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Acute disseminated encephalomyelitis 

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...
November 29, 2018: 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
Mary Iype, Anish Ts, Pa Mohammed Kunju, Geetha Saradakutty, Mini Sreedharan, Shahanaz M Ahamed
OBJECTIVE: To study the long-term outcome of Acute disseminated encephalomyelitis (ADEM). METHODS: A retrospective cohort study was done among children diagnosed with ADEM (fulfilling IPMSSG criteria). Major outcome variables were motor deficit, scholastic underperformance and behavioral abnormality. RESULTS: The inclusion criteria were fulfilled by 102 children. Three died in hospital. The follow up ranged from 1-10 years (median 4 years)...
September 21, 2018: Pediatric Neurology
Haeng-Jin Lee, Boram Kim, Patrick Waters, Mark Woodhall, Sarosh Irani, Sohyun Ahn, Seong-Joon Kim, Sung-Min Kim
BACKGROUND: Key clinical features of chronic relapsing inflammatory optic neuropathy (CRION) include relapsing inflammatory optic neuritis (ON) and steroid dependency, both of which have been reported among patients with myelin oligodendrocyte glycoprotein antibodies (MOG-Abs). We investigated the relevance of the presence of serum MOG-IgG with the current diagnostic criteria for CRION among patients with idiopathic inflammatory optic neuritis (iON). METHODS: Retrospective reviews of a database prospectively collated between 2011 and 2017 from the tertiary referral center for multiple sclerosis and neuromyelitis optica were performed...
October 31, 2018: Journal of Neuroinflammation
Yanis Tamzali, Sophie Demeret, Elie Haddad, Hélène Guillot, Eric Caumes, Stéphane Jauréguiberry
BACKGROUND: Post-malaria neurological syndrome (PMNS) is a debated entity, defined by neurological complications following a post-malaria symptom-free period and a negative blood smear. Four cases of PMNS are hereby reported and a review the literature performed to clarify the nosological framework of this syndrome. METHODS: A French teaching hospital infectious diseases database was investigated for all PMNS cases occurring between 1999 and 2016 and the PubMed database for cases reported by other institutions after 1997...
October 26, 2018: Malaria Journal
Arun Venkatesan, Olwen C Murphy
Viruses are a frequent cause of encephalitis. Common or important viruses causing encephalitis include herpesviruses, arboviruses, enteroviruses, parechoviruses, mumps, measles, rabies, Ebola, lymphocytic choriomeningitis virus, and henipaviruses. Other viruses may cause an encephalopathy. Host factors and clinical features of infection are important to consider in identifying the cause for encephalitis. Cerebrospinal fluid evaluation, serologic/polymerase chain reaction studies, and neuroimaging are cornerstones of diagnostic evaluation in encephalitis...
November 2018: Neurologic Clinics
Tetsuya Akaishi, Douglas Kazutoshi Sato, Toshiyuki Takahashi, Ichiro Nakashima
Immunoglobulin G (IgG) antibodies against myelin oligodendrocyte glycoprotein (MOG) are detected in the serum of some patients with demyelinating diseases. These patients are known to show repeated clinical episodes of inflammatory demyelinating attacks in the central nervous system. Although the associated pathogenicity and mechanism of inflammatory demyelination remains inconclusive, it is known that patients with MOG-IgG antibodies have a different clinical spectrum from those with other demyelinating diseases, such as multiple sclerosis...
October 23, 2018: Neurochemistry International
Jone Bocos-Portillo, José Luis Sánchez-Menoyo, Marian Gómez Beldarrain, Ibone Saralegui Prieto, Jose María Ontañón Garcés, Josep Dalmau, Juan Carlos García-Moncó
No abstract text is available yet for this article.
October 9, 2018: Clinical Neurology and Neurosurgery
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, Cynthia Wang, Peter Sguigna, Khalil Husari, Benjamin Greenberg
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...
December 15, 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...
November 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
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