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Acute disseminating encephalomyelitis

Vanessa Couras Regadas, Márcio de Castro E Silva, Lucas Giansante Abud, Luiz Mario Pereira Lopes Labadessa, Rafael Gouvêa Gomes de Oliveira, Cecília Hissae Miyake, Rodolfo Mendes Queiroz
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...
January 2018: Revista da Associação Médica Brasileira
Hana Ayed, Mohammed W Chaudhary, Raidah AlBaradie, Ali Mir
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. Immunosuppressive agents like cyclophosphamide have been used in adults with fulminant acute disseminated encephalomyelitis. We report a case of a 3-year-old boy with fulminant acute disseminated encephalomyelitis. Minimal improvement was seen with high-dose intravenous methylprednisolone, intravenous immunoglobulin, and plasmapheresis...
2018: Child Neurology Open
L Monge Galindo, A L Martínez de Morentín, V Pueyo Royo, J P García Iñiguez, S Sánchez Marco, J López-Pisón, J L Peña-Segura
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...
March 8, 2018: Neurología: Publicación Oficial de la Sociedad Española de Neurología
Raed Alroughani, Alexey Boyko
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. METHODS: The authors performed a literature search and reviewed the current understanding on risk factors and disease parameters in order to discuss the challenges in assessing and implementing diagnosis and therapy in clinical practice...
March 9, 2018: BMC Neurology
Michael R Pranzatelli, Tyler J Allison, Nathan R McGee, Elizabeth D Tate
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)...
February 27, 2018: Clinical and Experimental Immunology
Shinji Fukui, Yusuke Iemura, Yoshiaki Matsumura, Yoriaki Kagebayashi, Yuki Hotta, Sayaka Yoshida, Shoji Samma
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. A cerebrospinal fluid examination showed the elevation of cell number (cell number : 158/3, polynuclear cells : 29/3, and mononuclear cells : 129/3). Although spinal magnetic resonance imaging (MRI) did not show abnormal findings, fluid attenuated inversion recovery (FLAIR) image of the brain MRI showed a high signal area on the cerebral cortex...
January 2018: Hinyokika Kiyo. Acta Urologica Japonica
Sophie Duignan, Sukhvir Wright, Tom Rossor, John Cazabon, Kimberly Gilmour, Olga Ciccarelli, Evangeline Wassmer, Ming Lim, Cheryl Hemingway, Yael Hacohen
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...
February 22, 2018: Developmental Medicine and Child Neurology
Babikir Kheiri, Emad Abu Sitta, Anas Salih, Mohammed Al Qasmi, Ghassan Bachuwa
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.
February 2018: Clinical Case Reports
Y Y M Wong, Y Hacohen, T Armangue, E Wassmer, H Verhelst, C Hemingway, E D van Pelt, C E Catsman-Berrevoets, R Q Hintzen, K Deiva, M J Lim, K Rostásy, R F Neuteboom
BACKGROUND: Acute disseminated encephalomyelitis, followed by optic neuritis (ADEM-ON) is a rare demyelinating syndrome different than MS and neuromyelitis optica spectrum disorder. We aim to describe the disease course, treatment response and outcome of these children. METHODS: children <18 years were identified from 6 countries of the EU Paediatric Demyelinating Disease Consortium. Patients fulfilled the diagnostic criteria for ADEM, followed by at least one ON...
February 14, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Matthias Baumann, Astrid Grams, Tanja Djurdjevic, Eva-Maria Wendel, Christian Lechner, Bettina Behring, Astrid Blaschek, Katharina Diepold, Astrid Eisenkölbl, Joel Fluss, Michael Karenfort, Johannes Koch, Bahadir Konuşkan, Steffen Leiz, Andreas Merkenschlager, Daniela Pohl, Mareike Schimmel, Charlotte Thiels, Barbara Kornek, Kathrin Schanda, Markus Reindl, Kevin Rostásy
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. Children with acute disseminated encephalomyelitis (n = 36) or neuromyelitis optica spectrum disorder (n = 5) presented an imaging pattern characterized predominantly by poorly demarcated lesions with a wide supra- and infratentorial distribution...
