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    2759 results match your criteria Acute Disseminated Encephalomyelitis

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    Microcephaly caused by congenital Zika virus infection and viral detection in maternal urine during pregnancy.
    Rev Assoc Med Bras (1992) 2018 Jan;64(1):11-14
    Department of Radiology and Medical Imaging, Documenta - Hospital Materno Infantil Sinhá Junqueira, Ribeirão Preto, SP, Brazil.
    Currently Latin America is undergoing a major epidemic of Zika virus, which is transmitted by Aedes mosquitoes. Concern for Zika virus infection has been increasing as it is suspected of causing brain defects in newborns such as microcephaly and, more recently, potential neurological and autoimmune complications including Guillian-Barré syndrome and acute disseminated encephalomyelitis. We describe a case of virus infection in a 25-year-old woman during the first trimester of her pregnancy, confirmed by laboratory tests only for the detection of viral particles in maternal urine, with imaging studies demonstrating the progression of cranial and encephalic changes in the fetus and later in the newborn, such as head circumference reduction, cerebral calcifications and ventriculomegaly. Read More

    Use of Cyclophosphamide in a Child With Fulminant Acute Disseminated Encephalomyelitis.
    Child Neurol Open 2018 11;5:2329048X18754631. Epub 2018 Mar 11.
    Neuroscience department, King Fahad Special Hospital-Dammam, Al-Khobar, Dammam, Saudi Arabia.
    Acute disseminated encephalomyelitis is an immune-mediated inflammatory demyelinating disorder of the central nervous system. The first-line treatment is usually high-dose intravenous methylprednisolone. Intravenous immunoglobulin and plasmapheresis have also shown to be beneficial. Read More

    Dramatic response of postvaccinial acute disseminated encephalomyelitis to methylprednisolone : case report and review of literature.
    Neurol India 1993 Jan;41(1):39-41
    Department of Neurology, G. B. Pant Hospital, New Delhi, India.
    A case of post vaccinial (antirabies - BPL vaccine) acute disseminated encephalomyelitis, documented on MRI is reported. The patient had dramatic improvement with methylprednisolone, administered in a dose of 1gm daily for 5 days. Read More

    Optic neuritis in paediatric patients: Experience over 27 years and a management protocol.
    Neurologia 2018 Mar 8. Epub 2018 Mar 8.
    Sección de Neuropediatría, Hospital Infantil Universitario Miguel Servet, Zaragoza, España.
    Introduction And Objective: In this article, we present our experience on optic neuritis (ON) and provide a diagnostic/therapeutic protocol, intended to rule out other aetiologies (particularly infection), and a fact sheet for parents.

    Material And Methods: We conducted a descriptive, retrospective study of patients with ON over a 27-year period (1990-2017). A review of the available scientific evidence was performed in order to draft the protocol and fact sheet. Read More

    Pediatric multiple sclerosis: a review.
    BMC Neurol 2018 Mar 9;18(1):27. Epub 2018 Mar 9.
    Department of Neurology, Neurosurgery and Medical Genetic of the Pirogov's Russian National Research Medical University and MS Clinic at the Usupov's Hospital, Ostrovitianov str. 1, Moscow, 117997, Russia.
    Background: Pediatric-onset multiple sclerosis (POMS) prevalence and incidence rates are increasing globally. No disease-modifying therapy are approved for MS pediatric population. Hence, we aim to review the literature on POMS to guide treating physicians on the current understanding of diagnosis and management of pediatric MS. Read More

    Cerebrospinal fluid γδ T cell frequency is age-related: a case control study of 435 children with inflammatory and non-inflammatory neurologic disorders.
    Clin Exp Immunol 2018 Feb 27. Epub 2018 Feb 27.
    From the National Pediatric Myoclonus Center, Orlando, FL.
    Studies of cerebrospinal fluid (CSF) γδ T cells in children are limited, especially due to the lack of control data. In adults, gamma/delta T cells (TCR-γδ) residing in the intrathecal space are sometimes involved in neuroinflammation. To evaluate the possible role of γδ T cells in pediatric neuroinflammation, we immunophenotyped CSF and blood lymphocytes using flow cytometry in a case-control study of 100 children with non-inflammatory neurologic disorders (NIND), 312 with opsoclonus-myoclonus (OMS), and 23 with other inflammatory neurologic disorders (OIND). Read More

