3,025 results match your criteria Acute Disseminated Encephalomyelitis


Acute disseminated encephalomyelitis associated with a novel paraneoplastic process in hepatic epithelial hemangioendothelioma: A case report.

Clin Neurol Neurosurg 2020 May 12;194:105903. Epub 2020 May 12.

Department of Neurosurgery, Washington University School of Medicine, 660 South Euclid Ave, St. Louis, MO, 63110, USA. Electronic address:

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http://dx.doi.org/10.1016/j.clineuro.2020.105903DOI Listing

Seropositive anti-MOG antibody-associated acute disseminated encephalomyelitis (ADEM): a sequelae of infection.

BMJ Case Rep 2020 May 19;13(5). Epub 2020 May 19.

Department of Pediatric Infectious Disease, Kaiser Permanente Oakland Medical Center, Oakland, California, USA.

Acute disseminated encephalomyelitis (ADEM) is a demyelinating, autoimmune disease of the central nervous system (CNS). It causes motor and sensory deficits, altered mental status and other neurological symptoms. Though rarely fatal, it has been associated with residual motor and neurocognitive deficits. Read More

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http://dx.doi.org/10.1136/bcr-2020-234565DOI Listing

Seasonal distribution of attacks in aquaporin-4 antibody disease and myelin-oligodendrocyte antibody disease.

J Neurol Sci 2020 May 7;415:116881. Epub 2020 May 7.

Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, United Kingdom. Electronic address:

Background: Seasonal variation in incidence and exacerbations has been reported for neuroinflammatory conditions such as multiple sclerosis and acute disseminated encephalomyelitis (ADEM). It is unknown whether seasonality also influences aquaporin-4 antibody (AQP4-Ab) disease and myelin-oligodendrocyte antibody (MOG-Ab) disease.

Objective: We examined the seasonal distribution of attacks in AQP4-Ab disease and MOG-Ab disease. Read More

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http://dx.doi.org/10.1016/j.jns.2020.116881DOI Listing

Neurologic Manifestations of Severe Respiratory Viral Contagions.

Crit Care Explor 2020 Apr 29;2(4):e0107. Epub 2020 Apr 29.

Both authors: Departments of Neurology and Neurosurgery, University of Florida, Gainesville, FL.

Endemic and pandemic viral respiratory infections have recently emerged as a critical topic of investigation given the recent severe acute respiratory syndrome coronavirus-2 outbreak. Data from such outbreaks indicate that severe systemic comorbidities including acute neurologic illness are associated with illness and lead to significant outcome differences. Herein, we will discuss the neurologic manifestations of severe viral respiratory infections including coronavirus, influenza, respiratory syncytial virus, metapneumovirus, and enterovirus. Read More

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http://dx.doi.org/10.1097/CCE.0000000000000107DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7188429PMC

Myelin oligodendrocyte glycoprotein antibody-associated disease: an immunopathological study.

Brain 2020 May;143(5):1431-1446

Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan.

Conformation-sensitive antibodies against myelin oligodendrocyte glycoprotein (MOG) are detectable in patients with optic neuritis, myelitis, opticomyelitis, acute or multiphasic disseminated encephalomyelitis (ADEM/MDEM) and brainstem/cerebral cortical encephalitis, but are rarely detected in patients with prototypic multiple sclerosis. So far, there has been no systematic study on the pathological relationship between demyelinating lesions and cellular/humoral immunity in MOG antibody-associated disease. Furthermore, it is unclear whether the pathomechanisms of MOG antibody-mediated demyelination are similar to the demyelination patterns of multiple sclerosis, neuromyelitis optica spectrum disorders (NMOSD) with AQP4 antibody, or ADEM. Read More

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http://dx.doi.org/10.1093/brain/awaa102DOI Listing

Postvaccination Anti-Myelin Oligodendrocyte Glycoprotein Neuromyelitis Optica Spectrum Disorder: A Case Report and Literature Review of Postvaccination Demyelination.

Int J MS Care 2020 Mar-Apr;22(2):85-90

Stimulation of the immune response after vaccination can occasionally result in adverse effects, including demyelination of the central nervous system. The most common presentation of postvaccination demyelination is acute disseminated encephalomyelitis, but cases of optic neuritis, transverse myelitis, and multiple sclerosis relapses have been reported. More recently, an increasing number of postvaccination neuromyelitis optica spectrum disorder (NMOSD) cases have surfaced in the literature, especially in patients with aquaporin-4 antibodies. Read More

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http://dx.doi.org/10.7224/1537-2073.2018-104DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7204366PMC

Myelin oligodendrocyte glycoprotein antibody-associated disorders: clinical spectrum, diagnostic evaluation, and treatment options.

Clin Exp Pediatr 2020 May 14. Epub 2020 May 14.

Department of Pediatrics, Pusan National University Children's Hospital, Pusan National University College of Medicine, Yangsan, Korea.

