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Pediatr Emerg Care 2019 Apr 9. Epub 2019 Apr 9.

Objective: Our aim is to emphasize the varied presentation of acute disseminated encephalomyelitis (ADEM) to help health care professionals improve recognition of the disease in a timely manner, thereby allowing for the selection of an appropriate treatment regimen. Therefore, this may avoid neurocognitive consequences and the ultimate fatality of the patient.

Patients And Methods: This is a retrospective case series involving 7 cases of children presenting to the Pediatric Emergency Department of Hackensack University Medical Center who were ultimately diagnosed with ADEM. Read More

Front Neurol 2019 20;10:270. Epub 2019 Mar 20.

Department of Pediatrics, New York University School of Medicine, New York, NY, United States.

Longitudinally extensive spinal cord lesions (LECL) restricted to gray matter are poorly understood as are their neurodevelopmental repercussions in children. We herein report the critical case of a 13-year-old male presenting with progressive quadriparesis found to have cervical LECL restricted to the anterior horns. Challenged with a rare diagnostic dilemma, the clinical team systematically worked through potential vascular, genetic, infectious, rheumatologic, and paraneoplastic diagnoses before assigning a working diagnosis of acute inflammatory myelopathy. Read More

Mult Scler Relat Disord 2019 Mar 26;31:62-64. Epub 2019 Mar 26.

Department of Neurology and Clinical Pharmacology, Ehime University Graduate School of Medicine, Ehime, Japan.

A 20-year-old woman suffered right facial paralysis. The patient showed an abnormality in the perception of speech at an age of 25 years. At an age of 32 years, she developed acute headache and fever. Read More

J Family Med Prim Care 2019 Jan;8(1):292-295

Health Centre, Central University of Tamil Nadu, Thiruvarur, Tamil Nadu, India.

Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory demyelinating disease of central nervous system (CNS), characterized by multifocal white matter involvement with neurological deficits and accompanied by encephalopathy. ADEM is thought to be caused by autoimmune etiology. CNS autoantigens are produced by molecular mimicry triggered by an environmental stimulus, mostly infection (viral/bacterial) or post vaccination, in genetically susceptible individuals. Read More

Zhongguo Dang Dai Er Ke Za Zhi 2019 Mar;21(3):223-228

Department of Neurology, Children's Hospital of Chongqing Medical University/Ministry of Education Key Laboratory of Child Development and Disorders/China International Science and Technology Cooperation Base of Child Development and Critical Disorders/Chongqing Key Laboratory of Pediatrics, Chongqing 400014, China.

Objective: To study the clinical features of children with acute disseminated encephalomyelitis (ADEM) and related recurrence factors.

Methods: A retrospective analysis was performed for the clinical data and prognosis of 73 children with ADEM who were hospitalized from November 2011 to January 2017.

Results: Among the 73 children, 41 (56%) had a history of infection before onset and 7 (10%) had a history of vaccination. Read More

Mult Scler 2019 Mar 25:1352458519837705. Epub 2019 Mar 25.

Department of Neurology, Johns Hopkins University, Baltimore, MD, USA/Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.

Myelin oligodendrocyte glycoprotein (MOG) antibody disease is a rare autoimmune disorder with antibodies against the MOG predominantly involving the optic nerve and spinal cord leading to vision loss and paralysis. When MOG antibody disease involves the brain, the phenotype is similar to acute disseminated encephalomyelitis (ADEM). In this review, we discuss MOG-positive cases presenting with encephalitis, encephalopathy, or ADEM-like presentation based on recently published series. Read More

J Neurol Sci 2019 May 16;400:83-89. Epub 2019 Mar 16.

Department of Ophthalmology, Chinese PLA General Hospital, Fuxing Road No.28, Haidian district, Beijing 100853, China. Electronic address:

Purpose: To investigate the clinical characteristics and outcomes of myelin oligodendrocyte glycoprotein antibody-seropositive optic neuritis (MOG-ON) in patients with varying ages of onset in China.

