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Acute disseminated encephalomyelitis 

João Melo Alves, Inês Brás Marques, Raquel Gil-Gouveia
Acute disseminated encephalomyelitis is a rare inflammatory demyelinating multifocal disease of the central nervous system that typically occurs in children following vaccination or exanthematous viral infections and conveys an elevated risk of neurological sequelae unless promptly recognized and treated. We describe an adult case of acute disseminated encephalomyelitis following vaccination against Mumps, Measles and Rubella, presenting with fever and progressive neurological deficits which improved under systemic corticosteroid therapy...
February 1, 2019: Acta Médica Portuguesa
Benjamin M Greenberg, Chitra Krishnan, Lana Harder
IMPORTANCE: Patients afflicted with rare diseases often have a delay in diagnosis and treatment. Understanding the prevalence and impact of delayed diagnosis in transverse myelitis could trigger directed educational initiatives to increase clinician awareness and improve care. OBJECTIVE: To determine if symptoms at onset or care provider initially approached was associated with time to diagnosis, treatment or outcome in patients with transverse myelitis. DESIGN: This was an online patient and caregiver standardized survey to collect data about the initial medical experience...
January 31, 2019: Multiple Sclerosis and related Disorders
Yu Yi M Wong, C Louk de Mol, Roos M van der Vuurst de Vries, E Daniëlle van Pelt, Immy A Ketelslegers, Coriene E Catsman-Berrevoets, Rinze F Neuteboom, Rogier Q Hintzen
Objective: To compare the diagnostic accuracy of the McDonald 2017 vs the McDonald 2010 criteria to predict a second attack of MS (clinically definite MS [CDMS]) at the first attack of acquired demyelinating syndromes (ADS). Methods: One hundred sixty-four children (aged <18 years) with an incident attack of ADS were included in a prospective multicenter study between June 2006 and December 2016. Brain (and spinal if available) MRI was performed ≤3 months after symptom onset...
March 2019: Neurology® Neuroimmunology & Neuroinflammation
Saravanan Margabandhu, Suchitra Ranjit, Indira Jayakumar, Chitra Sundaramoorthy, Mahesh Janarthanan, Jayakumar Reddy, Meena Thiagarajan, Shyamala Jayamoorthy, Latha Vishwanathan
INTRODUCTION: Outcome data in pediatric plasma exchange, especially in nonrenal indications are scarce. We aimed to evaluate its role and outcome in our patients. SUBJECTS AND METHODS: A retrospective study of children admitted in the year 2016 to the Pediatric Intensive Care Unit requiring plasma exchange for nonrenal indications was undertaken. Plasma exchange was given as adjunctive therapy along with primary treatment for the disease concerned. Demographic and clinical data were studied, and descriptive statistical analysis was carried out...
July 2018: Asian Journal of Transfusion Science
Hiroyuki Torisu, Kenji Okada
While the basic definition of vaccination-associated acute disseminated encephalomyelitis (ADEM) is relatively clear and easily understandable, it is often difficult to diagnose ADEM based on clinical findings alone. ADEM is actually a heterogeneous clinical syndrome that can be approximately characterized by encephalomyelitis with multiple inflammatory demyelination, autoimmune causes, and relationship with a preceding infection or vaccination. The differential diagnosis of ADEM should exclude the possibility of infectious or other autoimmune encephalitis...
January 22, 2019: Vaccine
Kuljeet Singh Anand, Arun Kumar Agrawal, Jyoti Garg, Rajinder K Dhamija, Rakesh Kumar Mahajan
Chikungunya (CHIK) has re-emerged as a potential neurotropic virus, with outbreaks recently being reported from many parts of India. The present study was conducted to study the spectrum and outcome of neurological complications in patients of CHIK during the 2016 outbreak in Delhi. A total of 42 cases seropositive for IgM CHIK antibodies by MAC-ELISA and developing neurological complications were enrolled. The male:female ratio was 1:2 (age range = 18-90 years). The neurological manifestations observed were encephalitis (n = 12), bulbar palsy (n = 3), acute disseminated encephalomyelitis (n = 1), cerebellitis (n = 1), myelopathy (n = 1), radiculoneuropathy (n = 3), carpal tunnel syndrome (n = 9) and tremors (n = 1)...