Inflammatory or immune-mediated demyelinating central nervous system (CNS) syndromes include a broad spectrum of clinical phenotype and different overlapping diseases. Antibodies against myelin oligodendrocyte glycoprotein (MOG-Ab) have been found in some cases of these demyelinating diseases, particularly in children. MOG-Ab is associated with a wider clinical phenotype not limited to neuromyelitis optica spectrum disorder, with most patients presenting with optic neuritis, acute disseminated encephalomyelitis (ADEM) or ADEM-like encephalitis with brain demyelinating lesions, and/or myelitis. Read More

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http://dx.doi.org/10.3345/cep.2019.01305DOI Listing

Developmental brain abnormalities and acute encephalopathy in a patient with myopathy with extrapyramidal signs secondary to pathogenic variants in MICU1.

JIMD Rep 2020 May 20;53(1):22-28. Epub 2020 Mar 20.

Center for Individualized Medicine Mayo Clinic Rochester Minnesota USA.

Mitochondria play a variety of roles in the cell, far beyond their widely recognized role in ATP generation. One such role is the regulation and sequestration of calcium, which is done with the help of the mitochondrial calcium uniporter (MCU) and its regulators, MICU1 and MICU2. Genetic variations in MICU1 and MICU2 have been reported to cause myopathy, developmental disability and neurological symptoms typical of mitochondrial disorders. Read More

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http://dx.doi.org/10.1002/jmd2.12114DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7203647PMC

Neuromyelitis optica spectrum disorders and myelin oligodendrocyte glycoprotein antibody-associated disease: current topics.

Curr Opin Neurol 2020 Jun;33(3):300-308

University of Utah School of Medicine, Salt Lake City, Utah, USA.

Purpose Of Review: We reviewed present topics on neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein (MOG)-antibody-associated disease (MOGAD).

Recent Findings: The number of NMOSD-related publications have increased year by year after the discovery of aquaporin 4 (AQP4)-antibody, and those on MOGAD started to surge since 2012-2013. Recent clinic-epidemiological surveys in NMOSD suggest that some racial differences in the prevalence and the clinical course. Read More

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http://dx.doi.org/10.1097/WCO.0000000000000828DOI Listing

Case Report: Dengue Virus-Triggered Parkinsonism in an Adolescent.

Am J Trop Med Hyg 2020 May 4. Epub 2020 May 4.

Department of Pediatrics, All India Institute of Medical Sciences, Rishikesh, India.

Dengue fever continues to be an important cause of morbidity and mortality in tropical and subtropical countries. A wide range of neurological manifestations including dengue encephalopathy, Guillain-Barre syndrome, acute disseminated encephalomyelitis, transverse myelitis, cranial nerve palsies, and myositis have been reported following dengue infection. But parkinsonism secondary to dengue virus infection is uncommon, with only three published case reports in adults and one in children. Read More

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http://dx.doi.org/10.4269/ajtmh.20-0039DOI Listing
May 2020
2.699 Impact Factor

Teaching Video Neuroimages: Pathologic yawning: A sign of brainstem involvement in acute disseminated encephalomyelitis?

Neurology 2020 May 4. Epub 2020 May 4.

From the Department of Pediatrics (V.B., K.A.M.), Division of Child Neurology, and Department of Medical Imaging (C.S.-M.), Montreal Children's Hospital; Department of Neurology & Neurosurgery (K.A.M.), and Research Institute of the McGill University Health Centre (K.A.M.), McGill University Health Centre, Montreal, Canada.

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http://dx.doi.org/10.1212/WNL.0000000000009595DOI Listing

Neurological Manifestations of Influenza A (H1N1): Clinical Features, Intensive Care Needs, and Outcome.

Indian J Pediatr 2020 May 2. Epub 2020 May 2.

Department of Pediatrics, Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.

Objectives: To describe neurological manifestations in children with Influenza A (H1N1).

Methods: This retrospective study was conducted in the Pediatric intensive care unit (PICU) and Pediatric Neurology unit of a tertiary care teaching hospital in North India involving children with PCR confirmed Influenza A (H1N1) with neurological manifestations during 2019 outbreak.

Results: Six children (5 females, 1 male) were enrolled. Read More

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http://dx.doi.org/10.1007/s12098-020-03297-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7222163PMC

Neurological Implications of COVID-19 Infections.

Neurocrit Care 2020 Apr 28. Epub 2020 Apr 28.

Division of Anaesthesia, University of Cambridge, Cambridge, UK.

The magnitude of the COVID-19 pandemic will result in substantial neurological disease, whether through direct infection (rare), para-infectious complications (less rare), or critical illness more generally (common). Here, we raise the importance of stringent diagnosis and data collection regarding neurological complications of COVID-19; we urge caution in the over-diagnosis of neurological disease where it does not exist, but equally strongly encourage the concerted surveillance for such conditions. Additional to the direct neurological complications of COVID-19 infection, neurological patients are at risk of harm from both structural limitations (such as number of intensive care beds), and a hesitancy to treat with certain necessary medications given risk of nosocomial COVID-19 infection. Read More

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http://dx.doi.org/10.1007/s12028-020-00978-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7188454PMC

Spectrum of anti-myelin oligodendrocyte glycoprotein antibody (MOG-Ab)-associated diseases: an Indian perspective.