Methods: Patients displaying symptoms of MOG-ON were recruited from the Neuro-ophthalmology Department in the Chinese People's Liberation Army General Hospital from January 2016 to May 2018. They were assigned to one of three subgroups based on age of onset: pediatric (<18 years), young (18-46 years), and middle-aged (>46 years) MOG-ON. Read More

Am J Case Rep 2019 Mar 19;20:361-365. Epub 2019 Mar 19.

Department of Internal Medicine, Texas Tech University Health Science Center at the Permian Basin, Odessa, TX, USA.

BACKGROUND Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease that usually presents in pediatric patients, usually following a viral or bacterial infection. The clinical findings in ADEM include acute neurologic decline that typically presents with encephalopathy, with some cases progressing to multiple sclerosis. An atypical case of ADEM is reported that presented in a middle-aged adult. Read More

Front Immunol 2019 27;10:324. Epub 2019 Feb 27.

Chongqing Key Laboratory of Neurology, Department of Neurology, the First Affiliated Hospital of Chongqing Medical University, Chongqing, China.

Plasma exchange has been widely used in autoimmune neurological diseases and is the standard treatment for myasthenia gravis crisis and Guillain-Barre syndrome. A growing body of research suggests that, in the clinical application of steroid-responsive encephalopathy, such as for Hashimoto's encephalopathy, limbic encephalitis, systemic lupus erythematosus encephalopathy, ANCA-associated vasculitis encephalopathy, and acute disseminated encephalomyelitis, plasma exchange is a safe, and effective option when steroids or other immunosuppressive therapies are ineffective in the short term or when contraindications are present. Additionally, plasma exchange can also be used alone or in combination with steroids, immunoglobulins, or other immunosuppressive agents to treat steroid-responsive encephalopathy. Read More

Ugeskr Laeger 2019 Mar;181(11)

Acquired demyelinating syndromes are inflammatory demyelinating CNS diseases. They can be either monophasic, such as acute disseminated encephalomyelitis (ADEM), or relapsing, such as multiple sclerosis (MS). In children, ADEM is more common before puberty, whereas MS becomes increasingly more frequent during puberty. Read More

Front Pediatr 2019 26;7:51. Epub 2019 Feb 26.

Pediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.

The polyglandular autoimmune syndrome type I is a rare hereditary autosomal recessive disease. We describe a child with the classic triad of the disease and her sister with pure red cell aplasia and cerebellar hypoplasia. The latter received two haematopoietic stem cell transplantations, complicated by an acute disseminated encephalomyelitis. Read More

Neurol India 2019 Jan-Feb;67(1):284-286

Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

AJNR Am J Neuroradiol 2019 Apr 7;40(4):726-731. Epub 2019 Mar 7.

Section of Pediatric Neuroradiology (I.I.), Division of Neuroradiology, The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins School of Medicine, Baltimore, Maryland.

Background And Purpose: Differentiating pediatric-onset neuromyelitis optica spectrum disorder from acute disseminated encephalomyelitis could be challenging, especially in cases presenting with only brain manifestations. Our purpose was to investigate brain MR imaging features that may help distinguish these 2 entities.

Materials And Methods: We retrospectively examined initial brain MR imaging studies of 10 patients with pediatric-onset neuromyelitis optica spectrum disorder (female/male ratio, 7:3) and 10 patients with acute disseminated encephalomyelitis (female/male ratio, 2:8). Read More

Am J Emerg Med 2019 Feb 15. Epub 2019 Feb 15.

Division of Pediatric Emergency Medicine, Department of Pediatrics, Dana-Dwek Children's Hospital, University of Tel Aviv, Israel; Division of Clinical Pharmacology and Toxicology, Tel Aviv Sourasky Medical Center, University of Tel Aviv, Israel. Electronic address:

Background: Acute disseminated encephalomyelitis (ADEM) is an uncommon, treatable, primarily pediatric, immune-mediated disease. Diagnosis of ADEM requires two essential elements: typical clinical presentation and magnetic resonance imaging (MRI) findings. The aim of this study was to evaluate how clinical findings in the initial emergency department (ED) presentation influenced the timing of MRI. Read More

Curr Opin Neurol 2019 Feb 21. Epub 2019 Feb 21.

