scholarly journals Biomarkers in Rare Demyelinating Disease of the Central Nervous System

2020 ◽  
Vol 21 (21) ◽  
pp. 8409
Author(s):  
Marina Boziki ◽  
Styliani-Aggeliki Sintila ◽  
Panagiotis Ioannidis ◽  
Nikolaos Grigoriadis
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Demyelinating Disease ◽  
Inflammatory Diseases ◽  
Neurological Diseases ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Organ Specific ◽  
The Central Nervous System ◽  
Biomarker Research

Rare neurological diseases are a heterogeneous group corresponding approximately to 50% of all rare diseases. Neurologists are among the main specialists involved in their diagnostic investigation. At the moment, a consensus guideline on which neurologists may base clinical suspicion is not available. Moreover, neurologists need guidance with respect to screening investigations that may be performed. In this respect, biomarker research has emerged as a particularly active field due to its potential applications in clinical practice. With respect to autoimmune demyelinating diseases of the Central Nervous System (CNS), although these diseases occur in the frame of organ-specific autoimmunity, pathology of the disease itself is orchestrated among several anatomical and functional compartments. The differential diagnosis is broad and includes, but is not limited to, rare neurological diseases. Multiple Sclerosis (MS) needs to be differentially diagnosed from rare MS variants, Acute Disseminated Encephalomyelitis (ADEM), the range of Neuromyelitis Optica Spectrum Disorders (NMOSDs), Myelin Oligodendrocyte Glycoprotein (MOG) antibody disease and other systemic inflammatory diseases. Diagnostic biomarkers may facilitate timely diagnosis and proper disease management, preventing disease exacerbation due to misdiagnosis and false treatment. In this review, we will describe advances in biomarker research with respect to rare neuroinflammatory disease of the CNS.

scholarly journals Acute Disseminated Encephalomyelitis (ADEM): A Demyelinating Disease with Specific Morphological Features

2021 ◽  
pp. 106689692199356
Author(s):  
Fleur Cordier ◽  
Lars Velthof ◽  
David Creytens ◽  
Jo Van Dorpe
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Differential Diagnosis ◽  
Clinical Case ◽  
Demyelinating Disease ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Demyelinating Disorder ◽  
Immune Mediated ◽  
The Central Nervous System

Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated inflammatory and demyelinating disorder of the central nervous system. Its characteristic perivenular demyelination and inflammation aid in the differential diagnosis with other inflammatory demyelinating diseases. Here, we present a clinical case of ADEM, summarize its histological hallmarks, and discuss pitfalls concerning the most important neuropathological differential diagnoses.

Diagnosis of Multiple Sclerosis

2021 ◽  
pp. 540-547
Author(s):  
W. Oliver Tobin
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Demyelinating Disease ◽  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Spectrum Disorder ◽  
Demyelinating Diseases ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
The Central Nervous System

Multiple sclerosis is the most common idiopathic inflammatory demyelinating disease of the central nervous system (CNS), with a prevalence of 1 in 500 to 1 in 2,000 people, depending on geography and various other factors. Idiopathic inflammatory demyelinating diseases are a group of related disorders that include acute disseminated encephalomyelitis, neuromyelitis optica spectrum disorder, and myelin oligodendrocyte glycoprotein–immunoglobulin G–associated CNS demyelinating disease.

Demyelinating disorders of the central nervous system

2010 ◽  
pp. 4948-4963
Author(s):  
Siddharthan Chandran ◽  
Alastair Compston
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Demyelinating Disorder ◽  
System P ◽  
The Central Nervous System ◽  
Demyelinating Disorders ◽  
Brain And Spinal Cord

Clinicians suspect demyelination when episodes reflecting damage to white matter tracts within the central nervous system occur in young adults. The paucity of specific biological markers of discrete demyelinating syndromes places an emphasis on clinical phenotype—temporal and spatial patterns—when classifying demyelinating disorders. The diagnosis of multiple sclerosis, the most common demyelinating disorder, becomes probable when these symptoms and signs recur, involving different parts of the brain and spinal cord. Other important demyelinating diseases include post-infectious neurological disorders (acute disseminated encephalomyelitis), demyelination resulting from metabolic derangements (central pontine myelinosis), and inherited leucodystrophies that may present in children or in adults. Accepting differences in mechanism, presentation, and treatment, two observations can usefully be made when classifying demyelinating disorders. These are the presence or absence of inflammation, and the extent of focal vs. diffuse demyelination. Multiple sclerosis is prototypic for the former, whereas dysmyelinating disorders, such as leucodystrophies are representative of the latter....

