Pathologic Factors and Spectrum of Central Nervous System Inflammatory Demyelinating Diseases

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.

Diagnosis of Multiple Sclerosis

10.1093/med/9780197512166.003.0065 ◽

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 ◽

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

Oxford Textbook of Medicine ◽

10.1093/med/9780198746690.003.0591 ◽

2020 ◽

pp. 6026-6042

Author(s):

Alasdair Coles ◽

Siddharthan Chandran

Keyword(s):

Multiple Sclerosis ◽

Central Nervous System ◽

Nervous System ◽

Demyelinating Disease ◽

Demyelinating Diseases ◽

System P ◽

Industrialized Nations ◽

Distinct Disease ◽

Demyelinating Disorders

The common feature of all of the demyelinating diseases is that, initially at least, the oligodendrocyte-myelin unit is the primary target, with the axon comparatively spared. There are a range of causes, both acquired and inherited, which this chapter explores. Multiple sclerosis, the prototypic demyelinating disorder of the central nervous system, is the leading causing of neurological disability among young adults in many industrialized nations. In the last two decades therapies have been licensed with increasing capacity to suppress the inflammation which underlies the condition, leading to durable benefits to patients. The next most prevalent demyelinating disease is neuromyelitis optica. Originally thought to be a variant of multiple sclerosis, it is now recognized to be a distinct disease whose treatment is radically different from multiple sclerosis.

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

International Journal of Surgical Pathology ◽

10.1177/1066896921993562 ◽

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 ◽

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.

Multiple Sclerosis and Other Inflammatory Demyelinating Diseases of the Central Nervous System

Neurology in Clinical Practice ◽

10.1016/b978-1-4377-0434-1.00079-7 ◽

2012 ◽

pp. 1283-1313 ◽

Cited By ~ 4

Author(s):

Maria K. Houtchens ◽

Fred D. Lublin ◽

Aaron E. Miller ◽

Samia J. Khoury

Keyword(s):

Multiple Sclerosis ◽

Central Nervous System ◽

Nervous System ◽

Demyelinating Diseases ◽

Demyelinating disorders of the central nervous system

Oxford Textbook of Medicine ◽

10.1093/med/9780199204854.003.0241002_update_001 ◽

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 ◽

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....

Immunology of Multiple Sclerosis

10.1093/med/9780199341016.003.0004 ◽

2016 ◽

Author(s):

Laura Piccio ◽

Anne H. Cross

Keyword(s):

Multiple Sclerosis ◽

Central Nervous System ◽

Nervous System ◽

Monoclonal Antibodies ◽

B Lymphocytes ◽

Autoimmune Disorder ◽

Innate Immune ◽

Immune Systems ◽

Multiple Sclerosis Treatment

Multiple sclerosis (MS) is considered to be an autoimmune disease of the central nervous system that targets myelin but affects both white matter and gray matter. Multiple sclerosis is thought to be mediated by cells of the adaptive and innate immune systems. CD4+ T lymphocytes of the Th1 and Th17 subtypes are believed to be critical for the initiation of multiple sclerosis. Treatment with monoclonal antibodies that deplete B lymphocytes has proven that B cells are critical to relapse development in multiple sclerosis. While immunopathophysiology is clearly important in MS, whether multiple sclerosis is truly an autoimmune disorder and the target or targets of the autoimmunity remain unknown.

Immunology and Oxidative Stress in Multiple Sclerosis: Clinical and Basic Approach

Clinical and Developmental Immunology ◽

10.1155/2013/708659 ◽

2013 ◽

Vol 2013 ◽

pp. 1-14 ◽

Cited By ~ 86

Author(s):

Genaro G. Ortiz ◽

Fermín P. Pacheco-Moisés ◽

Oscar K. Bitzer-Quintero ◽

Ana C. Ramírez-Anguiano ◽

Luis J. Flores-Alvarado ◽

...