January 24, 2019: Tropical Doctor
Yusuke Kawanaka, Kumiko Ando, Reiichi Ishikura, Takayuki Katsuura, Yuki Wakata, Hiroshi Kodama, Haruyuki Takaki, Yoshihiro Takada, Junichiro Ono, Koichiro Yamakado
PURPOSE: To evaluate the frequency, characteristics, and clinical significance of transient hyperintensity foci on T1-weighted images (T1WI) in acute disseminated encephalomyelitis (ADEM). MATERIALS AND METHODS: Patients diagnosed with ADEM underwent MR studies at the time of disease onset and every 3 months or more often thereafter. The frequency and appearance timing of abnormal signals including T1WI and their morphological characteristics were evaluated. Relations between patient symptoms and abnormal signals on MRI were also evaluated...
January 22, 2019: Japanese Journal of Radiology
Jolene R Bowers, Michael Valentine, Veronica Harrison, Viacheslav Y Fofanov, John Gillece, Josie Delisle, Bethany Patton, James Schupp, Krystal Sheridan, Darrin Lemmer, Scott Ostdiek, Harlori K Bains, Jennifer Heim, Tammy Sylvester, Siru Prasai, Melissa Kretschmer, Nicole Fowle, Kenneth Komatsu, Shane Brady, Susan Robinson, Kathryn Fitzpatrick, Gholamabbas Amin Ostovar, Eric Alsop, Elizabeth Hutchins, Kendall Jensen, Paul Keim, David M Engelthaler
Enteroviruses are a common cause of respiratory and gastrointestinal illness, and multiple subtypes, including poliovirus, can cause neurologic disease. In recent years, enterovirus D68 (EV-D68) has been associated with serious neurologic illnesses, including acute flaccid myelitis (AFM), frequently preceded by respiratory disease. A cluster of 11 suspect cases of pediatric AFM was identified in September 2016 in Phoenix, AZ. To determine if these cases were associated with EV-D68, we performed multiple genomic analyses of nasopharyngeal (NP) swabs and cerebrospinal fluid (CSF) material from the patients, including real-time PCR and amplicon sequencing targeting the EV-D68 VP1 gene and unbiased microbiome and metagenomic sequencing...
January 22, 2019: MBio
B Ceronie, O C Cockerell
Acute disseminated encephalomyelitis (ADEM) is an uncommon, autoimmune, demyelinating disorder of of the central nervous system. It is rare in adults beyond 65 years. Here, we describe a novel presentation following urological surgery. Using illustrative features from our case study, we describe some of the clinical features, aetiologies, diagnostic uncertainties and pathogenic mechanisms of the disease. A 69 year old gentleman underwent transurethral resection of the prostate. He then developed confusion, unsteadiness, behavioural disturbance and left-sided hemiparesis...
March 2019: ENeurologicalSci
Arundhati Banerjee, Renu Suthar, Sameer Vyas, Mini P Singh
Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory-demyelinating disease of central nervous system. Several viral infections and vaccines act as precipitating factor for ADEM. We report an 8-year-old girl presenting with acute-onset flaccid paraparesis and bilateral painless vision loss. MRI brain showed extensive multifocal patchy demyelination and she made good clinical recovery with steroids and intravenous immunoglobulin therapy. She had history of bilateral parotid swelling before the onset of neurological symptoms and diagnosis of mumps was confirmed by positive ELISA in serum...
