurol.2018.12.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6450714PMC
May 2019
15 Reads

Macroglossia, Dry Skin, Developmental Delay, and Stippled Epiphysis: A Treatable Condition.

Pediatr Neurol 2019 May 4;94:82-83. Epub 2019 Jan 4.

Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.001DOI Listing
May 2019
2 Reads
1.504 Impact Factor

Genotypic Spectrum and Natural History of Cavitating Leukoencephalopathies in Childhood.

Pediatr Neurol 2019 May 8;94:38-47. Epub 2019 Jan 8.

Department of Pediatrics, Peking University First Hospital, Beijing, China. Electronic address:

Background: We aimed to delineate the pattern of natural course, neuroimaging features, and the genotypic spectrum of cavitating leukoencephalopathies.

Methods: Children (age of onset ≤16 years) who met the criteria for cavitating leukoencephalopathies from January 2009 to October 2018 were identified. Whole-exome sequencing and prospective follow-up study of the natural history and brain magnetic resonance imaging (MRI) were performed. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.002DOI Listing
May 2019
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Vacuum Extraction in Preterm Deliveries and Long-Term Neurological Outcome of the Offspring.

Pediatr Neurol 2019 May 15;94:55-60. Epub 2019 Jan 15.

Department of Obstetrics and Gynecology, Soroka University Medical Center, Ben Gurion University of the Negev, Beer Sheva, Israel.

Background: Concern exists regarding a possible harmful impact of vacuum extraction on the preterm newborn. We aimed to evaluate the long-term pediatric neurodevelopmental outcomes of the preterm offspring after vacuum extraction.

Methods: A population-based cohort analysis was performed comparing the risk for long-term neurological morbidity (up to age 18 years) in preterm (less than 37 completed weeks of gestation) children born via following three delivery modes: vacuum extraction, spontaneous delivery, and Caesarean delivery performed during the second stage of labor. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2018.12.010DOI Listing
May 2019
3 Reads
1.504 Impact Factor

Where to From Here?

Authors:
Nomazulu Dlamini

Pediatr Neurol 2018 Dec 20. Epub 2018 Dec 20.

Division of Neurology, The Hospital for Sick Children, Toronto, Canada. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2018.12.004DOI Listing
December 2018
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Children With Severe Traumatic Brain Injury, Intracranial Pressure, Cerebral Perfusion Pressure, What Does it Mean? A Review of the Literature.

Pediatr Neurol 2019 May 11;94:3-20. Epub 2019 Jan 11.

Division of Neurosurgery, Nationwide Children's Hospital, Columbus, Ohio.

Severe traumatic brain injury is a leading cause of morbidity and mortality in children. In 2003 the Brain Trauma Foundation released guidelines that have since been updated (2010) and have helped standardize and improve care. One area of care that remains controversial is whether the placement of an intracranial pressure monitor is advantageous in the management of traumatic brain injury. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2018.12.003DOI Listing
May 2019
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Cerebral Hemodynamics During Neonatal Cerebrospinal Fluid Removal.

Pediatr Neurol 2019 May 8;94:70-73. Epub 2019 Jan 8.

Division of Neonatology, Institute for Maternal and Child Health, IRCSS "Burlo Garofolo", Trieste, Italy. Electronic address:

Background: Standard treatment of neonatal posthemorrhagic hydrocephalus is cerebrospinal fluid removal. The aim of this study was to assess how much cerebrospinal fluid volume removal, by ventricular reservoir taps, is needed to improve cerebral hemodynamics and oxygenation in neonatal posthemorrhagic hydrocephalus.

Methods: Cerebral hemodynamics and oxygenation were continuously monitored by near-infrared spectroscopy in four newborns (one term and three preterm) during 28 ventricular reservoir taps. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.01.005DOI Listing
May 2019
2 Reads