    [Acute Urinary Retention Caused by Acute Disseminated Encephalomyelitis : A Case Report].
    Hinyokika Kiyo 2018 Jan;64(1):17-20
    The Department of Urology, Nara Prefecture General Medical Center.
    An 11-year-old boy was referred to our department with the chief complaint of acute urinary retention. He had had a history of viral enteritis a few days before the onset of dysuria. He presented with a slight fever, mild headache and weakness of the extremities. Read More

    Myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies are highly specific in children with acquired demyelinating syndromes.
    Dev Med Child Neurol 2018 Feb 22. Epub 2018 Feb 22.
    Department of Paediatric Neurology, Great Ormond Street Hospital for Children, London, UK.
    Aim: Our objectives were to evaluate the utility of measuring myelin oligodendrocyte glycoprotein (MOG) and aquaporin-4 (AQP4) antibodies (Ab) in clinical practice and describe their associated neurological phenotypes in children.

    Method: Between 2012 and 2017, 371 children with suspected acquired demyelinating syndromes (ADS) seen in three tertiary centres were tested for MOG-Ab and AQP4-Ab. Medical notes were retrospectively reviewed, and clinical and demographic data compiled. Read More

    Acute disseminated encephalomyelitis following Influenza A pneumonia.
    Clin Case Rep 2018 Feb 22;6(2):436-438. Epub 2017 Dec 22.
    Internal Medicine DepartmentHurley Medical Center/Michigan State UniversityTwo Hurley Plaza, Ste 212FlintMichigan48503.
    Acute disseminated encephalomyelitis (ADEM) is an autoimmune demyelinating disorder of the central nervous system and can present following influenza A infections as multifocal neurological deficits. ADEM remains a challenging diagnosis, and high clinical suspicious coupled with laboratory investigations and neuroimaging is required to exclude other primary and secondary demyelinating disorders. Read More

    Paediatric acute disseminated encephalomyelitis followed by optic neuritis: disease course, treatment response and outcome.
    Eur J Neurol 2018 Feb 14. Epub 2018 Feb 14.
    Department of Paediatric Neurology, Erasmus MC-Sophia, Rotterdam, The Netherlands.
    Background And Purpose: Acute disseminated encephalomyelitis followed by optic neuritis (ADEM-ON) is a rare demyelinating syndrome that is different from multiple sclerosis and neuromyelitis optica spectrum disorder. The aim of this study was to describe the disease course, treatment response and outcome of children with ADEM-ON.

    Methods: Children of <18 years of age were identified from six countries of the EU Paediatric Demyelinating Disease Consortium. Read More

    MRI of the first event in pediatric acquired demyelinating syndromes with antibodies to myelin oligodendrocyte glycoprotein.
    J Neurol 2018 Feb 8. Epub 2018 Feb 8.
    Department of Pediatric Neurology, Children's Hospital Datteln, Witten/Herdecke University, Datteln, Germany.
    Antibodies against the myelin oligodendrocyte glycoprotein (MOG-Ab) can be detected in various pediatric acquired demyelinating syndromes (ADS). Here, we analyze the spectrum of neuroradiologic findings in children with MOG-Ab and a first demyelinating event. The cerebral and spinal MRI of 69 children with different ADS was assessed in regard to the distribution and characteristics of lesions. Read More

    Differences in the Reponses to Apheresis Therapy of Patients With 3 Histopathologically Classified Immunopathological Patterns of Multiple Sclerosis.
    JAMA Neurol 2018 Feb 5. Epub 2018 Feb 5.
    Institute of Neuropathology, University Medical Center Goettingen, Goettingen, Germany.
    Importance: Plasma exchange and immunoadsorption are second-line apheresis therapies for patients experiencing multiple sclerosis relapses. Early active multiple sclerosis lesions can be classified into different histopathological patterns of demyelination. Pattern 1 and 2 lesions show T-cell- and macrophage-associated demyelination, and pattern 2 is selectively associated with immunoglobulin and complement deposits, suggesting a humoral immune response. Read More

    Disease Course and Treatment Responses in Children With Relapsing Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.
    JAMA Neurol 2018 Jan 5. Epub 2018 Jan 5.
    Children's Neurosciences, Evelina London Children's Hospital at Guy's and St Thomas' National Health Service Foundation Trust, King's Health Partners Academic Health Science Centre, London, United Kingdom.
    Importance: Myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) are consistently identified in a range of demyelinating disorders in adults and children. Current therapeutic strategies are largely center specific, and no treatments have been formally evaluated.

