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Mult Scler Relat Disord 2018 Sep 11;26:86-89. Epub 2018 Sep 11.

Duke University, Durham, NC, United States.

Background: Acute disseminated encephalomyelitis has been understood as a monophasic, often post-infectious illness that predominantly affects the pediatric population. Though that describes the majority of cases, exceptions do exist. In this case report, we present an adult case of recurrent disseminated encephalomyelitis (DEM) and review the available literature on this clinical entity. Read More

Radiologe 2018 Sep 20. Epub 2018 Sep 20.

Abteilung für Neuroradiologie, Kantonsspital Baden, Baden, Schweiz.

Clinical Issue: Autoimmune disorders of the central nervous system (CNS) are common but are also a heterogeneous group of diseases. The most common form is multiple sclerosis (MS), others are clinically isolated syndrome (CIS), acute demyelinating encephalomyelitis (ADEM) and neuromyelitis optica spectrum disorders (NMOSD). Paraneoplastic syndromes are rare and tumor-associated, they are not induced by direct invasion of tumor tissue but by tumor-associated autoantibodies mostly against specific CNS proteins, e. Read More

Cureus 2018 Jul 17;10(7):e2989. Epub 2018 Jul 17.

Internal Medicine, Shifa College Of Medicine, Islamabad, PAK.

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system (CNS) with no distinct etiology but implications include infections and commonly administered vaccinations. In this case report, we present the case of ADEM in a young female who was subsequently diagnosed with acute intermittent porphyria (AIP) that was the instigator of the initial CNS assault. Our case highlights the peculiar presentation of ADEM which can present as a diagnostic challenge and brings forth AIP as a new and previously unknown affiliate of this rare CNS disease. Read More

Rev Argent Microbiol 2018 Sep 17. Epub 2018 Sep 17.

Servicio de Pediatría, Sanatorio Mater Dei, Buenos Aires, Argentina.

We present here the case of a previously healthy 5 year-old boy hospitalized in an intensive care unit due to tonic-clonic seizures focused on the face and right side of the body, and axillary temperature of 37.4°C. Common bacterial and viral etiology was ruled out through studies of cerebrospinal fluid (CSF) samples. Read More

Indian J Pediatr 2018 Sep 19. Epub 2018 Sep 19.

Department of Pediatrics, Lady Hardinge Medical College and Associated Kalawati Saran Children's Hospital, New Delhi, India.

Acute encephalitis is a common cause of death and neurodevelopmental problems in children. The causative agents are numerous including infectious agents such as viruses, bacteria, mycobacteria and protozoa; para-infectious and immune mediated disorders such as acute disseminated encephalomyelitis (ADEM) and autoimmune encephalitis, especially the recently described anti-NMDA receptor encephalitis. Also, many viral associated encephalopathies such as acute necrotizing encephalopathy can mimic the presentation of acute encephalitis. Read More

Curr Neurol Neurosci Rep 2018 Sep 18;18(11):76. Epub 2018 Sep 18.

Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Purpose Of Review: Diagnostic criteria for pediatric-onset multiple sclerosis (POMS) and related demyelinating disorders have been updated, neuroimaging studies have revealed new insights, biological assays identify patients with specific antibodies that influence both diagnosis and treatment, clinical trials are informing on treatment efficacy and safety, and longitudinal studies of neurological, cognitive and quality of life outcomes are informing on the impact of these diseases. We provide updates to assist providers caring for these children.

Recent Findings: The recent 2017 McDonald Criteria for MS provide a simplified means to confirm diagnosis at onset and over time, and have been shown to be equally applicable for POMS. Read More

Mult Scler 2018 Jun 1:1352458518781992. Epub 2018 Jun 1.

Laboratório de Imunologia Viral, Instituto Oswaldo Cruz, Fundação Oswaldo Cruz (FIOCRUZ), Rio de Janeiro, Brazil.

Background: A range of different neurological manifestations has been reported in fetuses and adults after Zika virus (ZIKV) infection.

Objective: We describe a detection of the ZIKV in the brain tissue from a multiple sclerosis (MS) patient with acute disseminated encephalomyelitis (ADEM)-like event in Rio de Janeiro, Brazil.

Methods: Biological samples collected during the hospitalization were tested by serology and molecular diagnostic for various infectious agents. Read More

Dev Med Child Neurol 2018 Sep 17. Epub 2018 Sep 17.

Brain Autoimmunity Group, Kids Neuroscience Centre at the Children's Hospital at Westmead, Sydney Medical School, University of Sydney, Sydney, NSW, Australia.