Acta Neurol Belg 2020 Apr 20. Epub 2020 Apr 20.

Department of Neuromedicine, Bangur Institute of Neurosciences, 52/1a, Sambhunath Pandit Street, Kolkata, 700020, India.

Myelin oligodendrocyte glycoprotein antibody (MOG-Ab) is involved in the pathogenesis of central nervous system (CNS) demyelination disorders. We aimed to explore the spectrum of MOG-Ab-associated diseases in eastern India. A single-center, prospective observational study was done over a period of 2 years in a tertiary care hospital of eastern India. Read More

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http://dx.doi.org/10.1007/s13760-020-01356-9DOI Listing
April 2020
0.598 Impact Factor

[Clinical features of children with myelin oligodendrocyte glycoprotein antibody-associated disorders].

Zhongguo Dang Dai Er Ke Za Zhi 2020 Apr;22(4):368-373

Department of Neurology, Children's Hospital, Capital Institute of Pediatrics, Beijing 100020, China.

Objective: To study the clinical features and treatment outcome of children with myelin oligodendrocyte glycoprotein (MOG) antibody-associated disorders (MOGAD).

Methods: A retrospective analysis was performed for the clinical data of 28 children with MOGAD (with 38 demyelinating episodes).

Results: Among the disease spectrums of 28 children with MOGAD, optic neuritis was the most common (12 cases, 43%), followed by acute disseminated encephalomyelitis (9 cases, 32%). Read More

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Feasibility & safety of plasma exchange in paediatric neuro-immunology: A single center experience.

Eur J Paediatr Neurol 2020 Apr 10. Epub 2020 Apr 10.

Division of Child Neurology, Department of Pediatrics, Cohen Children's Medical Center, New Hyde Park, NY, USA. Electronic address:

Background: There is limited data available on the safety of therapeutic plasma exchange (TPE) for paediatric neuro-immunological disorders (PNID). In this study, we report our data on safety and feasibility of TPE for these disorders.

Methods: Retrospective chart review was performed to include all patient who received TPE for four major PNID conditions: autoimmune encephalitis (AIE), acute disseminated encephalomyelitis (ADEM), Neuromyelitis optic spectrum disorder (NMOSD) and transverse myelitis (TM). Read More

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http://dx.doi.org/10.1016/j.ejpn.2020.04.001DOI Listing

Etiology and prognosis of encephalitis in French Guianese children: a retrospective record-based study.

J Infect Public Health 2020 Apr 10. Epub 2020 Apr 10.

Pediatric Unit Cayenne General Hospital, Cayenne, French Guiana.

Acute encephalitis is an important cause of mortality and morbidity in children. We retrospectively identified children (≤15 years of age) admitted with suspected encephalitis at the Intensive Care Unit of the Pediatric Department of Cayenne Hospital between January 2007 and December 2018. A total of 30 children with acute encephalitis were identified. Read More

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http://dx.doi.org/10.1016/j.jiph.2020.01.315DOI Listing

Possible clinical role of MOG antibody testing in children presenting with acute neurological symptoms.

Neurol Sci 2020 Apr 3. Epub 2020 Apr 3.

Department of Laboratory Medicine, University-Hospital of Padova, via Giustiniani 2, 35128, Padova, Italy.

The differential diagnosis between acquired inflammatory demyelinating syndromes of the central nervous system (CNS), such as multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD) and acute disseminated encephalomyelitis (ADEM) can be very challenging at onset. Apart from cerebrospinal fluid oligoclonal bands and anti-aquaporin-4 antibodies (AQP4-Ab), definite diagnostic biomarkers are lacking. Anti-myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) have been increasingly described in children with AQP4-seronegative NMOSD, ADEM and other inflammatory demyelinating CND syndromes; despite partial overlaps with AQP4-Ab disease, a novel "MOG-Ab-disorder" phenotype has been suggested. Read More

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http://dx.doi.org/10.1007/s10072-020-04379-5DOI Listing

Clinical analysis of anti-NMDAR encephalitis combined with MOG antibody in children.

Mult Scler Relat Disord 2020 Feb 22;42:102018. Epub 2020 Feb 22.

Department of Neurology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, The 9# Jin Sui Road, Guangzhou, Guangdong 510623, China. Electronic address:

Objective: To analyze the clinical features in children with anti-NMDAR encephalitis combined with myelin oligodendrocyte glycoprotein antibody (MOG ab).

Methods: Clinical data of 7 children with anti-NMDAR encephalitis combined with MOG ab(+) were collected in Guangzhou Women and Children's Medical Center from January, 2016 to June, 2019. Children with NMDAR ab(+)/MOG ab(-) and MOG ab(+)/NMDAR ab(-) were randomly selected as controls. Read More

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http://dx.doi.org/10.1016/j.msard.2020.102018DOI Listing
February 2020

Seizure occurrence in myelin oligodendrocyte glycoprotein antibody-associated disease: A systematic review and meta-analysis.

Mult Scler Relat Disord 2020 Mar 14;42:102057. Epub 2020 Mar 14.

Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China. Electronic address:

Objective: Recent reports have suggested that seizures may be a component of the clinical presentation in myelin oligodendrocyte glycoprotein antibody (MOG-Ab)-associated disease. We aimed to conduct a systematic review and meta-analysis to comprehensively evaluate the occurrence of epileptic seizures in the disease.

Methods: We searched PubMed, MEDLINE and EMBASE for studies reporting the occurrence of acute symptomatic seizures in MOG-Ab-associated disease. Read More

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http://dx.doi.org/10.1016/j.msard.2020.102057DOI Listing

Multiphasic acute disseminated encephalomyelitis and differential with early onset multiple sclerosis.

Intractable Rare Dis Res 2020 Feb;9(1):61-63

Neurophysiology Department, Clínica das Américas, Curitiba, Brasil.

Multiple sclerosis is considered the most frequent demyelinating disorder of the Central Nervous System (CNS) among young adults, yet is very rare before 10 years old. Acute disseminated encephalomyelitis is a monophasic, polysymptomatic disorder that involves the CNS white matter with demyelinating lesions, which usually occurs after systemic viral infections. These two demyelinating diseases can present initially as an acute focal neurological syndrome and they can be difficult to distinguish. Read More

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http://dx.doi.org/10.5582/irdr.2020.01009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7062603PMC
February 2020

Neurologic Manifestations as Initial Clinical Presentation of Familial Hemophagocytic Lymphohistiocytosis Type2 Due to Mutation in Chinese Pediatric Patients.

Front Genet 2020 4;11:126. Epub 2020 Mar 4.

Neurology Department, National Center for Children's Health China, Beijing Children Hospital affiliated to Capital Medical University, Beijing, China.

Familial hemophagocytic lymphohistiocytosis Type 2 (FHL2) associated central nervous system (CNS) involvement is less understood in children, especially when considering neurologic manifestations as part of the initial presentation. We conducted a retrospective review of the clinical manifestations and genetic abnormality of four Han Chinese children with FHL2 who were patients at the neurology department of Beijing Children's Hospital from November 2015 to October 2018. These four patients initially manifested CNS symptoms in their disease presentation, and all four patients were misdiagnosed as having ademyelinating disease, such as acute disseminated encephalomyelitis and multiple sclerosis. Read More

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http://dx.doi.org/10.3389/fgene.2020.00126DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7064636PMC

En mann i 60-årene med gangvansker, dysartri og lett kognitiv svikt.

Tidsskr Nor Laegeforen 2020 Mar 9;140(4). Epub 2020 Mar 9.

Background: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome is an immune-mediated, treatable and inflammatory CNS disease first reported by Pittock et al. (2010).

Case Presentation: We describe a 66-year-old man with previous history of diabetes, atrial fibrillation and hypertension, who was admitted to hospital with reduced general condition. Read More

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http://dx.doi.org/10.4045/tidsskr.19.0198DOI Listing

Seizures and myelin oligodendrocyte glycoprotein (MOG) antibodies: Two paradigmatic cases and a review of the literature.

Mult Scler Relat Disord 2020 Feb 22;41:102011. Epub 2020 Feb 22.

Pediatric Clinic, Fondazione IRCCS Policlinico San Matteo, Pavia, University of Pavia,V.le C. Golgi, 19 - 27100 Pavia, Pavia, Italy.

Background: Myelin oligodendrocyte glycoprotein (MOG) antibodies (Abs) have been associated with a heterogeneous range of acquired CNS demyelinating disorders. More recently, increasing evidence correlates the presence of such Abs with seizures, occurring in concomitance with CNS demyelinating events, or even as isolated phenomena. In this scenario, the full clinical spectrum of MOG Ab-associated seizures and the contribution of such Abs to epileptogenesis are unclear. Read More

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http://dx.doi.org/10.1016/j.msard.2020.102011DOI Listing
February 2020

Serum α-synuclein and IL-1β are increased and correlated with measures of disease severity in children with epilepsy: potential prognostic biomarkers?

BMC Neurol 2020 Mar 9;20(1):85. Epub 2020 Mar 9.

Department of Microbiology, Brain Korea 21 Plus Project for Medical Science, Severance Biomedical Science Institute and Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, 50-1 Yonsei-ro Seodaemoon-gu Seoul, Seoul, 03722, South Korea.

Background: The search for noninvasive biomarkers of neuroinflammation and neurodegeneration has focused on various neurological disorders, including epilepsy. We sought to determine whether α-synuclein and cytokines are correlated with the degree of neuroinflammation and/or neurodegeneration in children with epilepsy and with acquired demyelinating disorders of the central nervous system (CNS), as a prototype of autoimmune neuroinflammatory disorders.

Methods: We analyzed serum and exosome levels of α-synuclein and serum proinflammatory and anti-inflammatory cytokines among 115 children with epilepsy and 10 acquired demyelinating disorders of the CNS and compared to 146 controls. Read More

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http://dx.doi.org/10.1186/s12883-020-01662-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7061464PMC

Neuro-ophthalmological manifestations as complication of an infection with Mycoplasma pneumoniae and subsequent development of disseminated acute encephalitis.

