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Acute disseminated encephalomyelitis 

Yanjun Liu, Xiangqi Tang
Autoimmune diseases of the nervous system (ADNS) consist of a group of severely disabling disorders characterized by abnormal immune attack against protein components of the nervous system. This type of attack behavior may occur in the central or peripheral nervous system, and in the neuromuscular junction, resulting in neuronal damage, axonal injury, demyelination or destruction of the neuromuscular junction. While the neurological deficits of patients with ADNS have received significant research attention, the manifestation of depression tends to be ignored...
2018: Frontiers in Psychiatry
Megan Barry, Truc M Le, Melissa C Gindville, Lori C Jordan
BACKGROUND: Pediatric stroke alerts or "code strokes" allow for rapid evaluation, imaging, and treatment of children presenting with stroke-like symptoms. In a previous study of emergency department-initiated pediatric stroke alerts, 24% of children had confirmed strokes. The purpose of this study was to characterize in-hospital pediatric stroke alerts. METHODS: Demographic and clinical information was obtained from a quality improvement database and medical records for children (zero to 20 years) at a single institution for whom a stroke alert was activated after hospital admission between April 2011 and December 2016...
August 10, 2018: Pediatric Neurology
Ram N Narayan
OBJECTIVE: To describe 2 atypical cases with Anti-MOG antibody related demyelinating syndrome. METHODOLOGY: Case series. RESULTS: We present two cases. Case 1 is an 18-year-old woman who presented with headache, blurred vision, and papilledema and was initially diagnosed with pseudotumor cerebri syndrome. CSF showed mildly elevated opening pressure and lymphocytic pleocytosis and a diagnosis of aseptic meningitis was considered. MRI brain and spinal cord revealed longitudinally extensive bilateral simultaneous optic neuritis and multiple spinal cord lesions...
October 3, 2018: Multiple Sclerosis and related Disorders
Oscar Mancera-Páez, Gustavo C Román, Rodrigo Pardo-Turriago, Yhojan Rodríguez, Juan-Manuel Anaya
We review post-infectious and post-vaccination neurological syndromes involving peripheral and central nervous system (CNS) and report an illustrative case of simultaneous occurrence of Guillain-Barré syndrome (GBS), confirmed by nerve conduction velocities, plus MRI-demonstrated transverse myelitis (TM) and acute encephalitis [acute disseminated encephalomyelitis] (ADEM+GBS) affecting a 24-year-old woman from Cúcuta, Colombia, who developed acute Zika virus (ZIKV) infection confirmed by serum reverse transcriptase-polymerase chain reaction (RT-PCR) and convalescent ZIKV IgG antibodies...
September 26, 2018: Journal of the Neurological Sciences
Eglė Sakalauskaitė-Juodeikienė, Giedrė Armalienė, Rasa Kizlaitienė, Loreta Bagdonaitė, Nataša Giedraitienė, Dalia Mickevičienė, Daiva Rastenytė, Gintaras Kaubrys, Dalius Jatužis
OBJECTIVES: Neuromyelitis optica (NMO) is frequently associated with aquaporin-4 autoantibodies (AQP4-Ab); however, studies of NMO in Lithuania are lacking. Therefore, the main objective of our study is to assess positivity for AQP4-Ab in patients presenting with inflammatory demyelinating central nervous system (CNS) diseases other than typical multiple sclerosis (MS) in Lithuania. MATERIALS AND METHODS: Data were collected from the two largest University hospitals in Lithuania...
October 3, 2018: Brain and Behavior
H Assadeck, M Toudou Daouda, É Adehossi Omar, Z Mamadou, F Hassane Djibo, D Douma Maiga
BACKGROUND: In sub-Saharan Africa (SSA), few studies have been reported on inflammatory demyelinating diseases of the central nervous system (CNS). Neuromyelitis optica spectrum disorders (NMOSD) seems to be the most frequent inflammatory demyelinating disease of CNS in sub-Saharan Africans or people of sub-Saharan African descent. METHODS: We report the observations of seven patients from Niger diagnosed with inflammatory demyelinating diseases of CNS over a period of 21 years (1996-2017)...
September 27, 2018: Revue Neurologique
S Jarius, F Paul, O Aktas, N Asgari, R C Dale, J de Seze, D Franciotta, K Fujihara, A Jacob, H J Kim, I Kleiter, T Kümpfel, M Levy, J Palace, K Ruprecht, A Saiz, C Trebst, B G Weinshenker, B Wildemann
Over the past few years, new-generation cell-based assays have demonstrated a robust association of autoantibodies to full-length human myelin oligodendrocyte glycoprotein (MOG-IgG) with (mostly recurrent) optic neuritis, myelitis and brainstem encephalitis, as well as with acute disseminated encephalomyelitis (ADEM)-like presentations. Most experts now consider MOG-IgG-associated encephalomyelitis (MOG-EM) a disease entity in its own right, immunopathogenetically distinct from both classic multiple sclerosis (MS) and aquaporin-4 (AQP4)-IgG-positive neuromyelitis optica spectrum disorders (NMOSD)...