    Objective: To examine the clinical phenotypes, treatment responses, and outcomes of children with relapsing MOG-Ab-associated disease. Read More

    'Leukodystrophy-like' phenotype in children with myelin oligodendrocyte glycoprotein antibody-associated disease.
    Dev Med Child Neurol 2017 Dec 30. Epub 2017 Dec 30.
    Department of Paediatric Neurology, Great Ormond Street Hospital for Children, London, UK.
    Aim: To review the demographics and clinical and paraclinical parameters of children with myelin oligodendrocyte glycoprotein (MOG) antibody-associated relapsing disease.

    Method: In this UK-based, multicentre study, 31 children with MOG antibody-associated relapsing disease were studied retrospectively.

    Results: Of the 31 children studied, 14 presented with acute disseminated encephalomyelitis (ADEM); they were younger (mean 4. Read More

    Chemokines CXCL10, CXCL11, and CXCL13 in acute disseminated encephalomyelitis, non-polio enterovirus aseptic meningitis, and neuroborreliosis: CXCL10 as initial discriminator in diagnostic algorithm?
    Neurol Sci 2018 Mar 29;39(3):471-479. Epub 2017 Dec 29.
    Pediatric Infectious Diseases Department, University Hospital for Infectious Diseases "dr.Fran Mihaljevic", Mirogojska 8, 10 000, Zagreb, Croatia.
    We investigated potential diagnostic usefulness of serum and cerebrospinal fluid (CSF) concentrations of chemokines CXCL10, CXCL11, and CXCL13 in pediatric patients with acute disseminated encephalomyelitis (ADEM) (n = 23), non-polio enterovirus aseptic meningitis (NPEV AM) (n = 20), and neuroborreliosis (NB) (n = 21) and children with acute infectious diseases with neurological symptoms but with excluded neuroinfection/neuroinflammation (controls, n = 20). CSF levels of CXCL10 and CXCL11 were higher in patients with NPEV AM than those in other children, and CXCL10 levels showed a high discriminative potential (area under the receiver operating characteristic curve, ROC, 0.982) with high specificity and sensitivity (both 95%). Read More

    Unihemispheric central nervous system vasculitis.
    eNeurologicalSci 2017 Jun 13;7:44-48. Epub 2017 May 13.
    Neurological Institute, University Hospitals Cleveland Medical Center, 11100 Euclid Ave, Cleveland, OH 44106, USA.
    Patients with primary central nervous system vasculitis (PCNSV) usually manifest with multiple enhancing bilateral hemispheric lesions. We presented an extremely rare clinical course and follow-up of a patient with PCNSV affecting only a single (right) hemisphere. A 33-year-old previously healthy man presented with a left hand clonic seizure followed by a secondary generalized tonic-clonic seizure and dysarthria. Read More

    Necrotic-hemorrhagic myelitis: A rare malignant variant of parainfectious acute disseminated encephalomyelitis in childhood.
    J Neurol Sci 2018 Jan 23;384:58-60. Epub 2017 Nov 23.
    Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy; Department of Neurology, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy. Electronic address:

    X-linked Charcot-Marie-Tooth disease with GJB1 mutation presenting as acute disseminated encephalomyelitis-like illness: A case report.
    Medicine (Baltimore) 2017 Dec;96(49):e9176
    Department of Pediatrics, Chonbuk National University School of Medicine, Jeonju, Korea.
    Rationale: Charcot-Marie-Tooth disease (CMT) is typically an autosomal dominant, inherited neuropathy, although there is a rare male X-linked CMT. Such patients show central nervous system (CNS) involvement in addition to peripheral neuropathy. Recently, we encountered a patient who presented with acute disseminated encephalomyelitis (ADEM)-like symptoms, but was later diagnosed as having X-linked CMT (CMTX) due to a mutation. Read More