Arch Soc Esp Oftalmol 2020 May 5;95(5):254-258. Epub 2020 Mar 5.

Departamento de Neuroftalmología, Hospital Clínico San Carlos, Madrid, España.

The purpose of this article is to describe two paediatric neuro-ophthalmological clinical cases caused by a systemic infection due to Mycoplasma pneumoniae (M. pneumoniae). The cases are two girls aged 14 and 12 seen in the Emergency Department: The first one had internuclear ophthalmoplegia and second with loss of vision and headache. Read More

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http://dx.doi.org/10.1016/j.oftal.2020.01.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7115784PMC

Tumefactive Acute Disseminated Encephalomyelitis.

Neurol India 2020 Jan-Feb;68(1):35-41

Department of Neurological Surgery, Houston Methodist Hospital, 6560 Fannin Street, Houston, TX, USA.

Tumefactive demyelination is a phenomenon involving the radiographic resemblance of an acute demyelinating process in the central nervous system to neoplasia. Although this has been described and characterized for multiple sclerosis, it has been reported in a few cases in patients with acute disseminated encephalomyelitis (ADEM) within the past decade. While it may be challenging to establish a diagnosis of tumefactive ADEM according to clinical and radiological data alone, a thorough review of the clinical history and following the patient over time can be supportive of the same. Read More

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http://dx.doi.org/10.4103/0028-3886.279688DOI Listing

A Fulminant Case of Demyelinating Encephalitis With Extensive Cortical Involvement Associated With Anti-MOG Antibodies.

Front Neurol 2020 14;11:31. Epub 2020 Feb 14.

Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria.

Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies (MOG-Abs) are commonly associated with clinical presentations as acute disseminated encephalomyelitis (ADEM) in both adults and children and anti-aquaporin 4 antibody-seronegative neuromyelitis optica spectrum disorder (NMOSD) and related syndromes such as optic neuritis, myelitis, and brainstem encephalitis. Most often, the presence of MOG-Abs is associated with a more benign clinical course and a good response to steroids. Here, we present a case report of a previously healthy 52-year-old female patient with fulminant demyelinating encephalitis, leading to death within a week after the first presenting symptoms from a massive brain edema irresponsive to high-dose intravenous steroids as well as osmotic therapy. Read More

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http://dx.doi.org/10.3389/fneur.2020.00031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7034704PMC
February 2020

Radiological Cerebrospinal Posterior Reversible Encephalopathy Syndrome Mimicking Acute Disseminated Encephalomyelitis in a Neurologically Asymptomatic Child.

Pediatr Neurol 2020 May 9;106:65-67. Epub 2020 Jan 9.

Department of Children's Neurosciences, Guys and St. Thomas' Hospital NHS Foundation Trust, Kings Health Partners, London, UK.

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.11.021DOI Listing

Complement factor I deficiency: A potentially treatable cause of fulminant cerebral inflammation.

Neurol Neuroimmunol Neuroinflamm 2020 May 25;7(3). Epub 2020 Feb 25.

From the Department of Paediatric Infectious Disease (T.A, S.O.), Newcastle upon Tyne Hospitals NHS Foundation Trust; Division of Infection and Immunity and Dementia Research Institute (M.T., B.P.M.), School of Medicine, Cardiff University; Department of Neuroradiology (D.M.), Newcastle upon Tyne Hospitals NHS Foundation Trust; Department of National Renal Complement Therapeutics Centre (N.S.S., D.K.), Newcastle upon Tyne Hospitals NHS Foundation Trust; Complement Therapeutics Research (N.S.S., D.K.), Translational and Clinical Research Institute, Newcastle University; Department of Paediatric Neurology (R.F.), Newcastle upon Tyne Hospitals NHS Foundation Trust; Neuroscience, Neurodisability and Neurological Disorders Groups (R.F.), Translational and Clinical Research Institute, Newcastle University, United Kingdom.

Objective: To raise awareness of complement factor I (CFI) deficiency as a potentially treatable cause of severe cerebral inflammation.

Methods: Case report with neuroradiology, neuropathology, and functional data describing the mutation with review of literature.

Results: We present a case of acute, fulminant, destructive cerebral edema in a previously well 11-year-old, demonstrating massive activation of complement pathways on neuropathology and compound heterozygote status for 2 pathogenic mutations in CFI which result in normal levels but completely abrogate function. Read More

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http://dx.doi.org/10.1212/NXI.0000000000000689DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7051217PMC

First Clinical Experience with Anti-myelin Oligodendrocyte Glycoprotein Antibody-Positive Optic Neuritis.

Klin Monbl Augenheilkd 2020 Apr 24;237(4):458-463. Epub 2020 Feb 24.

Department of Ophthalmology, Cantonal Hospital St. Gallen, St. Gallen, Switzerland.

Background: Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been consistently found in a range of demyelinating disorders. In this context, MOG-IgG-associated optic neuritis (ON) has been suggested as a new subset of optic neuropathy. However, clinical manifestations and distinctive characteristics have only rarely been described. Read More

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http://dx.doi.org/10.1055/a-1068-2506DOI Listing

[Clinical features and prognosis of pediatric myelin oligodendrocyte glycoprotein antibody associated acute disseminated encephalomyelitis].

