2,788 results match your criteria Acute Disseminated Encephalomyelitis


How should we diagnose acute disseminated encephalomyelitis?

Authors:
Todd A Hardy

Dev Med Child Neurol 2018 Jun 21. Epub 2018 Jun 21.

Brain & Mind Centre, University of Sydney, Sydney, NSW, Australia.

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Acute Disseminated Encephalomyelitis-Masquerading as Pediatric Stroke: Case Report.

J Pediatr Neurosci 2018 Jan-Mar;13(1):71-73

Department of Pediatrics, Mahatma Gandhi Institute of Medical Sciences (MGIMS), Sevagram, Wardha, Maharashtra, India.

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. Read More

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June 2018
1 Read

Transmission of rabies through solid organ transplantation: a notable problem in China.

BMC Infect Dis 2018 Jun 14;18(1):273. Epub 2018 Jun 14.

Beijing Friendship Hospital, Capital Medical University, Beijing, China.

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. The incubation periods were 44, 48, 158 and 303 days. Read More

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Interleukin-6, S-Nitrosothiols, and Neurodegeneration in Different Central Nervous System Demyelinating Disorders: Is There a Relationship?

J Clin Neurol 2018 05 31. Epub 2018 May 31.

Institute of Higher Nervous Activity and Neurophysiology Russian Academy of Sciences, Department of Functional Biochemistry of the Nervous System, Moscow, Russia.

Background And Purpose: A few groups have suggested that activated cytokines and nitrosative stress are closely involved in the pathogenesis of different demyelinating disorders induced by the neuroinflammatory destruction of neurons. The purpose of this study was to elucidate the associations of cytokines and S-nitrosothiols (RSNO) with the severity of neurodegeneration during relapse in demyelinating disorders of the central nervous system.

Methods: We measured levels of interleukin-6 (IL-6), erythropoietin, RSNO, and phosphorylated neurofilament heavy chain (pNfh) in cerebrospinal fluid (CSF) samples obtained from patients with different demyelinating disorders: multiple sclerosis (MS, n=52), acute disseminated encephalomyelitis (ADEM, n=9), and neuromyelitis optica spectrum disorders (NMOSD) with aquaporin-4 immunoglobulin G (AQP4-IgG, n=12). Read More

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May 2018
2 Reads

Unusual case of cerebral demyelination and bilateral optic neuritis in an infant with suppurative BCG lymphadenitis.

BMJ Case Rep 2018 May 30;2018. Epub 2018 May 30.

Paediatric Neurology, Hospital Kuala Lumpur, Kuala Lumpur, Malaysia.

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. Read More

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May 2018
2 Reads

Relapsing acute disseminated encephalomyelitis followed by optic neuritis in children; a clinical entity associated with anti-MOG antibody.

Authors:
F Brilot

Eur J Neurol 2018 May 23. Epub 2018 May 23.

Brain Autoimmunity Group, Kids Neuroscience Centre, Kids Research at the Children's Hospital at Westmead, Sydney, NSW, Australia.

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Vaccines and the risk of acute disseminated encephalomyelitis.

Vaccine 2018 06 18;36(26):3733-3739. Epub 2018 May 18.

Department of Expanded Program on Immunization, Jiangsu Provincial Center for Disease Control and Prevention, China. Electronic address:

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. Read More

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June 2018
1 Read

Update on pediatric-onset multiple sclerosis.

Authors:
A Ruet

Rev Neurol (Paris) 2018 Jun 19;174(6):398-407. Epub 2018 May 19.

University of Bordeaux, 146, rue Léo Saignat, 33076 Bordeaux cedex, France; Inserm U1215, neurocentre Magendie, 146, rue Léo Saignat, 33000 Bordeaux, France; Hospital of Bordeaux, place Amélie Raba Léon, 33076 Bordeaux cedex, France. Electronic address:

Pediatric-onset multiple sclerosis (POMS) has distinctive features compared with adult-onset multiple sclerosis (AOMS), and warrants caution despite being a rare form of MS. POMS diagnostic criteria are somewhat different from those used in AOMS, with acute disseminated encephalomyelitis being a key differential diagnosis of MS in children. Other differential diagnoses that have to be ruled out before diagnosing MS include demyelinating syndromes, autoimmune and systemic pathologies, and infectious, genetic, metabolic and neoplastic diseases. Read More

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Implications of the International Paediatric Multiple Sclerosis Study Group consensus criteria for paediatric acute disseminated encephalomyelitis: a nationwide validation study.