    Childhood acute disseminated encephalomyelitis: an Egyptian pilot study.
    Acta Neurol Belg 2017 Dec 8. Epub 2017 Dec 8.
    Faculty of Medicine, Paediatrics Department, Ain Shams University, Cairo, Egypt.
    Describing the variable clinical features, laboratory findings, neuroimaging findings, and treatments given to children who presented with ADEM and following them up both clinically and radiologically. 21 patients were recruited: 14 new cases, and 7 old ones presenting over the preceding 5 years (retrospective review of existing data). 11 males and 10 females, with a mean age of 4. Read More

    Imaging of Acquired Demyelinating Syndrome With 18F-FDG PET/CT.
    Clin Nucl Med 2018 Feb;43(2):103-105
    Acquired demyelinating syndromes include acute disseminated encephalomyelitis, transverse myelitis and may progress to multiple sclerosis (MS). Acute disseminated encephalomyelitis is characterized by impairment of level of consciousness and multifocal neurological deficits and transverse myelitis by back pain, weakness and sphincter dysfunction. Only a few cases of acquired demyelinating syndrome have been imaged with F-FDG PET/CT. Read More

    Diffuse glioblastoma resembling acute hemorrhagic leukoencephalitis.
    Quant Imaging Med Surg 2017 Oct;7(5):592-597
    Unit of Neuroradiology, Department of Advanced Biomedical Sciences, "Federico II" University, Naples, Italy.
    We report the case of a young man with sudden onset of diplopia after an upper respiratory tract infection. Based on the first radiological findings acute hemorrhagic leukoencephalitis, a variant of acute disseminated encephalomyelitis, was suspected and treatment with high dose intravenous dexamethasone was started but it was stopped for intolerance. The patient clinically worsened, developing gait instability, ataxia and ophthalmoplegia; brain MRI performed 20 days later showed severe progression of the disease with subependymal dissemination. Read More

    Diffusion Tensor Imaging as a Biomarker to Differentiate Acute Disseminated Encephalomyelitis From Multiple Sclerosis at First Demyelination.
    Pediatr Neurol 2018 Jan 28;78:70-74. Epub 2017 Sep 28.
    Department of Pediatric and Developmental Neurology, Washington University School of Medicine, St. Louis, Missouri. Electronic address:
    Background: There are no clinical features or biomarkers that can reliably differentiate acute disseminated encephalomyelitis from multiple sclerosis at the first demyelination attack. Consequently, a final diagnosis is sometimes delayed by months and years of follow-up. Early treatment for multiple sclerosis is recommended to reduce long-term disability. Read More

    Biomarkers in the evolution of multiple sclerosis.
    Neurodegener Dis Manag 2017 Nov;7(6s):3-6
    Clinical Department of Neurology, Medical University of Innsbruck, Anichstrasse 35, 6020 Innsbruck, Austria.
    Nonimaging biomarkers can be applied in differential diagnosis, evaluation of disease progression and therapy monitoring of multiple sclerosis (MS). Presence of oligoclonal IgG bands in cerebrospinal fluid is a diagnostic element and a negative predictor of MS evolution. AQP4 antibodies are pathogenic and diagnostic for neuromyelitis optica spectrum disorder. Read More

    Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination.
    J Neurol Neurosurg Psychiatry 2018 Feb 15;89(2):127-137. Epub 2017 Nov 15.
    TY Nelson Department of Neurology and Neurosurgery, Children's Hospital, Westmead, New South Wales, Australia.
    Objective: We characterised the clinical course, treatment and outcomes in 59 patients with relapsing myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination.

    Methods: We evaluated clinical phenotypes, annualised relapse rates (ARR) prior and on immunotherapy and Expanded Disability Status Scale (EDSS), in 218 demyelinating episodes from 33 paediatric and 26 adult patients.