Zhonghua Yi Xue Za Zhi 2020 Feb;100(5):339-344

Department of Neurology, Guangzhou Women and Children's Medical Center, Guangzhou 510120, China.

To analyze the clinical features, outcome and prognosis of pediatric myelin oligodendrocyte glycoprotein (MOG) antibody associated acute disseminated encephalomyelitis (ADEM), and provide evidence for improving the diagnosis and treatment of this disease. This study involved 30 MOG antibody-associated ADEM patients in the Department of Neurology, Guangzhou Women and Children's Medical Center. Patients' clinical information were analyzed. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0376-2491.2020.05.005DOI Listing
February 2020

Case Report: An Adolescent Girl with Isolated Neuropsychiatric Features and Apparent Post-Malaria Neurological Syndrome.

Am J Trop Med Hyg 2020 May;102(5):1030-1032

Pediatric Specialist, District Hospital, Jaipur, India.

The post-malaria neurological syndrome (PMNS) is an unusual and relatively underreported complication of malaria, which usually occurs after the resolution of acute febrile illness and the patient is free from parasitemia. The clinical spectrum of the PMNS varies from acute-onset cerebellar ataxia to significant encephalopathy with focal deficits resembling acute disseminated encephalomyelitis. Uncommon presentations of PMNS include Guillain-Barre syndrome, postural tremor, or even isolated neuropsychiatric features. Read More

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http://dx.doi.org/10.4269/ajtmh.19-0791DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7204597PMC
May 2020
2.699 Impact Factor

Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: a multicentre observational study.

Lancet Neurol 2020 Mar 10;19(3):234-246. Epub 2020 Feb 10.

Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, Universitat de Barcelona, Barcelona, Spain; Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA; Catalan Institute for Research and Advanced Studies, Barcelona, Spain. Electronic address:

Background: Investigations of myelin oligodendrocyte glycoprotein (MOG) antibodies are usually focused on demyelinating syndromes, but the entire spectrum of MOG antibody-associated syndromes in children is unknown. In this study, we aimed to determine the frequency and distribution of paediatric demyelinating and encephalitic syndromes with MOG antibodies, their response to treatment, and the phenotypes associated with poor prognosis.

Methods: In this prospective observational study, children with demyelinating syndromes and with encephalitis other than acute disseminated encephalomyelitis (ADEM) recruited from 40 secondary and tertiary centres in Spain were investigated for MOG antibodies. Read More

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http://dx.doi.org/10.1016/S1474-4422(19)30488-0DOI Listing

MOG encephalomyelitis: distinct clinical, MRI and CSF features in patients with longitudinal extensive transverse myelitis as first clinical presentation.

J Neurol 2020 Feb 13. Epub 2020 Feb 13.

Department of Neurology, Focus Program Translational Neuroscience (FTN), Rhine Main Neuroscience Network (rmn2), University Medical Center of the Johannes Gutenberg-University of Mainz, Mainz, Germany.

Background: Based on clinical, immunological and histopathological evidence, MOG-IgG-associated encephalomyelitis (MOG-EM) has emerged as a distinct disease entity different from multiple sclerosis (MS) and aquaporin-4-antibody-positive neuromyelitis optica spectrum disorder (NMOSD). MOG-EM is associated with a broader clinical phenotype including optic neuritis, myelitis, brainstem lesions and acute disseminated encephalomyelitis with a substantial clinical and radiological overlap to other demyelinating CNS disorders.

Objective: To evaluate common clinical, MRI and CSF findings, as well as therapy responses in patients with longitudinal extensive transverse myelitis (LETM) as initial clinical presentation of MOG-EM. Read More

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http://dx.doi.org/10.1007/s00415-020-09755-xDOI Listing
February 2020

The pathology of central nervous system inflammatory demyelinating disease accompanying myelin oligodendrocyte glycoprotein autoantibody.

Acta Neuropathol 2020 May 11;139(5):875-892. Epub 2020 Feb 11.

Department of Neurology, Mayo Clinic, Rochester, MN, USA.

We sought to define the pathological features of myelin oligodendrocyte glycoprotein (MOG) antibody associated disorders (MOGAD) in an archival autopsy/biopsy cohort. We histopathologically analyzed 2 autopsies and 22 brain biopsies from patients with CNS inflammatory demyelinating diseases seropositive for MOG-antibody by live-cell-based-assay with full length MOG in its conformational form. MOGAD autopsies (ages 52 and 67) demonstrate the full spectrum of histopathological features observed within the 22 brain biopsies (median age, 10 years; range, 1-66; 56% female). Read More

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http://dx.doi.org/10.1007/s00401-020-02132-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7181560PMC

Pediatric autoimmune encephalitis: Recognition and diagnosis.

Neurol Neuroimmunol Neuroinflamm 2020 May 11;7(3). Epub 2020 Feb 11.

