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Acute disseminated encephalomyelitis 

Cynthia Wang, Ram Narayan, Benjamin Greenberg
BACKGROUND: Anti-myelin oligodendrocyte glycoprotein (MOG) antibody associated disorders frequently manifest as optic neuritis, transverse myelitis, and acute disseminated encephalomyelitis. While their clinical phenotypes overlap with relapsing inflammatory Central nervous system (CNS) conditions such as multiple sclerosis and neuromyelitis optica spectrum disorder, MOG-related syndromes frequently occur in a younger age group. In children, longitudinally extensive transverse myelitis (LETM) is less specific for anti-aquaporin-4 associated neuromyelitis optica spectrum disorder, and has also been reported in pediatric multiple sclerosis, idiopathic transverse myelitis, and acute flaccid myelitis...
July 10, 2018: Pediatric Neurology
Ryuji Sakakibara
PURPOSE: Multiple sclerosis (MS), neuromyelitis optica (NMO), and related disorders are major immune-mediated central nervous system diseases affecting the spinal cord. We reviewed the occurrence of neurogenic lower urinary tract dysfunction (NLUTD) in these categories of diseases. METHODS: We systematically reviewed the literature regarding bladder dysfunction in MS, NMO spectrum disorder (NMOSD), and related disorders (acute disseminated encephalomyelitis, acute immune-mediated myelopathy, and meningitis-retention syndrome)...
August 3, 2018: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
Manuela de Oliveira Fragomeni, Denis Bernardi Bichuetti, Enedina Maria Lobato Oliveira
BACKGROUND: Multiple Sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD) are acquired demyelinating syndromes of the central nervous system more frequently in young adults and their beginning before 18 years of age is rare. They are autoimmune diseases with distinct pathophysiology, clinical presentation, treatment and prognoses. During childhood these conditions often present similar clinical features and differential diagnosis among pNMOSD, pMS and acute disseminated encephalomyelitis (ADEM) is still difficult at disease onset...
July 20, 2018: Multiple Sclerosis and related Disorders
Nicolas Desse, Aurore Sellier, Cédric Bernard, Arnaud Dagain
We present the case of a patient who died of a fatal meningoencephalitis after removal of a third ventricle colloid cyst. Postoperative clinical and iconographic evolution let us think about an acute disseminated encephalomyelitis probably due to cerebrospinal fluid contamination by inflammatory proteins contained in the colloid cyst. This case raises the question of a possibility of colloid cyst content spraying while using an ultrasonic aspiration device.
July 29, 2018: Acta Neurochirurgica
Magnus Spangsberg Boesen, Poul Erik Hyldgaard Jensen, Melinda Magyari, Alfred Peter Born, Peter Vilhelm Uldall, Morten Blinkenberg, Finn Sellebjerg
BACKGROUND: Chitinase 3-like 1 (CHI3L1), neurofilament light chain (NFL) and oligoclonal bands (OCB) in cerebrospinal fluid are associated with central nervous system demyelination in adults. CHI3L1 and OCB are markers of central nervous system inflammation, whereas NFL is a marker of white-matter axonal injury. The aim was to examine whether CHI3L1 and NFL in cerebrospinal fluid are associated with acquired demyelinating syndromes at disease onset in a pediatric population. METHODS: Children (<18 years) referred to hospital for possible neuroinflammatory disease were retrospectively included from 2010 to 2016...
June 11, 2018: Multiple Sclerosis and related Disorders
G Balloy, J Pelletier, L Suchet, C Lebrun, M Cohen, P Vermersch, H Zephir, E Duhin, O Gout, R Deschamps, E Le Page, G Edan, P Labauge, C Carra-Dallieres, L Rumbach, E Berger, P Lejeune, P Devos, J-B N'Kendjuo, M Coustans, E Auffray-Calvier, B Daumas-Duport, L Michel, F Lefrere, D A Laplaud, C Brosset, P Derkinderen, J de Seze, S Wiertlewski
BACKGROUND: Tumefactive demyelinating lesions of the central nervous system can be the initial presentation in various pathological entities [multiple sclerosis (the most common), Balo's concentric sclerosis, Schilder's disease and acute disseminated encephalomyelitis] with overlapping clinical presentation. The aim of our study was to better characterize these patients. METHODS: Eighty-seven patients (62 women and 25 men) from different MS centers in France were studied retrospectively...