    Results: The most common initial presentation in the cohort was optic neuritis (ON) in 54% (bilateral (BON) 32%, unilateral (UON) 22%), followed by acute disseminated encephalomyelitis (ADEM) (20%), which occurred exclusively in children. Read More

    Short term outcome of ADEM: Results from a retrospective cohort study from South India.
    Mult Scler Relat Disord 2017 Nov 21;18:128-134. Epub 2017 Sep 21.
    Government Medical College Trivandrum, Kerala 695011, India. Electronic address:
    Introduction: Acute disseminated encephalomyelitis (ADEM), an immune mediated inflammatory disease is common in children. The profile and immediate outcome of children hospitalized with ADEM is scarce in the available literature.

    Objectives: We aimed to study the clinical profile of children with ADEM and to look for prognostic factors for outcome at discharge from hospital METHODS: We chose a retrospective cohort study of all children diagnosed with ADEM at our institution between January 2006 and December 2015, and they were evaluated, after excluding other diagnoses when they were summoned for a follow up visit. Read More

    Guillain-Barré Syndrome, Acute Disseminated Encephalomyelitis and Encephalitis Associated with Zika Virus Infection in Brazil: Detection of Viral RNA and Isolation of Virus during Late Infection.
    Am J Trop Med Hyg 2017 Nov;97(5):1405-1409
    Aggeu Magalhães Institute, Oswaldo Cruz Foundation (Fiocruz), Recife, Pernambuco, Brazil.
    Zika virus (ZIKV) emerged in Brazil in 2015, which was followed by an increase of Guillain-Barre Syndrome (GBS) cases. We report the epidemiological, clinical, and laboratory findings of the first six neurological cases associated with ZIKV in Brazil seen in a reference neurology hospital in Pernambuco, Brazil. In all cases, ZIKV was detected in serum and/or cerebrospinal fluid (CSF) samples. Read More

    Severe oxidative stress in an acute inflammatory demyelinating model in the rhesus monkey.
    PLoS One 2017 14;12(11):e0188013. Epub 2017 Nov 14.
    Department of Immunobiology, Biomedical Primate Research Centre, Rijswijk, The Netherlands.
    Oxidative stress is increasingly implicated as a co-factor of tissue injury in inflammatory/demyelinating disorders of the central nervous system (CNS), such as multiple sclerosis (MS). While rodent experimental autoimmune encephalomyelitis (EAE) models diverge from human demyelinating disorders with respect to limited oxidative injury, we observed that in a non-human primate (NHP) model for MS, namely EAE in the common marmoset, key pathological features of the disease were recapitulated, including oxidative tissue injury. Here, we investigated the presence of oxidative injury in another NHP EAE model, i. Read More

    New spectrum of the neurologic consequences of Zika.
    J Neurol Sci 2017 Dec 1;383:214-215. Epub 2017 Nov 1.
    Faculty of Medical Sciences, National Autonomous University of Honduras, Honduras.
    Zika virus infection represents a new neuropathological agent with association to a wide spectrum of neurological complications: a) Congenital Zika Syndrome by affecting the neural stem cells of the human fetal brain; b) Guillain-Barré Syndrome by an autoimmune response against peripheral myelin and/or axonal components or probable direct inflammatory reaction; c) Encephalitis/meningoencephalitis and myelitis by a direct viral inflammatory process on the central nervous system; d) Sensory neuropathy by infecting directly the peripheral neurons and causing substantial cell death and pathogenic transcriptional dysregulation; e) Acute Disseminated Encephalomyelitis and optic neuropathy; f) Seizures and Epilepsy and g) childhood arterial ischemic stroke by probable inflammatory reaction and endothelial injury. Read More

    The Spectrum of Inflammatory Acquired Demyelinating Syndromes in Children.
    Semin Pediatr Neurol 2017 Aug 23;24(3):189-200. Epub 2017 Aug 23.
    Department of Pediatric Neurology, Sick Kids Children's Hospital, Toronto, Canada.
    Acquired demyelinating syndromes in childhood comprise a spectrum of monophasic and recurrent inflammatory conditions of the central nervous system. Examples of monophasic conditions include, clinically isolated syndromes such as optic neuritis and transverse myelitis, as well as acute disseminated encephalomyelitis, whereas recurrent disorders include entities such as multiple sclerosis and neuromyelitis optica spectrum disorder. Knowledge about these disorders has expanded due to rigorously evaluated diagnostic criteria, magnetic resonance imaging features, outcomes, and serum biomarkers in these disorders. Read More