From the Department of Neurology (M.A.A.M.d.B., A.L.B., A.E.M.B., A.v.S., P.A.E.S.S., M.J.T.), Department of Immunology (M.W.J.S.), and Department of Pediatric Neurology (R.F.N.), Erasmus MC University Medical Center, Rotterdam; Haga Hospital (A.v.S.), The Hague; and Sophia Children's Hospital (R.F.N.), Rotterdam, the Netherlands. A.v.S. is currently working at Medisch Centrum Haaglanden, The Hague.

Objective: The aims of this study were (1) to describe the incidence of autoimmune encephalitis (AIE) and acute disseminated encephalomyelitis (ADEM) in children, (2) to validate the currently used clinical criteria to diagnose AIE, and (3) to describe pitfalls in the diagnosis of pediatric autoimmune (AI) and inflammatory neurologic disorders.

Methods: This study cohort consists of 3 patient categories: (1) children with antibody-mediated AIE (n = 21), (2) children with ADEM (n = 32), and (3) children with suspicion of an AI etiology of their neurologic symptoms (n = 60). Baseline and follow-up clinical data were used to validate the current guideline to diagnose AIE. Read More

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http://dx.doi.org/10.1212/NXI.0000000000000682DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7051211PMC

Variation in Treatment and Outcomes of Children With Acute Disseminated Encephalomyelitis.

Hosp Pediatr 2020 Feb;10(2):159-165

Department of Neurology, Seattle Children's Hospital, Seattle, Washington.

Objectives: To characterize variation in treatments and outcomes of pediatric patients admitted to children's hospitals with acute disseminated encephalomyelitis (ADEM).

Methods: In this retrospective cohort study, we used data from the Pediatric Health Information System. Children >30 days old who were hospitalized from 2010 to 2015 with ADEM were included. Read More

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http://dx.doi.org/10.1542/hpeds.2019-0129DOI Listing
February 2020

Cryptococcus neoformans var. gattii isolate with Unusual Morphology.

Rev Soc Bras Med Trop 2020;53:e20190412. Epub 2020 Jan 27.

Department of Marine Biology, Microbiology and Biochemistry, School of Marine Sciences, Cochin University of Science and Technology, Fine Arts Avenue, Kochi, Kerala, India.

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http://dx.doi.org/10.1590/0037-8682-0412-2019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7083375PMC
March 2020
0.942 Impact Factor

Acute disseminated encephalomyelitis (ADEM) associated with mosquito-borne diseases: Chikungunya virus X yellow fever immunization.

Rev Soc Bras Med Trop 2020 27;53:e20190160. Epub 2020 Jan 27.

Universidade Federal do Rio de Janeiro, Faculdade de Medicina, Departamento de Oftalmologia, Rio de Janeiro, RJ, Brasil.

Acute disseminated encephalomyelitis (ADEM) is a demyelinating autoimmune neuropathic condition characterized by extensive bilateral and confluent lesions in the cerebral white matter and cerebellum. The basal ganglia and gray matter may also be involved. In most cases, the symptoms are preceded by viral infection or vaccination. Read More

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http://dx.doi.org/10.1590/0037-8682-0160-2019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7083384PMC

[Influenza - associated acute necrotizing encephalitis in adults].

Zh Nevrol Psikhiatr Im S S Korsakova 2019 ;119(12):100-105

Infectious Clinical Hospital #1, Moscow, Russia.

The authors describe a rare clinical case of non-fatal acute necrotizing encephalitis associated with influenza virus in an adult man. This clinical case is one of the few cases published in the world literature and the only one in the Russian literature. Read More

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http://dx.doi.org/10.17116/jnevro2019119121100DOI Listing
January 2020

Anti-GM2 Antibodies in Mycoplasma Pneumoniae-Associated Acute Encephalomyelitis.

Can J Neurol Sci 2020 Mar;47(2):258-260

Department of Neurology, General Hospital of Northern Theater Command, Shenyang City, China.

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http://dx.doi.org/10.1017/cjn.2019.337DOI Listing

Acute Disseminated Encephalomyelitis followed by Optic Neuritis: A Rare Syndrome of Uncertain Treatment and Prognosis.

Neuropediatrics 2020 Jan 14. Epub 2020 Jan 14.

Dipartimento di Scienze Mediche di Base, Neuroscienze ed Organi di Senso, Università degli Studi di Bari "Aldo Moro," Bari, Italy.

Aim:  Acute Disseminated Encephalomyelitis followed by optic neuritis (ADEM-ON), first described in 2013, is a rare demyelinating syndrome, typical of the pediatric age. We conducted a mini review of the existing literature, focusing on clinical, laboratory, radiological, therapeutic, and prognostic aspects in order to improve the identification of new cases.

Methods:  We searched PubMed and Cochrane Library for studies on ADEM-ON between 2013 and 2018. Read More

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http://dx.doi.org/10.1055/s-0039-3402004DOI Listing
January 2020

[Clinical and biochemical characteristics of atypical variants of multiple sclerosis].

Zh Nevrol Psikhiatr Im S S Korsakova 2019 ;119(10. Vyp. 2):81-86

Research Center of Neurology, Moscow, Russia.