Dev Med Child Neurol 2018 May 10. Epub 2018 May 10.

Department of Paediatrics, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.

Aim: The International Paediatric Multiple Sclerosis Study Group (IPMSSG) has proposed criteria for acute disseminated encephalomyelitis (ADEM) not evaluated in clinical practice. Our objective was to assess epidemiological implications of the IPMSSG criteria for ADEM in a cohort study using prospectively collected data.

Method: We identified all diagnosed cases of ADEM in Denmark between 2008 and 2015 from the Danish National Patient Register by International Classification of Diseases 10 codes assigned to acute demyelinating episodes, and we reviewed all medical records to validate ADEM. Read More

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Pediatric multiple sclerosis and fulminant disease course: Features and approaches to treatment - A case report and review of the literature.

J Clin Neurosci 2018 Jul 3;53:13-19. Epub 2018 May 3.

Department of Neurology, University of Szeged, Szeged, Hungary. Electronic address:

Multiple sclerosis (MS) is the autoimmune, neurodegenerative disease of the central nervous system (CNS). Typically, it affects the young adult population, however, up to 10% of the cases, it can develop in childhood. Atypical manifestations, such as the tumefactive variant (tMS) or acute disseminated encephalomyelitis (ADEM), especially coupled with fulminant disease course, are even more rare and pose a considerable differential diagnostic and therapeutic challenge. Read More

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July 2018
1 Read

MOG encephalomyelitis: international recommendations on diagnosis and antibody testing.

J Neuroinflammation 2018 May 3;15(1):134. Epub 2018 May 3.

Molecular Neuroimmunology Group, Department of Neurology, University Hospital Heidelberg, Im Neuenheimer Feld 350, 69120, Heidelberg, Germany.

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). Owing to a substantial overlap in clinicoradiological presentation, MOG-EM was often unwittingly misdiagnosed as MS in the past. Read More

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[A case of acute disseminated encephalomyelitis concomitant with polyneuropathy associated with anti-lactosylceramide antibody].

Rinsho Shinkeigaku 2018 May 28;58(5):297-301. Epub 2018 Apr 28.

Department of Neurology, Fujita Health University School of Medicine.

We report a case of acute disseminated encephalomyelitis (ADEM) concomitant with polyneuropathy associated with anti-lactosylceramide antibody. A 68-year-old man was admitted to our hospital with ophthalmoparesis, bulbar palsy, tetraplegia after suffering from upper respiratory infection and headache. Subsequently, he developed respiratory failure requiring mechanical ventilation. Read More

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May 2018
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Pediatric acquired demyelinating syndromes: a nationwide validation study of the Danish National Patient Register.

Clin Epidemiol 2018 10;10:391-399. Epub 2018 Apr 10.

National Institute of Public Health, University of Southern Denmark, Copenhagen, Denmark.

Objective: To validate the Danish National Patient Register's (NPR) diagnoses of pediatric acquired demyelinating syndromes (ADS) including multiple sclerosis (MS).

Study Design And Setting: We identified ADS diagnostic groups using International Classification of Diseases (ICD) codes and reviewed medical records to validate the NPR diagnoses during 2008-2015.

Results: Among 409 children in the study, 184 children had a validated and final ADS diagnosis after reviewing medical records as follows: optic neuritis (ON; n=46), transverse myelitis (TM; n=16), acute disseminated encephalomyelitis (ADEM; n=50), clinically isolated syndrome (CIS) including dissemination in space (CIS [DIS]) but not dissemination in time (n=6), neuromyelitis optica spectrum disorder (NMOsd; n=5), and MS (n=61). Read More

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Susac's Syndrome (Retinocochleocerebral Vasculopathy): Follow-up of a Pediatric Patient.

J Pediatr Neurosci 2017 Oct-Dec;12(4):374-377

Department of Pediatric Neurology, Ondokuz Mayis University School of Medicine, Samsun, Turkey.