Myelin oligodendrocyte glycoprotein (MOG) antibodies have a strong association with acute disseminated encephalomyelitis (ADEM) in children, and bilateral and recurrent optic neuritis in children and adults. Recent reports suggest that seizures and encephalopathy may occur in children and adults with MOG antibody-associated disease. We describe the clinical, laboratory, and radiological course of four MOG antibody-positive children who first presented with isolated seizures without fulfilling clinical or radiological criteria for ADEM or other central nervous system demyelination syndromes, who months to years later developed more typical demyelination. Read More

Neurodegener Dis Manag 2018 Sep 14. Epub 2018 Sep 14.

Medicine Institute, Mellen Center for Treatment & Research in Multiple Sclerosis, Neurological Institute, Cleveland Clinic, Cleveland, OH, 44195, USA.

Dr Bruce Trapp and Dr Daniel Ontaneda speak to Laura Dormer, Commissioning Editor: Bruce D Trapp, PhD, is a Chair of the Department of Neurosciences at the Lerner Research Institute, Cleveland Clinic (OH, USA) and Professor of Molecular Medicine at Case Western Reserve University (OH, USA). Dr Trapp received his PhD from Loyola University Stritch School of Medicine in Chicago (IL, USA). Dr Trapp's research investigates the cause of neurological disability in multiple sclerosis patients, cellular mechanism of brain repair in neurodegenerative diseases and the molecular biology of myelination in the central and peripheral nervous systems. Read More

Mult Scler Relat Disord 2018 Sep 10;26:1-7. Epub 2018 Sep 10.

Department of Pediatric Neurology, Hacettepe University Ihsan Dogramaci Children's Hospital, Ankara, Turkey. Electronic address:

Background: Knowledge has been expanding on myelin oligodendrocyte glycoprotein (MOG) antibody-associated central nervous system disorders. We delineate the clinical and paraclinical findings and outcome of our pediatric patients with MOG antibody seropositive disease.

Methods: We retrospectively analyzed the clinical presentation, cerebrospinal fluid findings, magnetic resonance imaging (MRI) studies, course and outcome of children seropositive for anti-MOG IgG. Read More

Medicina (B Aires) 2018 ;78 Suppl 2:75-81

Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina. E-mail:

Demyelinating diseases are a group of conditions of autoimmune etiology directed against the myelin of the central nervous system. In many cases, the onset of the illness is preceded by a nonspecific viral infection. Multiple sclerosis is a disease that evolves with relapses and remissions with polyfocal neurological deficits, being the most frequent optic neuritis, transverse myelitis and encephalic trunk involvement. Read More

Lancet Child Adolesc Health 2018 Mar 1;2(3):191-204. Epub 2018 Feb 1.

Division of Child Neurology, Department of Neurology, The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

Background: MRI and laboratory features have been incorporated into international diagnostic criteria for multiple sclerosis. We assessed the pattern of MRI lesions and contributions of cerebrospinal fluid (CSF) and serum antibody findings that best identifies children with multiple sclerosis, and the applicability of international diagnostic criteria in the paediatric context.

Methods: In this prospective cohort study, detailed clinical assessments, serum and CSF studies, and MRI scans were done in youth (aged 0·46-17·87 years) with incidental acquired demyelinating syndrome. Read More

Zh Nevrol Psikhiatr Im S S Korsakova 2018 ;118(8. Vyp. 2):95-102

Research Center of Neurology, Moscow, Russia.

Primary central nervous system lymphoma (PCNSL) is a rare aggressive extranodal non-Hodgkin lymphoma. Difficulties in diagnosing PCNSL are associated with the absence of pathognomonic clinical and neuroimaging findings of this disease. The article describes the clinical case of a female patient with autopsy-confirmed multifocal large-cell B-cell CNS lymphoma misdiagnosed as acute disseminated encephalomyelitis (ADEM). Read More

IDCases 2018 6;13:e00434. Epub 2018 Aug 6.

Department of Internal Medicine, Division of Hematology/Oncology, Faculty of Medicine, American University of Beirut, P.O. Box 11-0236, Riad El-Solh, Beirut, 1107 2020, Lebanon.

is an opportunistic parasite that infects a broad range of hosts including humans. The chronic latent phase of the disease manifests as intra-neuronal cerebral cysts tightly controlled by the host immune system. In immunocompromised patients, reactivation of cerebral toxoplasmosis can have severe neurological outcomes that may sometimes lead to death. Read More

Clin Neurol Neurosurg 2018 Oct 11;173:118-119. Epub 2018 Aug 11.

Department of Neurology, Centre Hospitalier Francois Mitterrand, Pau, France. Electronic address:

Weston-Hurst syndrome is an exceptional variant of ADEM characterized by brain hemorrhages. Lesions are usually supratentorial and death is a usual outcome. We report a cerebellar Weston-Hurst syndrome early treated by craniectomy, steroids and plasma exchange. Read More

Indian J Crit Care Med 2018 Jul;22(7):519-523

Department of Pediatrics, JN Medical College, KLE University, Belgaum, Karnataka, India.