Purpose Of Review: To review the clinical findings, differential diagnosis, treatment and outcome of pseudotumoral demyelinating lesions including tumefactive demyelination and Baló's concentric sclerosis.

Recent Findings: MRI findings, such as dynamic restricted diffusion changes at the edge of pseudotumoral lesions help to discriminate atypical demyelination from key differential diagnoses, and together with histopathological data, indicate that tissue hypoxia may be important aetiologically. CT-PET imaging can help to distinguish pseudotumoral lesions from high-grade tumours. Read More

Zhonghua Yan Ke Za Zhi 2019 Mar;55(3):174-179

Department of Ophthalmology, Chinese PLA General Hospital, Beijing 100853, China.

To investigate the clinical characteristics and prognosis of myelin oligodendrocyte glycoprotein(MOG) antibody-positive optic neuritis (MOG-ON). Retrospective case series study. A total of 65 patients diagnosed with MOG-ON at the Department of Ophthalmology of the Chinese People's Liberation Army General Hospital during January 2016 and October 2017 were selected. Read More

Brain Dev 2019 Mar 1. Epub 2019 Mar 1.

Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan.

The detailed clinical time course in acute disseminated encephalomyelitis (ADEM) from initial symptoms, through exacerbation, to remission has not been widely reported. Hence, this study aimed to investigate the clinical time course of pediatric ADEM. This was a multicenter retrospective study based on registry data from medical chart reviews. Read More

JAMA Neurol 2019 Mar 4. Epub 2019 Mar 4.

Faculdade de Medicina, Universidade Federal do Ceará, Fortaleza, Ceará, Brasil.

Mult Scler Relat Disord 2019 May 22;30:231-235. Epub 2019 Feb 22.

Department of Neurology, Johns Hopkins University, Baltimore, MD, USA.

Background: Serological antibodies against myelin oligodendrocyte glycoprotein (MOG) are associated with a relapsing autoimmune demyelinating disease of the central nervous system. Initially identified in the context of acute disseminated encephalomyelitis, persistent seropositivity of MOG antibodies is now recognized as a variant of neuromyelitis optica spectrum disorder (NMOSD).

Objectives: The aim of the study is to describe the epidemiological and clinical features of MOG antibody positive cases and compare our findings with those previously published. Read More

Cureus 2018 Dec 21;10(12):e3759. Epub 2018 Dec 21.

Internal Medicine, Geisinger Medical Center, Danville, USA.

Influenza and pneumococcal vaccines are recommended in inflammatory bowel disease patients. Several neurologic complications have been reported after influenza vaccines, such as Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy, and acute disseminated encephalomyelitis; however, rarely, oculomotor palsy will occur. We report the case of a 23-year-old male with a past medical history of ulcerative colitis on sulfasalazine who presented to the hospital with a complaint of blurry vision five days after an influenza vaccine. Read More

Acta Med Port 2019 Feb 1;32(1):81-85. Epub 2019 Feb 1.

Departamento de Neurologia. Hospital da Luz. Lisboa. Portugal.

Acute disseminated encephalomyelitis is a rare inflammatory demyelinating multifocal disease of the central nervous system that typically occurs in children following vaccination or exanthematous viral infections and conveys an elevated risk of neurological sequelae unless promptly recognized and treated. We describe an adult case of acute disseminated encephalomyelitis following vaccination against Mumps, Measles and Rubella, presenting with fever and progressive neurological deficits which improved under systemic corticosteroid therapy. Considering the ongoing public debate regarding universal vaccination and the surge of previously controlled infectious diseases, we aim not only to underline the need for a rigorous assessment of vaccination safety on adult patients in order to prevent misguidance of public opinion, but also to alert clinicians for an early diagnosis of acute disseminated encephalomyelitis in these patients, the incidence of which we speculate may be rising. Read More

Mult Scler Relat Disord 2019 May 31;30:42-44. Epub 2019 Jan 31.