July 27, 2018: Journal of Neurology
Hussein Algahtani, Ahmed Absi, Wafaa Bassuni, Bader Shirah
Familial hemophagocytic lymphohistiocytosis (HLH) is a rare autosomal recessive life-threatening multisystem inflammatory disorder. It is characterized by excessive production of cytokines and uncontrolled activation of lymphocytes and macrophages leading to widespread organ infiltration and tissue destruction. Central nervous system involvement is common occurring in approximately 75% of patients. The neurological symptoms often develop during the course of the disease. However, they can be the initial presenting manifestation...
July 19, 2018: Multiple Sclerosis and related Disorders
Liaqat Ali Chaudhry, Waseem Babur, Ghazala Aslam Chaudhry, Feddah Eid Al-Atawi, Asirvatham Alwin Robert
Acute disseminated encephalomyelitis is a rare disease of central nervous system, which can present with a variety of clinical manifestations. That is why first attack of ADEM, in particular remains a diagnostic puzzle. Early anticipation and diagnosis is important for better outcomes. We present a case of acute disseminated encephalomyelitis which initially had atypical clinical features with cough, expectoration, fever and later manifested strange neurological features, diagnosed to be a case of acute disseminated encephalomyelitis based on radio-imaging...
2018: Pan African Medical Journal
Honglu Song, Huanfen Zhou, Mo Yang, Shaoying Tan, Junqing Wang, Quangang Xu, Hongjuan Liu, Shihui Wei
BACKGROUND/AIMS: To investigate clinical characteristics and prognosis of paediatric optic neuritis (PON) in patients seropositive for myelin oligodendrocyte glycoprotein antibody (MOG-Ab) in China. METHODS: Children displaying initial onset of optic neuritis (ON) were recruited from the Neuro-ophthalmology Department in the Chinese People's Liberation Army General Hospital from January 2016 to August 2017. They were assigned into three groups based on antibody status: MOG-Ab-seropositive ON (MOG-ON), aquaporin-4 antibody-seropositive ON (AQP4-ON) and double seronegative ON (seronegative-ON)...
July 26, 2018: British Journal of Ophthalmology
Ram Narayan, Alexandra Simpson, Katelyn Fritsche, Sara Salama, Santiago Pardo, Maureen Mealy, Friedemann Paul, Michael Levy
MOG antibody disease is an autoimmune disease of the central nervous system associated with a serological antibody against MOG, myelin oligodendrocyte glycoprotein. MOG is a glycoprotein expressed on the outer membrane of myelin and solely found within the central nervous system, including in the brain, optic nerves and spinal cord. Clinically, the disease resembles neuromyelitis optica spectrum disorders in the predilection for relapses of optic neuritis and transverse myelitis. In addition, acute disseminated encephalomyelitis (ADEM) is a well-recognized phenotype of MOG antibody disease in children...
July 23, 2018: Multiple Sclerosis and related Disorders
Kelli L Barr, Erum Khan, Joveria Q Farooqi, Kehkashan Imtiaz, Dhani Prakoso, Faisal Malik, John A Lednicky, Maureen T Long
Several arboviruses are endemic to and co-circulate in Pakistan. In recent years, Pakistan has observed a rise in arboviral infections. A cross-sectional study for arboviral diseases, which included screening for Chikungunya virus (CHIKV), was initiated in 2015 to determine which pathogens were causing disease in patients presenting to health care services. Exposure to CHIKV was verified via detection of viral nucleic acids or virus-specific IgM with virus-specific neutralizing antibodies. Out of 997 enrolled patients presenting with clinical features suggestive of arboviral disease, 102 patients were positive for CHIKV IgM antibodies and 60 patients were positive for CHIKV nucleic acids or neutralizing antibodies...
2018: Frontiers in Public Health
A Sebastian López-Chiriboga, Masoud Majed, James Fryer, Divyanshu Dubey, Andrew McKeon, Eoin P Flanagan, Jiraporn Jitprapaikulsan, Naga Kothapalli, Jan-Mendelt Tillema, John Chen, Brian Weinshenker, Dean Wingerchuk, Jessica Sagen, Avi Gadoth, Vanda A Lennon, B Mark Keegan, Claudia Lucchinetti, Sean J Pittock
Importance: Recent studies have reported a higher relapse rate following an initial inflammatory demyelinating disorder in pediatric patients with persistent seropositivity of antibodies targeting myelin oligodendrocyte glycoprotein (MOG-IgG1). To date, the clinical implications of longitudinal MOG-IgG1 seropositivity using live cell assays with IgG1 secondary antibodies in adults after acute disseminated encephalomyelitis (ADEM) are unknown. Objective: To determine whether MOG-IgG1 serostatus (transient vs persistent) and titer change over time provide clinical utility in predicting the likelihood of relapse after ADEM...