    An Adult Case of Recurrent Guillain-Barré Syndrome with Anti-galactocerebroside Antibodies.
    Intern Med 2018 Feb 1;57(3):409-412. Epub 2017 Nov 1.
    Department of Clinical Research, National Hospital Organization Maizuru Medical Center, Japan.
    A 79-year-old woman with a history of Guillain-Barré syndrome (GBS) developed somnolence and tetraparesis after pneumonia. Based on clinical and laboratory findings, she was diagnosed with complications of acute inflammatory demyelinating polyneuropathy (AIDP) and acute disseminated encephalomyelitis (ADEM). Anti-galactocerebroside (Gal-C) IgG antibodies were detected in her serum. Read More

    [A case of anti-MOG antibody-positive multiphasic disseminated encephalomyelitis co-occurring with unilateral cerebral cortical encephalitis].
    Rinsho Shinkeigaku 2017 Nov 26;57(11):723-728. Epub 2017 Oct 26.
    Division of Neurology, Department of Internal Medicine, Tokyo Women's Medical University Yachiyo Medical Center.
    A 20-year-old woman first developed acute disseminated encephalomyelitis (ADEM) at 11 years of age. At 17 years of age, she was hospitalized due to generalized seizure and diagnosed with encephalitis. Brain MRI revealed a FLAIR-hyperintense lesion in the unilateral cerebral cortex. Read More

    Human antibodies against the myelin oligodendrocyte glycoprotein can cause complement-dependent demyelination.
    J Neuroinflammation 2017 Oct 25;14(1):208. Epub 2017 Oct 25.
    Clinical Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria.
    Background: Antibodies to the myelin oligodendrocyte glycoprotein (MOG) are associated with a subset of inflammatory demyelinating diseases of the central nervous system such as acute disseminated encephalomyelitis and neuromyelitis optica spectrum disorders. However, whether human MOG antibodies are pathogenic or an epiphenomenon is still not completely clear. Although MOG is highly conserved within mammals, previous findings showed that not all human MOG antibodies bind to rodent MOG. Read More

    Clinical spectrum and IgG subclass analysis of anti-myelin oligodendrocyte glycoprotein antibody-associated syndromes: a multicenter study.
    J Neurol 2017 Dec 23;264(12):2420-2430. Epub 2017 Oct 23.
    Department of Neuroscience, Biomedicine and Movement Sciences, Neurology Unit, University of Verona, Verona, Italy.
    Anti-myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) recently emerged as a potential biomarker in patients with inflammatory demyelinating diseases of the central nervous system. We here compare the clinical and laboratory findings observed in a cohort of MOG-Ab seropositive and seronegative cases and describe IgG subclass analysis results. Consecutive serum samples referred to Verona University Neuropathology Laboratory for aquaporin-4 (AQP4)-Ab and/or MOG-Ab testing were analysed between March 2014 and May 2017. Read More

    Health-Related Quality of Life in Pediatric Patients With Demyelinating Diseases: Relevance of Disability, Relapsing Presentation, and Fatigue.
    J Pediatr Psychol 2018 Mar;43(2):133-142
    Department of Pediatrics, Baylor College of Medicine.
    Objective: Decreased health-related quality of life (HRQOL) in pediatric patients with multiple sclerosis is established, but little research has examined HRQOL in the broader pediatric demyelinating disease population, and predictors of reduced HRQOL are largely unexplored. We sought to (1) compare generic HRQOL and fatigue of pediatric patients with relapsing (i.e. Read More