September 27, 2018: Der Nervenarzt
Etsuro Nanishi, Takayuki Hoshina, Masafumi Sanefuji, Ryo Kadoya, Katsuhiko Kitazawa, Yukie Arahata, Tetsuya Sato, Yoshimichi Hirayama, Katsuki Hirai, Masaaki Yanai, Kaori Nikaido, Akihiko Maeda, Hiroyuki Torisu, Kenji Okada, Yasunari Sakai, Shouichi Ohga
Background: Japanese encephalitis (JE) is the leading cause of viral encephalitis with high mortality and morbidity in Asia. In Japan, however, the active recommendation of JE vaccine was retracted in 2005 because of the potential risk of acute disseminated encephalomyelitis. We aimed to determine the recent incidence of childhood-onset JE after the domestic change of vaccination policy in Japan, and to analyze the clinical features of affected children. Methods: A retrospective nationwide survey was conducted for pediatric patients with JE in Japan from 1995 to 2015...
September 24, 2018: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
Suma Shah, Deepal Shah, Mark B Skeen
BACKGROUND: Acute disseminated encephalomyelitis has been understood as a monophasic, often post-infectious illness that predominantly affects the pediatric population. Though that describes the majority of cases, exceptions do exist. In this case report, we present an adult case of recurrent disseminated encephalomyelitis (DEM) and review the available literature on this clinical entity. METHODS: PubMed search performed using the terms "MDEM" and "Recurrent ADEM" in April 2018...
September 11, 2018: Multiple Sclerosis and related Disorders
Abdul Ahad E Sheikh, Abu Baker Sheikh, Shazib Sagheer, Usman Tariq, Marvi M Bukhari, Zainab Fatima, Rao M Afzal
Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system (CNS) with no distinct etiology but implications include infections and commonly administered vaccinations. In this case report, we present the case of ADEM in a young female who was subsequently diagnosed with acute intermittent porphyria (AIP) that was the instigator of the initial CNS assault. Our case highlights the peculiar presentation of ADEM which can present as a diagnostic challenge and brings forth AIP as a new and previously unknown affiliate of this rare CNS disease...
July 17, 2018: Curēus
María Estela Cadario, Alejandro Ellis, Mónica Garea, Antonio Cairnie, Alicia Mistchenko, Cristian García Roig, María Cecilia Freire, Vilma Savy, Juan Antonio Sciarrotta
We present here the case of a previously healthy 5 year-old boy hospitalized in an intensive care unit due to tonic-clonic seizures focused on the face and right side of the body, and axillary temperature of 37.4°C. Common bacterial and viral etiology was ruled out through studies of cerebrospinal fluid (CSF) samples. Mycoplasma pneumoniae was suspected by a positive immunofluorescence serum test for IgM class antibodies. Finally, with a brain biopsy, M. pneumoniae was confirmed by polymerase chain reaction (PCR) and acute disseminated encephalomyelitis by pathological anatomy...
September 17, 2018: Revista Argentina de Microbiología
Satinder Aneja, Suvasini Sharma
Acute encephalitis is a common cause of death and neurodevelopmental problems in children. The causative agents are numerous including infectious agents such as viruses, bacteria, mycobacteria and protozoa; para-infectious and immune mediated disorders such as acute disseminated encephalomyelitis (ADEM) and autoimmune encephalitis, especially the recently described anti-NMDA receptor encephalitis. Also, many viral associated encephalopathies such as acute necrotizing encephalopathy can mimic the presentation of acute encephalitis...
September 19, 2018: Indian Journal of Pediatrics
Scott Otallah, Brenda Banwell
PURPOSE OF REVIEW: Diagnostic criteria for pediatric-onset multiple sclerosis (POMS) and related demyelinating disorders have been updated, neuroimaging studies have revealed new insights, biological assays identify patients with specific antibodies that influence both diagnosis and treatment, clinical trials are informing on treatment efficacy and safety, and longitudinal studies of neurological, cognitive and quality of life outcomes are informing on the impact of these diseases. We provide updates to assist providers caring for these children...
September 18, 2018: Current Neurology and Neuroscience Reports
Soniza Vieira Alves-Leon, Monique da Rocha Lima, Priscila Conrado Guerra Nunes, Leila Maria Cardão Chimelli, Kíssila Rabelo, Rita Maria Ribeiro Nogueira, Fernanda de Bruycker-Nogueira, Elzinandes Leal de Azeredo, Paulo Roberto Bahia, Fernanda Cristina Rueda Lopes, Jorge P Barreto Marcondes de Souza, Fabrícia Lima Fontes-Dantas, Marciano Viana Paes, Elba Rs Lemos, Flavia Barreto Santos
BACKGROUND: A range of different neurological manifestations has been reported in fetuses and adults after Zika virus (ZIKV) infection. OBJECTIVE: We describe a detection of the ZIKV in the brain tissue from a multiple sclerosis (MS) patient with acute disseminated encephalomyelitis (ADEM)-like event in Rio de Janeiro, Brazil. METHODS: Biological samples collected during the hospitalization were tested by serology and molecular diagnostic for various infectious agents...