Keyword(s):

Oxidative Stress ◽

Multiple Sclerosis ◽

Central Nervous System ◽

Nervous System ◽

Tissue Injury ◽

Autoimmune Disorder ◽

Demyelinating Lesions ◽

The Brain ◽

And Oxidative Stress

Multiple sclerosis (MS) exhibits many of the hallmarks of an inflammatory autoimmune disorder including breakdown of the blood-brain barrier (BBB), the recruitment of lymphocytes, microglia, and macrophages to lesion sites, the presence of multiple lesions, generally being more pronounced in the brain stem and spinal cord, the predominantly perivascular location of lesions, the temporal maturation of lesions from inflammation through demyelination, to gliosis and partial remyelination, and the presence of immunoglobulin in the central nervous system and cerebrospinal fluid. Lymphocytes activated in the periphery infiltrate the central nervous system to trigger a local immune response that ultimately damages myelin and axons. Pro-inflammatory cytokines amplify the inflammatory cascade by compromising the BBB, recruiting immune cells from the periphery, and activating resident microglia. inflammation-associated oxidative burst in activated microglia and macrophages plays an important role in the demyelination and free radical-mediated tissue injury in the pathogenesis of MS. The inflammatory environment in demyelinating lesions leads to the generation of oxygen- and nitrogen-free radicals as well as proinflammatory cytokines which contribute to the development and progression of the disease. Inflammation can lead to oxidative stress and vice versa. Thus, oxidative stress and inflammation are involved in a self-perpetuating cycle.

Blood serum amino acids profile in patients with Multiple Sclerosis

Current Issues in Pharmacy and Medical Sciences ◽

10.12923/j.2084-980x/26.1/a.10 ◽

2015 ◽

Vol 26 (1) ◽

pp. 50-53

Keyword(s):

Multiple Sclerosis ◽

Amino Acids ◽

Central Nervous System ◽

Nervous System ◽

Blood Serum ◽

Demyelinating Disease ◽

Initial Step ◽

Control Group ◽

Amino Acids Profile

Multiple sclerosis is the most common demyelinating disease of the central nervous system, affecting mostly young people. There were many risk factors for MS identified, however a direct cause of the disease is still unknown. Pathological changes in the SM lead to the myelin sheath damage around axons, what prevents proper transmission of nerve impulses in the central nervous system. The aim of this study was analyzing and comparing the amino acids profile in the blood serum of MS patients to control group of healthy individuals and evaluating the relationship between them. Significant (p<0.05) differences in the level of glutamate, aspartate and taurine in the blood serum of MS patients were revealed. A positive glutamate and aspartate level correlation in the serum has been demonstrated. Gender is significant only in the case of glutamate level in blood serum. The studies highlight the important role of neurotransmitters in MS and are the initial step in proteomic research.

Multiple Sclerosis and Other Inflammatory Demyelinating Diseases of the Central Nervous System

Neurology in Clinical Practice ◽

10.1016/b978-0-7506-7525-3.50092-3 ◽

2008 ◽

pp. 1583-1613 ◽

Cited By ~ 4

Author(s):

Fred D. Lublin ◽

Aaron E. Miller

Keyword(s):

Multiple Sclerosis ◽

Central Nervous System ◽

Nervous System ◽

Demyelinating Diseases ◽

Movement Disorders

Mayo Clinic Internal Medicine Board Review ◽

10.1093/med/9780190464868.003.0056 ◽

2016 ◽

pp. 619-624

Author(s):

Anhar Hassan ◽

Eduardo E. Benarroch

Keyword(s):

Multiple Sclerosis ◽

Central Nervous System ◽

Nervous System ◽

Young Adults ◽

Movement Disorders ◽

Demyelinating Disease ◽

Substantial Effect ◽

The most common inflammatory demyelinating disease of the central nervous system is multiple sclerosis, a disabling disorder that affects predominantly young adults between 20 and 50 years old. It affects women twice as often as men. Multiple sclerosis has a complex immunopathogenesis, variable prognosis, and an unpredictable course. Polygenic and environmental (possibly viral) factors probably have a substantial effect on susceptibility to multiple sclerosis.