University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75390, USA; Children's Health, Dallas, TX, USA.

Importance: Patients afflicted with rare diseases often have a delay in diagnosis and treatment. Understanding the prevalence and impact of delayed diagnosis in transverse myelitis could trigger directed educational initiatives to increase clinician awareness and improve care.

Objective: To determine if symptoms at onset or care provider initially approached was associated with time to diagnosis, treatment or outcome in patients with transverse myelitis. Read More

Mult Scler 2019 Feb 7:1352458518823486. Epub 2019 Feb 7.

Department of Paediatric Neurology, Great Ormond Street Hospital for Children, London, UK; Department of Neuroinflammation, Queen Square Multiple Sclerosis Centre, UCL Institute of Neurology, London, UK.

Objective:: To identify predictors of epilepsy and clinical relapses in children presenting with acute disseminated encephalomyelitis (ADEM).

Methods:: Children presenting with ADEM between 2005 and 2017 and tested clinically for MOG-Ab were identified from three tertiary paediatric neurology centres in the United Kingdom. Patients were followed up for a median of 6 years (range, 1-16 years). Read More

Neurology 2019 Mar 6;92(11):e1250-e1255. Epub 2019 Feb 6.

From the Oxford Autoimmune Neurology Group (P.J.W., M.W., S.R.I.), Nuffield Department of Clinical Neurosciences, UK; Institute for Experimental Immunology (L.K., S.L.), Affiliated to Euroimmun AG, Luebeck, Germany; and Departments of Neurology (M.M., E.P.F., A.C.K., A.M., S.J.P.) and Laboratory Medicine and Pathology (J.F., J.M., E.P.F., A.C.K., A.M., S.J.P.), Mayo Clinic, College of Medicine, Rochester, MN.

Objectives: To compares 3 different myelin oligodendrocyte glycoprotein-immunoglobulin G (IgG) cell-based assays (CBAs) from 3 international centers.

Methods: Serum samples from 394 patients were as follows: acute disseminated encephalomyelitis (28), seronegative neuromyelitis optica (27), optic neuritis (21 single, 2 relapsing), and longitudinally extensive (10 single, 3 recurrent). The control samples were from patients with multiple sclerosis (244), hypergammaglobulinemia (42), and other (17). Read More

Med Clin North Am 2019 Mar 3;103(2):325-336. Epub 2018 Dec 3.

Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA. Electronic address:

Autoimmune disorders of the central nervous system are common and often affect people in the most productive years of their lives. Among primary autoimmune diseases of the central nervous system, multiple sclerosis is most prevalent in the United States. Many other autoantibody-mediated neurologic syndromes have been identified within the past 2 to 3 decades, including neuromyelitis optica and anti-N-methyl-D aspartate receptor encephalitis. Read More

Neurol Neuroimmunol Neuroinflamm 2019 Mar 14;6(2):e528. Epub 2018 Dec 14.

Department of Neurology (Y.Y.M.W., C.L.d.M., R.M.v.d.V.d.V., E.D.v.P., I.A.K., R.Q.H.), MS Centre ErasMS, Erasmus MC, Rotterdam, The Netherlands; and Department of Pediatric Neurology (C.E.C.-B., R.F.N.), Erasmus MC, Rotterdam, The Netherlands.

Objective: To compare the diagnostic accuracy of the McDonald 2017 vs the McDonald 2010 criteria to predict a second attack of MS (clinically definite MS [CDMS]) at the first attack of acquired demyelinating syndromes (ADS).

Methods: One hundred sixty-four children (aged <18 years) with an incident attack of ADS were included in a prospective multicenter study between June 2006 and December 2016. Brain (and spinal if available) MRI was performed ≤3 months after symptom onset. Read More

Front Neurol 2018 15;9:1156. Epub 2019 Jan 15.