Susac's syndrome (SS) is a triad of encephalopathy, branch retinal artery occlusion (BRAO), and sensorineural hearing loss as a result of microvascular occlusions of the brain, retina, and inner ear. It is also a disorder of autoimmune endotheliopathy. SS usually affects young women between the age of 20 and 40 years. Read More

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April 2018
1 Read

Classification and diagnostic criteria for demyelinating diseases of the central nervous system: Where do we stand today?

Rev Neurol (Paris) 2018 Jun 16;174(6):378-390. Epub 2018 Apr 16.

Neurology Unit, University Hospital of Nancy, Hôpital Central, 29 avenue du Maréchal de Lattre de Tassigny, 54035 Nancy Cedex, France.

The diagnosis of multiple sclerosis (MS) and other demyelinating diseases of the central nervous system is challenging, and although the currently available biological and imaging tools offer considerable support to physicians, these tools often fail to provide a simple and final answer at the time of a first event. Thus, sets of diagnostic criteria have been published and tested on patient cohorts, and are now used in clinical trials and in daily clinical practice. These criteria have evolved over time to take into account physicians' and patients' needs, along with emerging paraclinical tests. Read More

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The neurological complications of chikungunya virus: A systematic review.

Rev Med Virol 2018 May 19;28(3):e1978. Epub 2018 Apr 19.

National Institute for Health Research Health Protection Research Unit in Emerging and Zoonotic Infections, University of Liverpool, Liverpool, UK.

We performed a systematic review on the neurological complications of chikungunya virus. Such complications are being reported increasingly, owing primarily to the scale of recent epidemics but also to a growing understanding of the virus' neurovirulence. We performed a thorough literature search using PubMed and Scopus databases, summating the data on all published reports of neurological disease associated with chikungunya virus. Read More

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Advances in Biomarker-Guided Therapy for Pediatric- and Adult-Onset Neuroinflammatory Disorders: Targeting Chemokines/Cytokines.

Front Immunol 2018 4;9:557. Epub 2018 Apr 4.

National Pediatric Neuroinflammation Organization, Inc., Orlando, FL, United States.

The concept and recognized components of "neuroinflammation" are expanding at the intersection of neurobiology and immunobiology. Chemokines (CKs), no longer merely necessary for immune cell trafficking and positioning, have multiple physiologic, developmental, and modulatory functionalities in the central nervous system (CNS) through neuron-glia interactions and other mechanisms affecting neurotransmission. They issue the "help me" cry of neurons and astrocytes in response to CNS injury, engaging invading lymphoid cells (T cells and B cells) and myeloid cells (dendritic cells, monocytes, and neutrophils) (adaptive immunity), as well as microglia and macrophages (innate immunity), in a cascade of events, some beneficial (reparative), others destructive (excitotoxic). Read More

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April 2018
5 Reads

MOG-IgG-Associated Optic Neuritis, Encephalitis, and Myelitis: Lessons Learned From Neuromyelitis Optica Spectrum Disorder.

Front Neurol 2018 4;9:217. Epub 2018 Apr 4.

School of Medicine, Brain Institute of Rio Grande do Sul (BraIns), Pontifical Catholic University of Rio Grande do Sul (PUCRS), Porto Alegre, Brazil.

Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been found in some cases diagnosed as seronegative neuromyelitis optica spectrum disorder (NMOSD). MOG-IgG allowed the identification of a subgroup with a clinical course distinct from that of NMOSD patients who are seropositive for aquaporin-4-IgG antibodies. MOG-IgG is associated with a wider clinical phenotype, not limited to NMOSD, with the majority of cases presenting with optic neuritis (ON), encephalitis with brain demyelinating lesions, and/or myelitis. Read More

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April 2018
4 Reads

Cerebral vasculitis and lateral rectus palsy - two rare central nervous system complications of dengue fever: two case reports and review of the literature.

J Med Case Rep 2018 Apr 19;12(1):100. Epub 2018 Apr 19.

Department of Microbiology, Faculty of Medicine, University of Ruhuna, Galle, Sri Lanka.

Background: Dengue fever is a common mosquito-borne viral illness with a clinical spectrum ranging from a simple febrile illness to potentially life-threatening complications such as dengue hemorrhagic fever and dengue shock syndrome. Dengue infection can affect many organs, including the central nervous system. The neurological manifestations reported in dengue infections are meningitis, encephalitis, stroke, acute disseminated encephalomyelitis, and Guillain-Barré syndrome. Read More

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April 2018
1 Read

Neurological complications of Zika virus infection.