    [Anti-Myelin Oligodendrocyte Glycoprotein Antibodies in Paediatric Patients with Optic Neuritis].
    Klin Monbl Augenheilkd 2017 Oct 12;234(10):1243-1249. Epub 2017 Oct 12.
    Neuropädiatrie, Universitätsklinik und Poliklinik für Kinder und Jugendliche Leipzig, Leipzig.
    Myelin oligodendrocyte glycoprotein (MOG) is located on the surface of oligodendrocytes and myelin in the central nervous system. MOG-IgG is associated with acute disseminated encephalomyelitis (ADEM), relapsing and bilateral optic neuritis (NNO), and transverse myelitis (TM) in both paediatric and adult patients. The combination of NNO and TM or other inflammatory brain lesions is a typical feature of neuromyelitis optica spectrum disorders (NMO-SD) which are associated with specific pathogenic autoantibodies against the water channel aquaporin-4 (AQP4-IgG). Read More

    Inflammatory demyelinating diseases of the central nervous system.
    Handb Clin Neurol 2017 ;145:263-283
    Center for Brain Research, Medical University of Vienna, Vienna, Austria. Electronic address:
    Inflammatory demyelinating diseases are a heterogeneous group of disorders, which occur against the background of an acute or chronic inflammatory process. The pathologic hallmark of multiple sclerosis (MS) is the presence of focal demyelinated lesions with partial axonal preservation and reactive astrogliosis. Demyelinated plaques are present in the white as well as gray matter, such as the cerebral or cerebellar cortex and brainstem nuclei. Read More

    Pediatric Optic Neuritis.
    Semin Pediatr Neurol 2017 May 10;24(2):122-128. Epub 2017 Apr 10.
    Department of Ophthalmology, Stein Eye Institute, University of California, Los Angeles, CA. Electronic address:
    Optic neuritis is rare in children in comparison to adults, but accounts for approximately 25% of pediatric acute demyelinating syndromes. Features of pediatric optic neuritis that differ from adults include a higher rate of bilaterality, poor visual acuity on presentation, and papillitis. Diagnostic work-up includes brain magnetic resonance imaging, lumbar puncture, and blood tests to exclude infectious and inflammatory disorders. Read More

    Multifocal Balo's Concentric Sclerosis in Children: Report of a Case and Review of Literature.
    J Neurosci Rural Pract 2017 Aug;8(Suppl 1):S136-S138
    Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
    Balo's concentric sclerosis (BCS) is a rare demyelinating lesion considered to be a variant of multiple sclerosis (MS). On magnetic resonance imaging (MRI) Balo's concentric sclerosis shows the typical concentric pattern. We report a case of 10 year old child with BCS who presented as post infectious acute disseminated encephalomyelitis (ADEM). Read More

    Liver Failure as the Presentation of Ornithine Transcarbamylase Deficiency in a 13-Month-Old Female.
    JIMD Rep 2017 Sep 9. Epub 2017 Sep 9.
    Division of Genetics and Genomics, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02115, USA.
    Ornithine transcarbamylase deficiency (OTCD) is an X-linked urea cycle disorder with variable expressivity in heterozygous females. While liver function testing is often abnormal in patients with OTCD, liver failure is uncommon on presentation. A 13-month-old female with no significant past medical history presented with irritability, right arm weakness, and decreased appetite. Read More

    MOG Spectrum Disorders and Role of MOG-Antibodies in Clinical Practice.
    Neuropediatrics 2018 02 31;49(1):3-11. Epub 2017 Aug 31.
    Department of Pediatric Neurology, Vestische Kinder- und Jugendklinik Datteln, Witten/Herdecke University, Datteln, Germany.
    Myelin oligodendrocyte glycoprotein (MOG) antibodies (abs) are present in one third of all children with an acute demyelinating syndrome (ADS). MOG-abs can be found in acute disseminated encephalomyelitis (ADEM), transverse myelitis, isolated optic neuritis (ON), or recurrent demyelinating diseases, such as multiphasic neuromyelitis optica spectrum disorders (NMOSD) without aquaporin-4 (AQP4) abs or multiphasic ADEM (MDEM), but rarely in children who subsequently develop multiple sclerosis (MS). The presence of MOG-abs is age dependent with the highest seropositivity rates found in young children and an episode of ADEM, whereas older children with MOG-abs present with ON, myelitis, or brainstem symptoms. Read More