scholarly journals Acute Disseminated Encephalomyelitis Following Typhoid Fever: A Case Report

2014 ◽  
Vol 9 (4) ◽  
pp. 55-58
Author(s):  
R Adhikari ◽  
A Tayal ◽  
PK Chhetri ◽  
B Pokhrel
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Case Report ◽  
Typhoid Fever ◽  
Demyelinating Disease ◽  
Acute Disseminated Encephalomyelitis ◽  
Medical Sciences ◽  
Cerebellar Syndrome ◽  
Immune Mediated ◽  
The Central Nervous System

The involvement of central nervous system in children with typhoid fever is common. Acute disseminated encephalomyelitis is a rare immune mediated and demyelinating disease of the central nervous system that usually affects children. We report a 7-year-old child with typhoid fever who developed acute cerebellar syndrome due to acute disseminated encephalomyelitis.Journal of College of Medical Sciences-Nepal, 2013, Vol-9, No-4, 55-58 DOI: http://dx.doi.org/10.3126/jcmsn.v9i4.10237

scholarly journals The management of patients with neurological diseases during the COVID-19 pandemic

2020 ◽  
Vol 26 (4) ◽  
pp. 447-461
Author(s):  
M. P. Topuzova ◽  
T. M. Alekseeva ◽  
A. D. Chaykovskaya ◽  
I. K. Ternovyh ◽  
P. Sh. Isabekova
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Inflammatory Diseases ◽  
Neurological Diseases ◽  
Diagnostic Errors ◽  
Neurological Complications ◽  
Benign Intracranial Hypertension ◽  
Risk Of Infection ◽  
Limited Mobility ◽  
The Central Nervous System

The article discusses the management of patients with various neurological diseases during the COVID-19 pandemic, taking into account the determination of the level of risk of infection. The possibility of increasing the risk of infection in patients with disability, especially with impaired function of the respiratory and bulbar muscles, limited mobility, and the presence of concomitant diseases, is indicated. The recommendations on the treatment of patients with stroke, neuromuscular diseases, inflammatory and autoimmune diseases of the central nervous system, in particular, multiple sclerosis and neuromyelitis optica spectrum disorder (NMOSD), as well as non-inflammatory diseases of the central nervous system (epilepsy, Parkinson’s disease, atypical parkinsonism, dystonia, hereditary spastic paraplegia, infantile cerebral palsy, benign intracranial hypertension) are considered. Interactions of drugs for the treatment of COVID-19 and neurological diseases are given. Potential neurological complications of COVID-19 are overviewed. Potential neurological complications of COVID-19 were noted: anosmia, ageusia, viral meningitis, encephalitis, post-infectious stem encephalitis, acute necrotizing hemorrhagic leukoencephalopathy, Guillain-Barre syndrome, myositis. The importance of the fact that during the COVID-19 pandemic, when examining patients with neurological diseases, clinicians should take into account the possibility of a patient with COVID-19, and also consider it as a differential diagnosis in order to avoid diagnostic errors, prescribe timely treatment and prevent the spread of infection.

scholarly journals THE RAPID PRODUCTION OF ACUTE DISSEMINATED ENCEPHALOMYELITIS IN RHESUS MONKEYS BY INJECTION OF HETEROLOGOUS AND HOMOLOGOUS BRAIN TISSUE WITH ADJUVANTS

1947 ◽  
Vol 85 (1) ◽  
pp. 117-130 ◽  
Author(s):  
Elvin A. Kabat ◽  
Abner Wolf ◽  
Ada E. Bezer
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Rhesus Monkeys ◽  
Demyelinating Disease ◽  
Acute Disseminated Encephalomyelitis ◽  
Rabbit Brain ◽  
Adult Rabbit ◽  
Rabbit Lung ◽  
The Central Nervous System ◽  
Experimental Findings

1. A picture resembling acute disseminated encephalomyelitis in the human being has been regularly and rapidly produced in rhesus monkeys by injection of emulsions of adult rabbit and monkey brain administered with adjuvants. 2. No lesions of the central nervous system resulted from injection of similar emulsions of fetal rabbit brain or adult rabbit lung. 3. A description of the gross and histological findings in the central nervous system is given and compared with features of human demyelinating disease. 4. The experimental findings are in accord with the hypothesis that antibody to the injected brain emulsion reacts with the tissues of the nervous system of the animal to produce the pathological changes.