Expert Rev Anti Infect Ther 2018 May 26;16(5):399-410. Epub 2018 Apr 26.

a Neurology Department , Raigmore Hospital , Inverness , UK.

Introduction: Zika virus (ZIKV) disease is a vector-borne infectious disease transmitted by Aedes mosquitoes. Recently, ZIKV has caused outbreaks in most American countries. Areas covered: Publications about neurological complications of ZIKV infection retrieved from pubmed searchers were reviewed, and reference lists and relevant articles from review articles were also examined. Read More

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May 2018
10 Reads

Rituximab was effective for acute disseminated encephalomyelitis followed by recurrent optic neuritis with anti-myelin oligodendrocyte glycoprotein antibodies.

Brain Dev 2018 Apr 13. Epub 2018 Apr 13.

Department of Pediatrics, Jichi Medical University, Japan.

Background: The effect of rituximab on acute disseminated encephalomyelitis (ADEM) followed by recurrent optic neuritis (ON) is not yet known.

Patient: We are reporting the case of a 4-year-old Japanese girl who was diagnosed with anti-myelin oligodendrocyte glycoprotein (MOG) antibody positive ADEM followed by recurrent ON. She developed altered mental status, left facial paralysis, left paresis, and experienced three episodes of ON. Read More

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Encephalomyelitis After Varicella Zoster Reactivation in an Immunocompromised Patient.

Neurohospitalist 2018 Apr 11;8(2):NP1-NP2. Epub 2017 May 11.

Neuroradiology, Department of Radiology and Biomedical Imaging, University of California San Francisco, San Francisco, CA, USA.

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April 2018
1 Read

Acute Hemorrhagic Encephalitis Responding to Combined Decompressive Craniectomy, Intravenous Immunoglobulin, and Corticosteroid Therapies: Association with Novel Variant.

Front Neurol 2018 12;9:130. Epub 2018 Mar 12.

Division of Pediatric Neurology, Department of Pediatrics, Montreal Children's Hospital, Montreal, QC, Canada.

Background: Acute hemorrhagic encephalomyelitis (AHEM) is considered as a rare form of acute disseminated encephalomyelitis characterized by fulminant encephalopathy with hemorrhagic necrosis and most often fatal outcome.

Objective: To report the association with Ran Binding Protein ( gene variant and the response to decompressive craniectomy and high-dose intravenous methylprednisolone (IVMP) in life-threatening AHEM.

Design: Single case study. Read More

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March 2018
4 Reads

Acute Disseminated Encephalomyelitis After Herpes Simplex Virus Meningoencephalitis in Adult.

Am J Med 2018 Mar 23. Epub 2018 Mar 23.

Department of Endocrinology, Mitoyo General Hospital, Kanonji-city, Kagawa, Japan.

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March 2018
1 Read

Acute disseminated encephalomyelitis, a rare post-malaria neurological complication: Case report and review of the literature.

Travel Med Infect Dis 2018 Mar 21. Epub 2018 Mar 21.

Department of Infectious Diseases, Centro Hospitalar de Setúbal, Setúbal, Portugal.

There are four neurological complications that can occur after malaria treatment at a time when the patient is aparasitaemic: delayed cerebellar ataxia, acute inflammatory demyelinating polyneuropathy, post-malaria neurological syndrome and acute disseminated encephalomyelitis (ADEM). The authors describe a case of a 54-year-old male who presented with encephalopathy and generalized seizures forty-three days after complete recovery from acute malaria by Plasmodium falciparum. Diagnosis of post-malaria ADEM was made based on the acute onset of the neurological symptoms, characteristic findings in magnetic resonance imaging of the brain and prompt response to steroid therapy. Read More

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March 2018
4 Reads

Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies in patients with optic neuritis and seizures.

J Neurol Sci 2018 Apr 2;387:170-173. Epub 2018 Feb 2.

Department of Neurology, NYU Langone Health, New York, NY, USA.

We describe four patients who experienced optic neuritis (ON) and seizures and were found to have antibodies to myelin oligodendrocyte glycoprotein (MOG) in serum. The index case was a previously healthy 39-year-old man who developed steroid dependent ON and had a generalized seizure when steroids were tapered. He tested positive for antibodies to MOG. Read More

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April 2018
1 Read

Microcephaly caused by congenital Zika virus infection and viral detection in maternal urine during pregnancy.