December 27, 2018: BMJ Case Reports
Pekes Codjia, Xavier Ayrignac, Clarisse Carra-Dalliere, Mikael Cohen, Mahmoud Charif, Anais Lippi, Nicolas Collongues, Lucas Corti, Jerome De Seze, Christine Lebrun, Sandra Vukusic, Francoise Durand-Dubief, Pierre Labauge
BACKGROUND: The recent 2017 modification have increased the sensitivity of McDonald criteria for MS. Nevertheless, some MS patients with atypical MRI findings have been identified, leading to prolonged delay to diagnosis and high costs to look for alternative diagnoses. OBJECTIVE: To describe a series of MS patients with atypical MRI presentation. MATERIAL AND METHODS: Patients with atypical MS were identified through a nationwide retrospective study...
December 18, 2018: Multiple Sclerosis and related Disorders
Esra Sarigecili, Meltem Direk Cobanogullari, Mustafa Komur, Cetin Okuyaz
BACKGROUND: Myelin Oligodendrocyte Glycoprotein antibodies (MOG) may be used as a biomarker for diagnosis of many demyelinating diseases. Especially, patients of acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), aquaporin-4 (AQP4) seronegative neuromyelitis optica spectrum disorder (NMOSD), monophasic or recurrent optic neuritis (ON), transverse myelitis and N-methyl-d-aspartate (NMDA) receptor encephalitis (NMDARe) can overlap with Myelin Oligodendrocyte Glycoprotein antibodies...
December 14, 2018: Multiple Sclerosis and related Disorders
Divyanshu Dubey, Sean J Pittock, Karl N Krecke, Padraig P Morris, Elia Sechi, Nicholas L Zalewski, Brian G Weinshenker, Eslam Shosha, Claudia F Lucchinetti, James P Fryer, A Sebastian Lopez-Chiriboga, John C Chen, Jiraporn Jitprapaikulsan, Andrew McKeon, Avi Gadoth, B Mark Keegan, Jan-Mendelt Tillema, Elie Naddaf, Marc C Patterson, Kevin Messacar, Kenneth L Tyler, Eoin P Flanagan
Importance: Recognizing the characteristics of myelin oligodendrocyte glycoprotein autoantibody (MOG-IgG) myelitis is essential for early accurate diagnosis and treatment. Objective: To evaluate the clinical, radiologic, and prognostic features of MOG-IgG myelitis and compare with myelitis with aquaporin-4-IgG (AQP4-IgG) and multiple sclerosis (MS). Design, Setting, and Participants: We retrospectively identified 199 MOG-IgG-positive Mayo Clinic patients from January 1, 2000, through December 31, 2017, through our neuroimmunology laboratory...
December 21, 2018: JAMA Neurology
Markus Reindl, Patrick Waters
Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies (MOG-Abs) were first detected by immunoblot and enzyme-linked immunosorbent assay nearly 30 years ago, but their association with multiple sclerosis (MS) was not specific. Use of cell-based assays with native MOG as the substrate enabled identification of a group of MOG-Ab-positive patients with demyelinating phenotypes. Initially, MOG-Abs were reported in children with acute disseminated encephalomyelitis (ADEM). Further studies identified MOG-Abs in adults and children with ADEM, seizures, encephalitis, anti-aquaporin-4-antibody (AQP4-Ab)-seronegative neuromyelitis optica spectrum disorder (NMOSD) and related syndromes (optic neuritis, myelitis and brainstem encephalitis), but rarely in MS...
December 17, 2018: Nature Reviews. Neurology
Franziska Di Pauli, Thomas Berger
Inflammatory demyelinating CNS syndromes include, besides their most common entity multiple sclerosis (MS), several different diseases of either monophasic or recurrent character-including neuromyelitis optica spectrum disorders (NMOSDs) and acute disseminated encephalomyelitis (ADEM). Early diagnostic differentiation is crucial for devising individual treatment strategies. However, due to overlapping clinical and paraclinical features diagnosis at the first demyelinating event is not always possible. A multiplicity of potential biological markers that could discriminate the different diseases was studied...