Recurrent Demyelinating Episodes as Sole Manifestation of Inherited CD59 Deficiency

2019 ◽  
Vol 51 (03) ◽  
pp. 206-210
Author(s):  
Ismail Solmaz ◽  
Elif Soyak Aytekin ◽  
Deniz Çağdaş ◽  
Cagman Tan ◽  
Ilhan Tezcan ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Inflammatory Diseases ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Neurological Diseases ◽  
Homozygous Mutation ◽  
Recurrent Strokes ◽  
Chronic Hemolysis ◽  
The Central Nervous System ◽  
Autosomal Recessive Condition

AbstractDefects in the regulatory components of the complement system can lead to inflammatory diseases. We present a patient who had four episodes of demyelination in the central nervous system as the only manifestation of inherited CD59 deficiency. Relapsing encephalopathy partially responsive to intravenous immunoglobulin and steroid treatments on the background of parental consanguinity suggested an inherited immune dysregulation. Next generation sequencing revealed homozygous mutation in the CD59 gene, confirmed by lack of CD59 expression on flow cytometry. Inherited CD59 deficiency is a rare autosomal recessive condition characterized by chronic hemolysis, recurrent strokes, and relapsing peripheral demyelinating neuropathy mimicking Guillain–Barré syndrome or chronic inflammatory demyelinating polyneuropathy. Recurrent central nervous system demyelinating episodes as the only manifestation has not been reported to date in inherited CD59 deficiency. This entity should be considered in the differential diagnosis of patients with early-onset recurrent neurological diseases with central or peripheral origin.

Pathologic Factors and Spectrum of Central Nervous System Inflammatory Demyelinating Diseases

2021 ◽  
pp. 531-539
Author(s):  
Claudia F. Lucchinetti ◽  
Yong Guo
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Inflammatory Process ◽  
Demyelinating Disease ◽  
Autoimmune Disorder ◽  
Demyelinating Diseases ◽  
Autoreactive T Cells ◽  
Common Cause ◽  
The Central Nervous System

Multiple sclerosis (MS) is the most common cause of nontraumatic disability in young adults. It is a chronic inflammatory demyelinating disease of the central nervous system. Traditionally, MS has been considered an autoimmune disorder consisting of myelin autoreactive T cells that drive an inflammatory process, leading to secondary macrophage recruitment and subsequent myelin destruction. However, accumulating data based on increasing numbers of probes that can be effectively applied to MS tissue have indicated that the events involved in the immunopathogenesis of MS may be more complicated.

scholarly journals Cellular approaches to central nervous system remyelination stimulation: thyroid hormone to promote myelin repair via endogenous stem and precursor cells

2009 ◽  
Vol 44 (1) ◽  
pp. 13-23 ◽  
Author(s):  
Laura Calzà ◽  
Mercedes Fernandez ◽  
Luciana Giardino
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Demyelinating Disease ◽  
Neurological Diseases ◽  
Critical Role ◽  
Precursor Cells ◽  
Demyelinating Diseases ◽  
New Frontier ◽  
Undifferentiated Cells ◽  
Spinal Cord Repair

Brain and spinal cord repair is a very difficult task in view of the extremely limited repair capability of the mature central nervous system (CNS). Thus, cellular therapies are regarded as a new frontier for both acute and chronic neurological diseases characterized by neuron or oligodendroglia degeneration. Although cell replacement has been considered as the primary goal of such approaches, in recent years greater attention has been devoted to the possibility that new undifferentiated cells in damaged nervous tissue might also act in autocrine–paracrine fashion, regulating the micro-environment through the release of growth factor and cytokines, also regulating immune response and local inflammation. In this review, repair of demyelinating disease using endogenous cells will be discussed in view of the critical role played by thyroid hormones (THs) during developmental myelination, focusing on the following points: 1) endogenous stem and precursor cells during demyelinating diseases; 2) TH homeostasis in the CNS; 3) cellular and molecular mechanism regulated by TH during developmental myelination and 4) a working hypothesis to develop a rationale for the use of THs to improve remyelination through endogenous stem and precursor cells in the course of demyelinating diseases.

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