Neurology Unit, Multiple Sclerosis Center, Department of Neuroscience of Bambino Gesù Children's Hospital, Rome, Italy.

The aim of the study was to estimate the rate of evolution or for multiple sclerosis (MS), after a first acute demyelinating event (ADE) in pediatric patients, and to investigate the variables that predict this evolution. We retrospectively evaluated the clinical and neuroradiological features of children who presented a first ADE between January 2005 and April 2017. All patients included underwent a baseline MRI, a cerebrospinal fluid and blood analysis, including virological examinations. Read More

Noro Psikiyatr Ars 2018 ;55(Suppl 1):S1-S9

Department of Neurology, İstanbul Bağcılar Research and Training Hospital, İstanbul, Turkey.

The diagnostic criteria for multiple sclerosis (MS) have been continuously evolved since 1950's, and gained speed parallel to the development of detailed laboratory methods. The common aim for all the defined criteria up to now, is to establish the dissemination in space and time of the clinical picture caused by the lesions in the central nervous system (CNS), and to rule out other diseases which might mimic MS. There is no definite measure or laboratory marker for the diagnosis of MS, yet. Read More

Asian J Transfus Sci 2018 Jul-Dec;12(2):127-135

Department of Paediatrics, Apollo Children's Hospitals, Chennai, Tamil Nadu, India.

Introduction: Outcome data in pediatric plasma exchange, especially in nonrenal indications are scarce. We aimed to evaluate its role and outcome in our patients.

Subjects And Methods: A retrospective study of children admitted in the year 2016 to the Pediatric Intensive Care Unit requiring plasma exchange for nonrenal indications was undertaken. Read More

Vaccine 2019 Feb 23;37(8):1126-1129. Epub 2019 Jan 23.

Division of Basic Nursing, Fukuoka Nursing College, 2-15-1 Tamura, Sawara-ku, Fukuoka 814-0193, Japan.

While the basic definition of vaccination-associated acute disseminated encephalomyelitis (ADEM) is relatively clear and easily understandable, it is often difficult to diagnose ADEM based on clinical findings alone. ADEM is actually a heterogeneous clinical syndrome that can be approximately characterized by encephalomyelitis with multiple inflammatory demyelination, autoimmune causes, and relationship with a preceding infection or vaccination. The differential diagnosis of ADEM should exclude the possibility of infectious or other autoimmune encephalitis. Read More

Trop Doct 2019 Jan 24:49475518825219. Epub 2019 Jan 24.

5 Professor, Department of Microbiology, PGIMER and DR. R.M.L. Hospital, New Delhi, India.

Chikungunya (CHIK) has re-emerged as a potential neurotropic virus, with outbreaks recently being reported from many parts of India. The present study was conducted to study the spectrum and outcome of neurological complications in patients of CHIK during the 2016 outbreak in Delhi. A total of 42 cases seropositive for IgM CHIK antibodies by MAC-ELISA and developing neurological complications were enrolled. Read More

Jpn J Radiol 2019 Apr 22;37(4):277-282. Epub 2019 Jan 22.

Department of Radiology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo Prefecture, Japan.

Purpose: To evaluate the frequency, characteristics, and clinical significance of transient hyperintensity foci on T1-weighted images (T1WI) in acute disseminated encephalomyelitis (ADEM).

Materials And Methods: Patients diagnosed with ADEM underwent MR studies at the time of disease onset and every 3 months or more often thereafter. The frequency and appearance timing of abnormal signals including T1WI and their morphological characteristics were evaluated. Read More

MBio 2019 01 22;10(1). Epub 2019 Jan 22.