Rev Assoc Med Bras (1992) 2018 Jan;64(1):11-14

Department of Radiology and Medical Imaging, Documenta - Hospital Materno Infantil Sinhá Junqueira, Ribeirão Preto, SP, Brazil.

Currently Latin America is undergoing a major epidemic of Zika virus, which is transmitted by Aedes mosquitoes. Concern for Zika virus infection has been increasing as it is suspected of causing brain defects in newborns such as microcephaly and, more recently, potential neurological and autoimmune complications including Guillian-Barré syndrome and acute disseminated encephalomyelitis. We describe a case of virus infection in a 25-year-old woman during the first trimester of her pregnancy, confirmed by laboratory tests only for the detection of viral particles in maternal urine, with imaging studies demonstrating the progression of cranial and encephalic changes in the fetus and later in the newborn, such as head circumference reduction, cerebral calcifications and ventriculomegaly. Read More

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January 2018
3 Reads

Use of Cyclophosphamide in a Child With Fulminant Acute Disseminated Encephalomyelitis.

Child Neurol Open 2018 11;5:2329048X18754631. Epub 2018 Mar 11.

Neuroscience department, King Fahad Special Hospital-Dammam, Al-Khobar, Dammam, Saudi Arabia.

Acute disseminated encephalomyelitis is an immune-mediated inflammatory demyelinating disorder of the central nervous system. The first-line treatment is usually high-dose intravenous methylprednisolone. Intravenous immunoglobulin and plasmapheresis have also shown to be beneficial. Read More

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March 2018
1 Read

Optic neuritis in paediatric patients: Experience over 27 years and a management protocol.

Neurologia 2018 Mar 8. Epub 2018 Mar 8.

Sección de Neuropediatría, Hospital Infantil Universitario Miguel Servet, Zaragoza, España.

Introduction And Objective: In this article, we present our experience on optic neuritis (ON) and provide a diagnostic/therapeutic protocol, intended to rule out other aetiologies (particularly infection), and a fact sheet for parents.

Material And Methods: We conducted a descriptive, retrospective study of patients with ON over a 27-year period (1990-2017). A review of the available scientific evidence was performed in order to draft the protocol and fact sheet. Read More

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March 2018
1 Read

Pediatric multiple sclerosis: a review.

BMC Neurol 2018 Mar 9;18(1):27. Epub 2018 Mar 9.

Department of Neurology, Neurosurgery and Medical Genetic of the Pirogov's Russian National Research Medical University and MS Clinic at the Usupov's Hospital, Ostrovitianov str. 1, Moscow, 117997, Russia.

Background: Pediatric-onset multiple sclerosis (POMS) prevalence and incidence rates are increasing globally. No disease-modifying therapy are approved for MS pediatric population. Hence, we aim to review the literature on POMS to guide treating physicians on the current understanding of diagnosis and management of pediatric MS. Read More

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March 2018
11 Reads

Cerebrospinal fluid γδ T cell frequency is age-related: a case-control study of 435 children with inflammatory and non-inflammatory neurological disorders.

Clin Exp Immunol 2018 Feb 27. Epub 2018 Feb 27.

National Pediatric Myoclonus Center, Orlando, FL, USA.

Studies of cerebrospinal fluid (CSF) γδ T cells in children are limited, due especially to the lack of control data. In adults, gamma/delta T cells (TCR-γδ) residing in the intrathecal space are sometimes involved in neuroinflammation. To evaluate the possible role of γδ T cells in paediatric neuroinflammation, we immunophenotyped cerebrospinal fluid (CSF) and blood lymphocytes using flow cytometry in a case-control study of 100 children with non-inflammatory neurological disorders (NIND), 312 with opsoclonus-myoclonus (OMS) and 23 with other inflammatory neurological disorders (OIND). Read More

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February 2018
2 Reads

[Acute Urinary Retention Caused by Acute Disseminated Encephalomyelitis : A Case Report].

Hinyokika Kiyo 2018 Jan;64(1):17-20

The Department of Urology, Nara Prefecture General Medical Center.