2018: Frontiers in Immunology
Parth Bhatt, Leonita Bray, Sneha Raju, Fredrick Dapaah-Siakwan, Achint Patel, Riddhi Chaudhari, Keyur Donda, Neel S Bhatt, Mihir Dave, Vijay Gandhi Linga, Anusha Lekshminarayanan, Samir V Patel, Zeenia C Billimoria, Samuel Zuckerman, Priyank Yagnik, Dinesh Singh
OBJECTIVE: To determine the temporal trends in the epidemiology of acute disseminated encephalomyelitis (ADEM) and hospitalization outcomes in the US from 2006 through 2014. STUDY DESIGN: Pediatric (≤18 years of age) hospitalizations with ADEM discharge diagnosis were identified from the National (Nationwide) Inpatient Sample (NIS) for years 2006 through 2014. Trends in the incidence of ADEM with respect to age, sex, race, and region were examined. Outcomes of ADEM in terms of mortality, length of stay (LOS), cost of hospitalization, and seasonal variation were analyzed...
December 6, 2018: Journal of Pediatrics
Yukako Yae, Go Kawano, Takaoki Yokochi, Toru Imagi, Yukihiro Akita, Keizo Ohbu, Toyojiro Matsuishi
Acute disseminated encephalomyelitis (ADEM) is a typically monophasic inflammatory demyelinating disease of the central nervous system with a favorable outcome. However, 2% of ADEM involves acute hemorrhagic leukoencephalitis (AHLE), which is a fulminant and hyperacute variant of ADEM with a poor outcome and high mortality. There are limited case reports of fulminant ADEM including AHLE in children. Herein, we report two pediatric cases of fulminant ADEM. Both cases had a rapid deterioration of consciousness, repetitive seizures, and brain edema on neuroimaging, in addition to atypical neuroradiological findings on magnetic resonance imaging (MRI), a reversible splenial lesion in case 1, and bilateral frontal and occipital cortical lesions in case 2...
December 3, 2018: Brain & Development
Tetsuo Nakayama
Vaccine adverse events and controversial safety issues have occurred in recent decades in Japan: aseptic meningitis following the measles-mumps-rubella combined vaccine (MMR), anaphylaxis after immunization with live virus vaccines and inactivated split influenza vaccine, an increased incidence of febrile illness following the simultaneous administration of inactivated vaccines, and chronic pain with neurological illness after immunization with the human papilloma virus vaccine (HPV). Vaccine adverse events are a matter of concern for the public as well as general practitioners; some are within the range of assumptions that adverse reactions after live attenuated vaccines are related to the nature of their parental wild-type viruses...
January 7, 2019: Vaccine
Faiza Rasheed, Qasim S Mehdi, Shoaib Bhatti, Muhammad Mannan Ali Khan
Variegate porphyria (VP) is one of the groups of rare inherited disorders of hemoglobin synthesis called Porphyria. It has two distinct manifestations, that is, those of cutaneous and nervous system. Posterior reversible encephalopathy syndrome (PRES) is a rare complication of porphyria. It occurs due to vasogenic edema in white matter of predominantly parieto-occipital lobes, characterized by headache, visual disturbances, altered mental state, hypertension, and seizures. We report a child diagnosed with VP who presents with clinical signs and radiological manifestations suggestive of PRES...
September 24, 2018: Curēus
Rotem Orbach, Nira Schneebaum Sender, Ronit Lubetzky, Aviva Fattal-Valevski
OBJECTIVE: To assess the intracranial pressure in pediatric acute disseminated encephalomyelitis using spinal tap opening pressure on lumbar puncture, which is routinely performed as part of suspected acute disseminated encephalomyelitis workup. Compared to other cerebrospinal fluid parameters such as cell count, protein concentration, and presence of oligoclonal bands, cerebrospinal fluid opening pressure is infrequently recorded. METHODS: A retrospective chart review of demographic, clinical, and laboratory data of children diagnosed with acute disseminated encephalomyelitis admitted to a tertiary referral hospital between 2005 and 2016...
November 27, 2018: Journal of Child Neurology
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