Pathogen and Microbiome Division, Translational Genomics Research Institute, Flagstaff, Arizona, USA

Enteroviruses are a common cause of respiratory and gastrointestinal illness, and multiple subtypes, including poliovirus, can cause neurologic disease. In recent years, enterovirus D68 (EV-D68) has been associated with serious neurologic illnesses, including acute flaccid myelitis (AFM), frequently preceded by respiratory disease. A cluster of 11 suspect cases of pediatric AFM was identified in September 2016 in Phoenix, AZ. Read More

Clin Case Rep 2019 Jan 28;7(1):160-163. Epub 2018 Nov 28.

Department of Pediatrics University of Kansas Medical Center Kansas City Kansas.

Intravenous immunoglobulin therapy should be considered in pediatric acute hemorrhagic leukoencephalitis that is refractory to steroid and plasmapheresis. Read More

Rinsho Ketsueki 2018;59(12):2588-2593

Hematology, Graduate School of Medicine, Osaka City University.

Chronic graft versus host disease (cGVHD) of the central nervous system is a rare condition that could occur after allogeneic hematopoietic stem cell transplantation (SCT) but has been poorly documented. Here, we report a patient diagnosed with recurrent acute disseminated encephalomyelitis (ADEM) with longitudinal extensive transverse myelitis (LETM) as cGVHD after HLA haploidentical peripheral blood SCT using posttransplantation cyclophosphamide for mixed-phenotype acute leukemia. We observed clinical and radiological improvement after the rituximab treatment of the condition that had been refractory to steroids. Read More

Case Rep Neurol Med 2018 28;2018:8409247. Epub 2018 Nov 28.

Ege University, Faculty of Medicine, Department of Radiology, Izmir, Turkey.

Demyelinating lesions larger than 2 cm in diameter, with or without edema, are known as tumefactive demyelinating lesions (TDLs). They constitute a rare inflammatory demyelinating disorder of the central nervous system. TDLs are typically characterized by headaches, cortical symptoms such as aphasia, hemiparesis, hemisensory deficits, seizures, and changes in consciousness. Read More

eNeurologicalSci 2019 Mar 22;14:40-42. Epub 2018 Nov 22.

The London Clinic, London, UK.

Acute disseminated encephalomyelitis (ADEM) is an uncommon, autoimmune, demyelinating disorder of of the central nervous system. It is rare in adults beyond 65 years. Here, we describe a novel presentation following urological surgery. Read More

Rinsho Shinkeigaku 2019 Jan 29;59(1):33-36. Epub 2018 Dec 29.

Department of Neurology, Fujita Health University, School of Medicine.

A 48-year-old man with rapid onset of fever elevation developed acute myelitis over a period of a week. MRI of the spinal cord revealed a longitudinal T-hyperintense intraspinal lesion extending from C6 to Th8 level. Clinical symptoms and findings resolved with immunotherapy. Read More

BMJ Case Rep 2018 Dec 27;11(1). Epub 2018 Dec 27.

Department of Virology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory-demyelinating disease of central nervous system. Several viral infections and vaccines act as precipitating factor for ADEM. We report an 8-year-old girl presenting with acute-onset flaccid paraparesis and bilateral painless vision loss. Read More

Mult Scler Relat Disord 2019 Feb 18;28:109-116. Epub 2018 Dec 18.

MS Center and National Reference Center of adult Leukodystrophies, Department of Neurology, Montpellier University Hospital, 34 295 Montpellier Cedex 5, France. Electronic address:

Background: The recent 2017 modification have increased the sensitivity of McDonald criteria for MS. Nevertheless, some MS patients with atypical MRI findings have been identified, leading to prolonged delay to diagnosis and high costs to look for alternative diagnoses.

Objective: To describe a series of MS patients with atypical MRI presentation. Read More

Mult Scler Relat Disord 2019 Feb 14;28:101-103. Epub 2018 Dec 14.