An 11-year-old boy was referred to our department with the chief complaint of acute urinary retention. He had had a history of viral enteritis a few days before the onset of dysuria. He presented with a slight fever, mild headache and weakness of the extremities. Read More

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January 2018
1 Read

Myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies are highly specific in children with acquired demyelinating syndromes.

Dev Med Child Neurol 2018 Feb 22. Epub 2018 Feb 22.

Department of Paediatric Neurology, Great Ormond Street Hospital for Children, London, UK.

Aim: Our objectives were to evaluate the utility of measuring myelin oligodendrocyte glycoprotein (MOG) and aquaporin-4 (AQP4) antibodies (Ab) in clinical practice and describe their associated neurological phenotypes in children.

Method: Between 2012 and 2017, 371 children with suspected acquired demyelinating syndromes (ADS) seen in three tertiary centres were tested for MOG-Ab and AQP4-Ab. Medical notes were retrospectively reviewed, and clinical and demographic data compiled. Read More

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February 2018
3 Reads

Acute disseminated encephalomyelitis following Influenza A pneumonia.

Clin Case Rep 2018 Feb 22;6(2):436-438. Epub 2017 Dec 22.

Internal Medicine DepartmentHurley Medical Center/Michigan State UniversityTwo Hurley Plaza, Ste 212FlintMichigan48503.

Acute disseminated encephalomyelitis (ADEM) is an autoimmune demyelinating disorder of the central nervous system and can present following influenza A infections as multifocal neurological deficits. ADEM remains a challenging diagnosis, and high clinical suspicious coupled with laboratory investigations and neuroimaging is required to exclude other primary and secondary demyelinating disorders. Read More

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February 2018
1 Read

Paediatric acute disseminated encephalomyelitis followed by optic neuritis: disease course, treatment response and outcome.

Eur J Neurol 2018 May 9;25(5):782-786. Epub 2018 Mar 9.

Department of Paediatric Neurology, Erasmus MC-Sophia, Rotterdam, The Netherlands.

Background And Purpose: Acute disseminated encephalomyelitis followed by optic neuritis (ADEM-ON) is a rare demyelinating syndrome that is different from multiple sclerosis and neuromyelitis optica spectrum disorder. The aim of this study was to describe the disease course, treatment response and outcome of children with ADEM-ON.

Methods: Children of <18 years of age were identified from six countries of the EU Paediatric Demyelinating Disease Consortium. Read More

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May 2018
2 Reads

MRI of the first event in pediatric acquired demyelinating syndromes with antibodies to myelin oligodendrocyte glycoprotein.

J Neurol 2018 Apr 8;265(4):845-855. Epub 2018 Feb 8.

Department of Pediatric Neurology, Children's Hospital Datteln, Witten/Herdecke University, Datteln, Germany.

Antibodies against the myelin oligodendrocyte glycoprotein (MOG-Ab) can be detected in various pediatric acquired demyelinating syndromes (ADS). Here, we analyze the spectrum of neuroradiologic findings in children with MOG-Ab and a first demyelinating event. The cerebral and spinal MRI of 69 children with different ADS was assessed in regard to the distribution and characteristics of lesions. Read More

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April 2018
8 Reads

Differences in the Reponses to Apheresis Therapy of Patients With 3 Histopathologically Classified Immunopathological Patterns of Multiple Sclerosis.

JAMA Neurol 2018 Apr;75(4):428-435

Institute of Neuropathology, University Medical Center Goettingen, Goettingen, Germany.

Importance: Plasma exchange and immunoadsorption are second-line apheresis therapies for patients experiencing multiple sclerosis relapses. Early active multiple sclerosis lesions can be classified into different histopathological patterns of demyelination. Pattern 1 and 2 lesions show T-cell- and macrophage-associated demyelination, and pattern 2 is selectively associated with immunoglobulin and complement deposits, suggesting a humoral immune response. Read More

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April 2018
1 Read

Disease Course and Treatment Responses in Children With Relapsing Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.

JAMA Neurol 2018 Apr;75(4):478-487

Children's Neurosciences, Evelina London Children's Hospital at Guy's and St Thomas' National Health Service Foundation Trust, King's Health Partners Academic Health Science Centre, London, United Kingdom.

Importance: Myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) are consistently identified in a range of demyelinating disorders in adults and children. Current therapeutic strategies are largely center specific, and no treatments have been formally evaluated.