June 1, 2018: Multiple Sclerosis: Clinical and Laboratory Research
Sudarshini Ramanathan, Gina L O'grady, Stephen Malone, Claire G Spooner, David A Brown, Deepak Gill, Fabienne Brilot, Russell C Dale
Myelin oligodendrocyte glycoprotein (MOG) antibodies have a strong association with acute disseminated encephalomyelitis (ADEM) in children, and bilateral and recurrent optic neuritis in children and adults. Recent reports suggest that seizures and encephalopathy may occur in children and adults with MOG antibody-associated disease. We describe the clinical, laboratory, and radiological course of four MOG antibody-positive children who first presented with isolated seizures without fulfilling clinical or radiological criteria for ADEM or other central nervous system demyelination syndromes, who months to years later developed more typical demyelination...
September 17, 2018: Developmental Medicine and Child Neurology
Bruce D Trapp, Daniel Ontaneda
Dr Bruce Trapp and Dr Daniel Ontaneda speak to Laura Dormer, Commissioning Editor: Bruce D Trapp, PhD, is a Chair of the Department of Neurosciences at the Lerner Research Institute, Cleveland Clinic (OH, USA) and Professor of Molecular Medicine at Case Western Reserve University (OH, USA). Dr Trapp received his PhD from Loyola University Stritch School of Medicine in Chicago (IL, USA). Dr Trapp's research investigates the cause of neurological disability in multiple sclerosis patients, cellular mechanism of brain repair in neurodegenerative diseases and the molecular biology of myelination in the central and peripheral nervous systems...
September 14, 2018: Neurodegenerative Disease Management
Bahadır Konuskan, Mirac Yildirim, Rahsan Gocmen, Tuncay Derya Okur, Ipek Polat, Huseyin Kilic, Sema Saltik, Zeynep Ozturk, Kivilcim Gucuyener, Sakir Altunbasak, Tamer Celik, Gulsen Kose, Arzu Yilmaz, Mustafa Komur, Hulya Kayilioglu, Banu Anlar
BACKGROUND: Knowledge has been expanding on myelin oligodendrocyte glycoprotein (MOG) antibody-associated central nervous system disorders. We delineate the clinical and paraclinical findings and outcome of our pediatric patients with MOG antibody seropositive disease. METHODS: We retrospectively analyzed the clinical presentation, cerebrospinal fluid findings, magnetic resonance imaging (MRI) studies, course and outcome of children seropositive for anti-MOG IgG...
September 10, 2018: Multiple Sclerosis and related Disorders
Andrea Savransky
Demyelinating diseases are a group of conditions of autoimmune etiology directed against the myelin of the central nervous system. In many cases, the onset of the illness is preceded by a nonspecific viral infection. Multiple sclerosis is a disease that evolves with relapses and remissions with polyfocal neurological deficits, being the most frequent optic neuritis, transverse myelitis and encephalic trunk involvement. Typically, magnetic resonance image (MRI) shows peri-ventricular, peri-callosal, cerebellum, brain stem and spinal cord hyperintensive lesions in T2 and FLAIR weighted images...
2018: Medicina
Giulia Fadda, Robert A Brown, Giulia Longoni, Denise A Castro, Julia O'Mahony, Leonard H Verhey, Helen M Branson, Patrick Waters, Amit Bar-Or, Ruth Ann Marrie, E Ann Yeh, Sridar Narayanan, Douglas L Arnold, Brenda Banwell
BACKGROUND: MRI and laboratory features have been incorporated into international diagnostic criteria for multiple sclerosis. We assessed the pattern of MRI lesions and contributions of cerebrospinal fluid (CSF) and serum antibody findings that best identifies children with multiple sclerosis, and the applicability of international diagnostic criteria in the paediatric context. METHODS: In this prospective cohort study, detailed clinical assessments, serum and CSF studies, and MRI scans were done in youth (aged 0·46-17·87 years) with incidental acquired demyelinating syndrome...
March 2018: Lancet Child & Adolescent Health
I S Bakulin, N I Stoyda, L Sh Askarova, R N Konovalov, P R Prokazova, A A Bormin, R V Polishchuk, Yu V Ryabinkina, P L Anufriev, T S Gulevskaya, M N Zakharova
Primary central nervous system lymphoma (PCNSL) is a rare aggressive extranodal non-Hodgkin lymphoma. Difficulties in diagnosing PCNSL are associated with the absence of pathognomonic clinical and neuroimaging findings of this disease. The article describes the clinical case of a female patient with autopsy-confirmed multifocal large-cell B-cell CNS lymphoma misdiagnosed as acute disseminated encephalomyelitis (ADEM). Clinical and neuroimaging characteristics of PCNSL and ADEM as well as the role of diagnostic methods in establishing the correct diagnosis are discussed...
2018: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
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