Mersin University, Faculty of Medicine, Department of Pediatric Neurology, Mersin, Turkey. Electronic address:

Background: Myelin Oligodendrocyte Glycoprotein antibodies (MOG) may be used as a biomarker for diagnosis of many demyelinating diseases. Especially, patients of acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), aquaporin-4 (AQP4) seronegative neuromyelitis optica spectrum disorder (NMOSD), monophasic or recurrent optic neuritis (ON), transverse myelitis and N-methyl-d-aspartate (NMDA) receptor encephalitis (NMDARe) can overlap with Myelin Oligodendrocyte Glycoprotein antibodies. We present a child with autoimmune encephalitis in whom antibodies against Myelin Oligodendrocyte Glycoprotein (MOG) and N-methyl-d-aspartate receptor (NMDAR) were simultaneously detected. Read More

JAMA Neurol 2018 Dec 21. Epub 2018 Dec 21.

Department of Neurology, Mayo Clinic College of Medicine, Rochester, Minnesota.

Importance: Recognizing the characteristics of myelin oligodendrocyte glycoprotein autoantibody (MOG-IgG) myelitis is essential for early accurate diagnosis and treatment.

Objective: To evaluate the clinical, radiologic, and prognostic features of MOG-IgG myelitis and compare with myelitis with aquaporin-4-IgG (AQP4-IgG) and multiple sclerosis (MS).

Design, Setting, And Participants: We retrospectively identified 199 MOG-IgG-positive Mayo Clinic patients from January 1, 2000, through December 31, 2017, through our neuroimmunology laboratory. Read More

Nat Rev Neurol 2019 Feb;15(2):89-102

Oxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK.

Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies (MOG-Abs) were first detected by immunoblot and enzyme-linked immunosorbent assay nearly 30 years ago, but their association with multiple sclerosis (MS) was not specific. Use of cell-based assays with native MOG as the substrate enabled identification of a group of MOG-Ab-positive patients with demyelinating phenotypes. Initially, MOG-Abs were reported in children with acute disseminated encephalomyelitis (ADEM). Read More

Front Immunol 2018 29;9:2753. Epub 2018 Nov 29.

Clinical Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria.

Inflammatory demyelinating CNS syndromes include, besides their most common entity multiple sclerosis (MS), several different diseases of either monophasic or recurrent character-including neuromyelitis optica spectrum disorders (NMOSDs) and acute disseminated encephalomyelitis (ADEM). Early diagnostic differentiation is crucial for devising individual treatment strategies. However, due to overlapping clinical and paraclinical features diagnosis at the first demyelinating event is not always possible. Read More

Brain Dev 2019 Apr 3;41(4):373-377. Epub 2018 Dec 3.

Department of Pediatrics, St Mary's Hospital, 422 Tsubukuhonmachi, Kurume, Fukuoka 830-8543, Japan; Research Centre for Children and Research Centre for Rett Syndrome, St Mary's Hospital, 422 Tsubukuhonmachi, Kurume, Fukuoka 830-8543, Japan. Electronic address:

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. Read More

J Pediatr 2019 Mar 6;206:26-32.e1. Epub 2018 Dec 6.

Department of Pediatrics, Tulane University, New Orleans, LA; Department of Pediatrics, Texas Tech University Health Sciences Center, Amarillo, TX.

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. Read More

Vaccine 2019 Jan 30;37(2):366-371. Epub 2018 Nov 30.

Kitasato Institute for Life Sciences, Laboratory of Viral Infection, 5-9-１ Shirokane Minato-ku, Tokyo 108-8641, Japan. Electronic address:

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. Vaccines stimulate the innate immunity of host immunological defense mechanisms and induce the development of specific acquired immunity. Read More

Cureus 2018 Sep 24;10(9):e3351. Epub 2018 Sep 24.

Orthopaedics, Dow University of Health Sciences, Karachi, PAK.

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. Read More

J Child Neurol 2019 Feb 27;34(2):99-103. Epub 2018 Nov 27.

2 Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Tel Aviv, Israel.

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. Read More

Can J Neurol Sci 2018 11;45(6):686-687

1Division of Neurology,Department of Medicine,University of Toronto,Toronto,Ontario,Canada.