Objective: To examine the clinical phenotypes, treatment responses, and outcomes of children with relapsing MOG-Ab-associated disease. Read More

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April 2018
8 Reads

'Leukodystrophy-like' phenotype in children with myelin oligodendrocyte glycoprotein antibody-associated disease.

Dev Med Child Neurol 2018 04 30;60(4):417-423. Epub 2017 Dec 30.

Department of Paediatric Neurology, Great Ormond Street Hospital for Children, London, UK.

Aim: To review the demographics and clinical and paraclinical parameters of children with myelin oligodendrocyte glycoprotein (MOG) antibody-associated relapsing disease.

Method: In this UK-based, multicentre study, 31 children with MOG antibody-associated relapsing disease were studied retrospectively.

Results: Of the 31 children studied, 14 presented with acute disseminated encephalomyelitis (ADEM); they were younger (mean 4. Read More

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April 2018
6 Reads

Chemokines CXCL10, CXCL11, and CXCL13 in acute disseminated encephalomyelitis, non-polio enterovirus aseptic meningitis, and neuroborreliosis: CXCL10 as initial discriminator in diagnostic algorithm?

Neurol Sci 2018 Mar 29;39(3):471-479. Epub 2017 Dec 29.

Pediatric Infectious Diseases Department, University Hospital for Infectious Diseases "dr.Fran Mihaljevic", Mirogojska 8, 10 000, Zagreb, Croatia.

We investigated potential diagnostic usefulness of serum and cerebrospinal fluid (CSF) concentrations of chemokines CXCL10, CXCL11, and CXCL13 in pediatric patients with acute disseminated encephalomyelitis (ADEM) (n = 23), non-polio enterovirus aseptic meningitis (NPEV AM) (n = 20), and neuroborreliosis (NB) (n = 21) and children with acute infectious diseases with neurological symptoms but with excluded neuroinfection/neuroinflammation (controls, n = 20). CSF levels of CXCL10 and CXCL11 were higher in patients with NPEV AM than those in other children, and CXCL10 levels showed a high discriminative potential (area under the receiver operating characteristic curve, ROC, 0.982) with high specificity and sensitivity (both 95%). Read More

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March 2018
11 Reads

Unihemispheric central nervous system vasculitis.

eNeurologicalSci 2017 Jun 13;7:44-48. Epub 2017 May 13.

Neurological Institute, University Hospitals Cleveland Medical Center, 11100 Euclid Ave, Cleveland, OH 44106, USA.

Patients with primary central nervous system vasculitis (PCNSV) usually manifest with multiple enhancing bilateral hemispheric lesions. We presented an extremely rare clinical course and follow-up of a patient with PCNSV affecting only a single (right) hemisphere. A 33-year-old previously healthy man presented with a left hand clonic seizure followed by a secondary generalized tonic-clonic seizure and dysarthria. Read More

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June 2017
3 Reads

Necrotic-hemorrhagic myelitis: A rare malignant variant of parainfectious acute disseminated encephalomyelitis in childhood.

J Neurol Sci 2018 Jan 23;384:58-60. Epub 2017 Nov 23.

Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy; Department of Neurology, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy. Electronic address:

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January 2018
5 Reads

X-linked Charcot-Marie-Tooth disease with GJB1 mutation presenting as acute disseminated encephalomyelitis-like illness: A case report.

Medicine (Baltimore) 2017 Dec;96(49):e9176

Department of Pediatrics, Chonbuk National University School of Medicine, Jeonju, Korea.

Rationale: Charcot-Marie-Tooth disease (CMT) is typically an autosomal dominant, inherited neuropathy, although there is a rare male X-linked CMT. Such patients show central nervous system (CNS) involvement in addition to peripheral neuropathy. Recently, we encountered a patient who presented with acute disseminated encephalomyelitis (ADEM)-like symptoms, but was later diagnosed as having X-linked CMT (CMTX) due to a mutation. Read More

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December 2017
8 Reads

Childhood acute disseminated encephalomyelitis: an Egyptian pilot study.

Acta Neurol Belg 2017 Dec 8. Epub 2017 Dec 8.

Faculty of Medicine, Paediatrics Department, Ain Shams University, Cairo, Egypt.