July 16, 2018: JAMA Neurology
Lu Chen, Chen Chen, Xiaonan Zhong, Xiaobo Sun, Haixia Zhu, Xiaojing Li, Hui Yang, Yaqing Shu, Yanyu Chang, Xueqiang Hu, Zhengqi Lu, Lisheng Peng, Wei Qiu
BACKGROUND: Immunoglobulin against myelin oligodendrocyte glycoprotein (MOG-IgG) is a potential demyelinating disease-associated autoantibody. Whether clinical features of MOG antibody-associated demyelinating diseases change with age remains unclear. OBJECT: To investigate the different clinical features between pediatric-onset and adult-onset MOG-IgG-seropositive patients in a relatively large cohort. METHODS: A total of 816 consecutive patients with suspected demyelinating disease were prospectively enrolled from three tertiary academic centers in South China from February 2016 to December 2016...
August 15, 2018: Journal of Neuroimmunology
Alla Laila, Rania M El-Lababidi, Mohamed Hisham, Mohammad Mooty
We report a case of acute disseminated encephalomyelitis (ADEM) secondary to Mycoplasma pneumoniae infection that failed to improve with methylprednisolone and intravenous immunoglobulin (IVIG); who responded with plasmapheresis. A 21- year- old female with an unremarkable medical history, initially presented to an outside hospital with fever and an influenza-like illness and was subsequently intubated for worsening sensorium. Brain magnetic resonance imaging was suggestive of ADEM or vasculitis for which she received five days of pulse steroids and IVIG...
2018: IDCases
Todd A Hardy
No abstract text is available yet for this article.
June 21, 2018: Developmental Medicine and Child Neurology
Varsha H Chauhan, Richa Chaudhary, Payal Meshram
Acute disseminated encephalomyelitis (ADEM) is an acute monophasic syndrome caused by immune-mediated inflammatory demyelination, often associated with immunization or viral illness. ADEM is associated with multiple neurological symptoms. We are presenting a case of ADEM with stroke, which responded very well to high-dose steroids. Here we report a case of ADEM, masquerading as pediatric stroke.
January 2018: Journal of Pediatric Neurosciences
Jian Zhang, Jun Lin, Ye Tian, Linlin Ma, Wen Sun, Lei Zhang, Yichen Zhu, Wei Qiu, Lujia Zhang
BACKGROUND: Due to the increasing number of DCD transplantations since 2015, the transmission of rabies through solid organ transplantation has become a notable problem in China and has attracted the attention of the public. CASE PRESENTATION: From 2015 to 2017, four solid organ recipients in our centre were successively diagnosed with rabies that was considered to have been transmitted from two donors who died due to viral encephalitis of unknown cause and acute disseminated encephalomyelitis...
June 14, 2018: BMC Infectious Diseases
Poorani Anandakrishnan, Teik Beng Khoo
Cerebral demyelination and optic neuritis are often seen in children with acute disseminated encephalomyelitis following various infections and immunisations. An eight month old girl presented with a left axillary lymph node swelling and an erythematous lace-like rash over her cheeks and trunk. She then developed acute encephalopathy, bilateral nystagmus, right hemiparesis and left facial nerve palsy. Her electroencephalogram showed an encephalopathic process and visual evoked response study were grossly abnormal...
May 30, 2018: BMJ Case Reports
F Brilot
No abstract text is available yet for this article.
August 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Yong Chen, Fubao Ma, Yuanling Xu, Xuhua Chu, Jinlin Zhang
BACKGROUND: It is important to examine the risk of Acute disseminated encephalomyelitis (ADEM) after vaccination. METHODS: We conducted a nested case-control study between January 2011 and December 2015. Four controls per case were matched for age, gender, address. An independent expert committee validated the diagnoses of cases and controls. Data on vaccinations were obtained from computerized vaccination records. The analyses were conducted with the use of conditional logistic regression...
June 18, 2018: Vaccine
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