Treatment and outcome of children with acute encephalopathy depend on the cause, prompt treatment of the underlying cause, and use of adequate supportive measures. Many novel causes of acute encephalopathy are emerging where lumbar puncture, computed tomography of the head, and routine biochemical testing can be normal such as acute disseminated encephalomyelitis and febrile infection-related refractory epilepsy syndrome. Magnetic resonance imaging (MRI) plays an important role in the workup of children with acute leukoencephalopathy. Read More

Clin Neuropathol 2018 Sep/Oct;37(5):206-208

Pediatr Neurol 2018 Jul 10. Epub 2018 Jul 10.

University of Texas Southwestern Medical Center, Dallas, Texas.

Background: Anti-myelin oligodendrocyte glycoprotein (MOG) antibody associated disorders frequently manifest as optic neuritis, transverse myelitis, and acute disseminated encephalomyelitis. While their clinical phenotypes overlap with relapsing inflammatory Central nervous system (CNS) conditions such as multiple sclerosis and neuromyelitis optica spectrum disorder, MOG-related syndromes frequently occur in a younger age group. In children, longitudinally extensive transverse myelitis (LETM) is less specific for anti-aquaporin-4 associated neuromyelitis optica spectrum disorder, and has also been reported in pediatric multiple sclerosis, idiopathic transverse myelitis, and acute flaccid myelitis. Read More

Clin Auton Res 2018 Aug 3. Epub 2018 Aug 3.

Neurology, Internal Medicine, Sakura Medical Center, Toho University, 564-1 Shimoshizu, Sakura, 285-8741, Japan.

Purpose: Multiple sclerosis (MS), neuromyelitis optica (NMO), and related disorders are major immune-mediated central nervous system diseases affecting the spinal cord. We reviewed the occurrence of neurogenic lower urinary tract dysfunction (NLUTD) in these categories of diseases.

Methods: We systematically reviewed the literature regarding bladder dysfunction in MS, NMO spectrum disorder (NMOSD), and related disorders (acute disseminated encephalomyelitis, acute immune-mediated myelopathy, and meningitis-retention syndrome). Read More

Mult Scler Relat Disord 2018 Jul 20;25:138-142. Epub 2018 Jul 20.

Department of Neurology, Universidade Federal de São Paulo (UNIFESP), São Paulo, Brazil.

Background: Multiple Sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD) are acquired demyelinating syndromes of the central nervous system more frequently in young adults and their beginning before 18 years of age is rare. They are autoimmune diseases with distinct pathophysiology, clinical presentation, treatment and prognoses. During childhood these conditions often present similar clinical features and differential diagnosis among pNMOSD, pMS and acute disseminated encephalomyelitis (ADEM) is still difficult at disease onset. Read More

Acta Neurochir (Wien) 2018 Sep 29;160(9):1789-1792. Epub 2018 Jul 29.

Service de Neurochirurgie, Hôpital d'Instruction des Armées Sainte Anne, BP600, 83800, Toulon Cedex 9, France.

We present the case of a patient who died of a fatal meningoencephalitis after removal of a third ventricle colloid cyst. Postoperative clinical and iconographic evolution let us think about an acute disseminated encephalomyelitis probably due to cerebrospinal fluid contamination by inflammatory proteins contained in the colloid cyst. This case raises the question of a possibility of colloid cyst content spraying while using an ultrasonic aspiration device. Read More

Mult Scler Relat Disord 2018 Aug 11;24:175-183. Epub 2018 Jun 11.

Danish Multiple Sclerosis Center, Department of Neurology, Rigshospitalet, Copenhagen University Hospital, Denmark.

Background: Chitinase 3-like 1 (CHI3L1), neurofilament light chain (NFL) and oligoclonal bands (OCB) in cerebrospinal fluid are associated with central nervous system demyelination in adults. CHI3L1 and OCB are markers of central nervous system inflammation, whereas NFL is a marker of white-matter axonal injury. The aim was to examine whether CHI3L1 and NFL in cerebrospinal fluid are associated with acquired demyelinating syndromes at disease onset in a pediatric population. Read More

J Neurol 2018 Jul 27. Epub 2018 Jul 27.

Neurology Department, University of Nantes Hospital, Nantes, France.

Background: Tumefactive demyelinating lesions of the central nervous system can be the initial presentation in various pathological entities [multiple sclerosis (the most common), Balo's concentric sclerosis, Schilder's disease and acute disseminated encephalomyelitis] with overlapping clinical presentation. The aim of our study was to better characterize these patients.

Methods: Eighty-seven patients (62 women and 25 men) from different MS centers in France were studied retrospectively. Read More

Mult Scler Relat Disord 2018 Jul 19;25:77-82. Epub 2018 Jul 19.

King Abdullah International Medical Research Center, King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia.