Describing the variable clinical features, laboratory findings, neuroimaging findings, and treatments given to children who presented with ADEM and following them up both clinically and radiologically. 21 patients were recruited: 14 new cases, and 7 old ones presenting over the preceding 5 years (retrospective review of existing data). 11 males and 10 females, with a mean age of 4. Read More

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December 2017
5 Reads

Imaging of Acquired Demyelinating Syndrome With 18F-FDG PET/CT.

Clin Nucl Med 2018 Feb;43(2):103-105

Acquired demyelinating syndromes include acute disseminated encephalomyelitis, transverse myelitis and may progress to multiple sclerosis (MS). Acute disseminated encephalomyelitis is characterized by impairment of level of consciousness and multifocal neurological deficits and transverse myelitis by back pain, weakness and sphincter dysfunction. Only a few cases of acquired demyelinating syndrome have been imaged with F-FDG PET/CT. Read More

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February 2018
4 Reads

Diffuse glioblastoma resembling acute hemorrhagic leukoencephalitis.

Quant Imaging Med Surg 2017 Oct;7(5):592-597

Unit of Neuroradiology, Department of Advanced Biomedical Sciences, "Federico II" University, Naples, Italy.

We report the case of a young man with sudden onset of diplopia after an upper respiratory tract infection. Based on the first radiological findings acute hemorrhagic leukoencephalitis, a variant of acute disseminated encephalomyelitis, was suspected and treatment with high dose intravenous dexamethasone was started but it was stopped for intolerance. The patient clinically worsened, developing gait instability, ataxia and ophthalmoplegia; brain MRI performed 20 days later showed severe progression of the disease with subependymal dissemination. Read More

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October 2017
5 Reads

Acute disseminated encephalomyelitis associated with debut of a late onset coeliac disease-a case report.

Neurol Sci 2018 Apr 27;39(4):799-801. Epub 2017 Nov 27.

Department of Neurology, Akershus University Hospital, PO Box 1000, 1478, Lørenskog, Norway.

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April 2018
3 Reads

Diffusion Tensor Imaging as a Biomarker to Differentiate Acute Disseminated Encephalomyelitis From Multiple Sclerosis at First Demyelination.

Pediatr Neurol 2018 Jan 28;78:70-74. Epub 2017 Sep 28.

Department of Pediatric and Developmental Neurology, Washington University School of Medicine, St. Louis, Missouri. Electronic address:

Background: There are no clinical features or biomarkers that can reliably differentiate acute disseminated encephalomyelitis from multiple sclerosis at the first demyelination attack. Consequently, a final diagnosis is sometimes delayed by months and years of follow-up. Early treatment for multiple sclerosis is recommended to reduce long-term disability. Read More

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January 2018
3 Reads

Biomarkers in the evolution of multiple sclerosis.

Authors:
Thomas Berger

Neurodegener Dis Manag 2017 Nov;7(6s):3-6

Clinical Department of Neurology, Medical University of Innsbruck, Anichstrasse 35, 6020 Innsbruck, Austria.

Nonimaging biomarkers can be applied in differential diagnosis, evaluation of disease progression and therapy monitoring of multiple sclerosis (MS). Presence of oligoclonal IgG bands in cerebrospinal fluid is a diagnostic element and a negative predictor of MS evolution. AQP4 antibodies are pathogenic and diagnostic for neuromyelitis optica spectrum disorder. Read More

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November 2017
3 Reads

Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination.

J Neurol Neurosurg Psychiatry 2018 Feb 15;89(2):127-137. Epub 2017 Nov 15.

TY Nelson Department of Neurology and Neurosurgery, Children's Hospital, Westmead, New South Wales, Australia.

Objective: We characterised the clinical course, treatment and outcomes in 59 patients with relapsing myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination.

Methods: We evaluated clinical phenotypes, annualised relapse rates (ARR) prior and on immunotherapy and Expanded Disability Status Scale (EDSS), in 218 demyelinating episodes from 33 paediatric and 26 adult patients.

Results: The most common initial presentation in the cohort was optic neuritis (ON) in 54% (bilateral (BON) 32%, unilateral (UON) 22%), followed by acute disseminated encephalomyelitis (ADEM) (20%), which occurred exclusively in children. Read More

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February 2018
5 Reads