February 8, 2018: Journal of Neurology
Lidia Stork, David Ellenberger, Tim Beißbarth, Tim Friede, Claudia F Lucchinetti, Wolfgang Brück, Imke Metz
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. Pattern 3 lesions show signs of oligodendrocyte degeneration...
February 5, 2018: JAMA Neurology
Yael Hacohen, Yu Yi Wong, Christian Lechner, Maciej Jurynczyk, Sukhvir Wright, Bahadir Konuskan, Judith Kalser, Anne Lise Poulat, Helene Maurey, Esther Ganelin-Cohen, Evangeline Wassmer, Chery Hemingway, Rob Forsyth, Eva Maria Hennes, M Isabel Leite, Olga Ciccarelli, Banu Anlar, Rogier Hintzen, Romain Marignier, Jacqueline Palace, Matthias Baumann, Kevin Rostásy, Rinze Neuteboom, Kumaran Deiva, Ming Lim
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. Design, Setting, and Participants: This study prospectively collected demographic, clinical, and radiologic data from 102 patients from 8 countries of the EU Paediatric Demyelinating Disease Consortium from January 1, 2014, through December 31, 2016...
January 5, 2018: JAMA Neurology
Yael Hacohen, Thomas Rossor, Kshitij Mankad, Wk 'Kling' Chong, Andrew Lux, Evangeline Wassmer, Ming Lim, Frederik Barkhof, Olga Ciccarelli, Cheryl Hemingway
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 4.1y) than the remainder (mean 8...
December 30, 2017: Developmental Medicine and Child Neurology
Lorna Stemberger Maric, Snjezana Zidovec Lepej, Lana Gorenec, Ivana Grgic, Vladimir Trkulja, Oktavija Dakovic Rode, Srdan Roglic, Tonci Grmoja, Nina Barisic, Goran Tesovic
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...
December 29, 2017: Neurological Sciences
Sikawat Thanaviratananich, Bashar Katirji
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. MRI brain revealed multiple hyperintense lesions confined to only the right hemisphere with contrast enhancement, involving both white and grey matters...
June 2017: ENeurologicalSci
Jin Kyu Kim, Seung-A Han, Sun Jun Kim
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. PATIENT CONCERNS: A previously healthy 11-year-old boy was admitted for a sudden transient weakness of his left side extremities...
December 2017: Medicine (Baltimore)
Hamed Ahmed Elkhayat, Omnia Fathy El-Rashidy, Iman Ali Elagouza, Raghda Zaitoun, Yasser Abdel Azeem Abbas
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 4.4 years ± 2.7 SD, were included. All new patients were subject to full history, examination and a panel of investigations including MRI of the brain...
December 8, 2017: Acta Neurologica Belgica
Caroline Malo-Pion, Raymond Lambert, Jean-Claude Décarie, Sophie Turpin
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. We present two such cases.
February 2018: Clinical Nuclear Medicine
Carla Schettino, Ferdinando Caranci, Giacomo Lus, Elisabetta Signoriello, Marica Eoli, Elena Anghileri, Bianca Pollo, Mariarosa A B Melone, Giuseppe Di Iorio, Gaetano Finocchiaro, Lorenzo Ugga, Enrico Tedeschi
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...
October 2017: Quantitative Imaging in Medicine and Surgery
Wint Yan Aung, Parinaz Massoumzadeh, Safa Najmi, Amber Salter, Jodi Heaps, Tammie L S Benzinger, Soe Mar
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. Therefore, we intend to explore neuroimaging biomarkers that can reliably distinguish between the two diagnoses. METHODS: We reviewed prospectively collected clinical, standard MRI and diffusion tensor imaging data of 12 pediatric patients who presented with acute demyelination with and without encephalopathy...
September 28, 2017: Pediatric Neurology
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