    Neurological Presentation of Zika Virus Infection Beyond the Perinatal Period.
    Curr Infect Dis Rep 2017 Aug 16;19(10):35. Epub 2017 Aug 16.
    Infectious Diseases and Tropical Medicine, University hospital, 38700, Grenoble, France.
    Purpose Of Review: Our purpose was to summarize the current knowledge about the neurological presentation of Zika virus infection after the perinatal period. Other Flaviviruses infections, such as West Nile virus (WNV) or Japanese encephalitis virus (JEV), can result in neuro-invasive disease such as myelitis, encephalitis, or meningitis. We aimed at describing the specificities of ZV neurological infection. Read More

    Pediatric Optic Neuritis: What Is New.
    J Neuroophthalmol 2017 Sep;37 Suppl 1:S14-S22
    The Vision Center (MB), Children's Hospital Los Angeles, Los Angeles, California; Departments of Ophthalmology and Neurology (MB), Keck School of Medicine, University of Southern California, Los Angeles, California; Neuro-ophthalmology Service (GTL), Division of Ophthalmology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Departments of Neurology and Ophthalmology (GTL), Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania; Department of Ophthalmology (SP), Stein Eye Institute, University of California, Los Angeles, California; Division of Neurology (ATW), Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; and Departments of Neurology and Pediatrics (ATW), Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
    Few diseases blur the margins between their childhood and adult-onset varieties as much as optic neuritis. This report will review our state of knowledge of pediatric optic neuritis, as well as its relationship to the latest consensus definitions of neuroinflammatory disease. Current diagnostic and treatment options will be explored, as well as our potential to uncover an understanding of pediatric optic neuritis through systematic prospective studies. Read More

    Prognostic relevance of MOG antibodies in children with an acquired demyelinating syndrome.
    Neurology 2017 Aug 2;89(9):900-908. Epub 2017 Aug 2.
    Author affiliations are provided at the end of the article.
    Objective: To assess the prognostic value of MOG antibodies (abs) in the differential diagnosis of acquired demyelinating syndromes (ADS).

    Methods: Clinical course, MRI, MOG-abs, AQP4-abs, and CSF cells and oligoclonal bands (OCB) in children with ADS and 24 months of follow-up were reviewed in this observational prospective multicenter hospital-based study.

    Results: Two hundred ten children with ADS were included and diagnosed with acute disseminated encephalomyelitis (ADEM) (n = 60), neuromyelitis optica spectrum disorder (NMOSD) (n = 12), clinically isolated syndrome (CIS) (n = 101), and multiple sclerosis (MS) (n = 37) after the first episode. Read More

    Assessment of acute motor deficit in the pediatric emergency room.
    J Pediatr (Rio J) 2017 Nov - Dec;93 Suppl 1:26-35. Epub 2017 Jul 27.
    Universidade Federal Fluminense (UFF), Hospital Universitário Antônio Pedro, Departamento Materno Infantil, Niterói, RJ, Brazil.
    Objectives: This review article aimed to present a clinical approach, emphasizing the diagnostic investigation, to children and adolescents who present in the emergency room with acute-onset muscle weakness.

    Sources: A systematic search was performed in PubMed database during April and May 2017, using the following search terms in various combinations: "acute," "weakness," "motor deficit," "flaccid paralysis," "child," "pediatric," and "emergency". The articles chosen for this review were published over the past ten years, from 1997 through 2017. Read More

    Cerebellar fits in the 2000s.
    Brain Dev 2018 Jan 19;40(1):77-80. Epub 2017 Jul 19.
    Neurophysiopathology Unit, Department of Neuroscience, Bambino Gesù Pediatric Hospital, Rome, Italy.
    Acute compression on the brainstem or acute increase in intracranial pressure may induce non-epileptic events varying from tonic seizures to axial rigidity with motor automatism, sometimes clearly characterized by decerebrate or decorticate paroxysmal posturing. The EEG correlate is characterized by diffuse asynchronous slow waves of variable amplitude. The mechanism behind such events, known as "cerebellar seizures or fits", is linked to cerebellar herniation and brainstem compression and is not of cortical origin. Read More

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