Familial hemophagocytic lymphohistiocytosis (HLH) is a rare autosomal recessive life-threatening multisystem inflammatory disorder. It is characterized by excessive production of cytokines and uncontrolled activation of lymphocytes and macrophages leading to widespread organ infiltration and tissue destruction. Central nervous system involvement is common occurring in approximately 75% of patients. Read More

Pan Afr Med J 2018 2;29:138. Epub 2018 Mar 2.

Department of Endocrinology and Diabetes, Diabetes Treatment Center, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.

Acute disseminated encephalomyelitis is a rare disease of central nervous system, which can present with a variety of clinical manifestations. That is why first attack of ADEM, in particular remains a diagnostic puzzle. Early anticipation and diagnosis is important for better outcomes. Read More

Br J Ophthalmol 2018 Jul 26. Epub 2018 Jul 26.

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

Background/aims: To investigate clinical characteristics and prognosis of paediatric optic neuritis (PON) in patients seropositive for myelin oligodendrocyte glycoprotein antibody (MOG-Ab) in China.

Methods: Children displaying initial onset of optic neuritis (ON) were recruited from the Neuro-ophthalmology Department in the Chinese People's Liberation Army General Hospital from January 2016 to August 2017. They were assigned into three groups based on antibody status: MOG-Ab-seropositive ON (MOG-ON), aquaporin-4 antibody-seropositive ON (AQP4-ON) and double seronegative ON (seronegative-ON). Read More

Mult Scler Relat Disord 2018 Jul 23;25:66-72. Epub 2018 Jul 23.

Department of Neurology, Johns Hopkins University, Baltimore, MD, USA. Electronic address:

MOG antibody disease is an autoimmune disease of the central nervous system associated with a serological antibody against MOG, myelin oligodendrocyte glycoprotein. MOG is a glycoprotein expressed on the outer membrane of myelin and solely found within the central nervous system, including in the brain, optic nerves and spinal cord. Clinically, the disease resembles neuromyelitis optica spectrum disorders in the predilection for relapses of optic neuritis and transverse myelitis. Read More

Front Public Health 2018 10;6:186. Epub 2018 Jul 10.

Department of Comparative, Diagnostic, and Population Medicine, University of Florida, Gainesville, FL, United States.

Several arboviruses are endemic to and co-circulate in Pakistan. In recent years, Pakistan has observed a rise in arboviral infections. A cross-sectional study for arboviral diseases, which included screening for Chikungunya virus (CHIKV), was initiated in 2015 to determine which pathogens were causing disease in patients presenting to health care services. Read More

Neurol India 2018 Jul-Aug;66(4):1172-1174

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

JAMA Neurol 2018 Jul 16. Epub 2018 Jul 16.

Department of Neurology, Mayo Clinic, Rochester, Minnesota.

Importance: Recent studies have reported a higher relapse rate following an initial inflammatory demyelinating disorder in pediatric patients with persistent seropositivity of antibodies targeting myelin oligodendrocyte glycoprotein (MOG-IgG1). To date, the clinical implications of longitudinal MOG-IgG1 seropositivity using live cell assays with IgG1 secondary antibodies in adults after acute disseminated encephalomyelitis (ADEM) are unknown.

Objective: To determine whether MOG-IgG1 serostatus (transient vs persistent) and titer change over time provide clinical utility in predicting the likelihood of relapse after ADEM. Read More

J Neuroimmunol 2018 Aug 28;321:83-91. Epub 2018 May 28.

Department of Neurology, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China. Electronic address:

Background: Immunoglobulin against myelin oligodendrocyte glycoprotein (MOG-IgG) is a potential demyelinating disease-associated autoantibody. Whether clinical features of MOG antibody-associated demyelinating diseases change with age remains unclear.

Object: To investigate the different clinical features between pediatric-onset and adult-onset MOG-IgG-seropositive patients in a relatively large cohort. Read More

IDCases 2018 12;12:41-43. Epub 2018 Mar 12.

Infectious Diseases, Medical Subspecialty Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates.

We report a case of acute disseminated encephalomyelitis (ADEM) secondary to infection that failed to improve with methylprednisolone and intravenous immunoglobulin (IVIG); who responded with plasmapheresis. A 21- year- old female with an unremarkable medical history, initially presented to an outside hospital with fever and an influenza-like illness and was subsequently intubated for worsening sensorium. Brain magnetic resonance imaging was suggestive of ADEM or vasculitis for which she received five days of pulse steroids and IVIG. Read More

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

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

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

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

J Clin Neurol 2018 Jul 31;14(3):327-332. 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

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

Eur J Neurol 2018 Aug 23;25(8):1003-1004. Epub 2018 May 23.

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

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

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

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

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

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

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

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

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

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

Rev Med Virol 2018 05 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

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

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