Aim: To study the clinical and biochemical features of atypical variants of multiple sclerosis (MS) (tumefactive demyelination (TD), Balo's concentric sclerosis (BCS)) and acute disseminated encephalomyelitis (ADEM)).

Material And Methods: Forty-two patients were studied, including 32 patients with atypical variants of MS (6 patients with BCS and 26 patients with TD) and 10 patients with ADEM. The control group included 20 healthy volunteers. Read More

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http://dx.doi.org/10.17116/jnevro20191191081DOI Listing
February 2020

Therapeutic plasma exchange in pediatric intensive care: Indications, results and complications.

Ther Apher Dial 2020 Apr 17;24(2):221-229. Epub 2020 Feb 17.

Acıbadem Mehmet Ali Aydınlar University, Pediatric Bone Marrow Transplantation Uni, Istanbul, Turkey.

Therapeutic plasma exchange (TPE) is an effective treatment method in selective indications. Secondary to access and technical features, it is more difficult to apply in pediatric population than adults. The aim of this study is investigate safety, clinical indications, and results of this method in critically ill pediatric patients who need TPE treatment. Read More

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http://dx.doi.org/10.1111/1744-9987.13474DOI Listing

Extensive subpial cortical demyelination is specific to multiple sclerosis.

Brain Pathol 2020 May 3;30(3):641-652. Epub 2020 Feb 3.

Institute of Neuropathology, University Medical Center Göttingen, Georg August University Göttingen, Robert-Koch-Str. 40, 37075, Göttingen, Germany.

Cortical demyelinated lesions are frequent and widespread in chronic multiple sclerosis (MS) patients, and may contribute to disease progression. Inflammation and related oxidative stress have been proposed as central mediators of cortical damage, yet meningeal and cortical inflammation is not specific to MS, but also occurs in other diseases. The first aim of this study was to test whether cortical demyelination was specific for demyelinating CNS diseases compared to other CNS disorders with prominent meningeal and cortical inflammation. Read More

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http://dx.doi.org/10.1111/bpa.12813DOI Listing

Vanishing Pseudotumoral White Matter Lesions Presenting as Aphasia and Altered Mental Status in a 71-Year-Old Male.

Cureus 2019 Dec 3;11(12):e6284. Epub 2019 Dec 3.

Neurology, Centro Hospitalar São João, Porto, PRT.

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disorder that usually affects the central nervous system (CNS) after an infection and/or vaccination. It is more common in infancy. Here we present a case of late onset ADEM. Read More

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http://dx.doi.org/10.7759/cureus.6284DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6939973PMC
December 2019

Efficacy of levofloxacin on macrolide-unresponsive and corticosteroid-resistant refractory Mycoplasma pneumoniae pneumonia in children.

Ann Palliat Med 2019 Nov;8(5):632-639

Department of Pediatrics, The Second Affiliated Hospital of Wenzhou Medical University, WenZhou 325027, China.

Background: This study aimed to summarize the experience in treatment of Mycoplasma pneumoniae pneumonia (MPP) with worsening lung shadow despite treatment with appropriate antimicrobials and corticosteroid in children.

Methods: All patients satisfied refractory MP pneumonia (RMPP) diagnostic criteria were enrolled. The clinical manifestations, laboratory findings, imaging features, treatments, and outcomes were retrospectively reviewed. Read More

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http://dx.doi.org/10.21037/apm.2019.10.05DOI Listing
November 2019

[Encephalitis caused by type B influenza virus in an adult. Report of one case].

Rev Med Chil 2019 Jul;147(7):922-927

Laboratorio de Biología Molecular, Hospital Base de Valdivia, Valdivia, Chile.

Neurological manifestations associated with influenza virus infection include encephalitis, encephalopathy, acute necrotizing encephalitis, transverse myelitis, acute disseminated encephalomyelitis, mild encephalitis with reversible splenial syndrome (MERS), and Guillaín Barré syndrome. We report a 16-year-old female who was admitted at our emergency department with seizures, confusion, nystagmus and motor clumsiness five days after an upper a respiratory tract infection. Influenza type B virus infection was confirmed by chain polymerase reaction analysis. Read More

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http://dx.doi.org/10.4067/S0034-98872019000700922DOI Listing

Overlapping autoimmune syndrome: A case of concomitant anti-NMDAR encephalitis and myelin oligodendrocyte glycoprotein (MOG) antibody disease.

J Neuroimmunol 2020 Feb 6;339:577124. Epub 2019 Dec 6.

Division of Multiple Sclerosis and Neuroimmunology, Department of Neurology, University of Texas Health Science Center at Houston, Houston, TX, USA.

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder of the central nervous system that commonly manifests as a complex neuropsychiatric syndrome. Antibodies against myelin oligodendrocyte glycoprotein (MOG) are associated with a range of clinical presentations including acute disseminated encephalomyelitis (ADEM), optic neuritis, and transverse myelitis. The concurrence of NMDAR encephalitis and demyelinating syndromes is rare. Read More

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http://dx.doi.org/10.1016/j.jneuroim.2019.577124DOI Listing
February 2020
2.467 Impact Factor