scholarly journals State-of-the-Art Review: Demyelinating Diseases in Indonesia

2021 ◽  
Vol 2021 ◽  
pp. 1-15
Author(s):  
Hana Larassati ◽  
Riwanti Estiasari ◽  
Reyhan E. Yunus ◽  
Paul M. Parizel

Demyelinating diseases are more common in Indonesia than previously believed. However, it is still a challenge for a country such as Indonesia to implement the scientific medical advances, especially in the diagnostic process of demyelinating diseases, to achieve the best possible outcome for these groups of patients, within the constraints of what is socially, technologically, economically, and logistically achievable. In this review, we address the 4 major classes of demyelinating disease: multiple sclerosis (MS), neuromyelitis optica (NMO), anti-MOG-associated encephalomyelitis (MOG-EM), and acute disseminated encephalomyelitis (ADEM), and discuss their prevalence, demographics, clinical diagnosis workup, and imaging features in the Indonesian population, as well as the challenges we face in their diagnosis and therapeutic approach. We hope that this overview will lead to a better awareness of the spectrum of demyelinating diseases of the central nervous system in Indonesia.

2021 ◽  
pp. 540-547
Author(s):  
W. Oliver Tobin

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.


2021 ◽  
pp. 106689692199356
Author(s):  
Fleur Cordier ◽  
Lars Velthof ◽  
David Creytens ◽  
Jo Van Dorpe

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.


Author(s):  
Siddharthan Chandran ◽  
Alastair Compston

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....


Author(s):  
G. Chuprina

Multiple sclerosis is a chronic inflammatory demyelinating disease of an infectious and allergic origin, which usually begins at a young age, manifested by signs of a multifocal lesion mainly in the central nervous system, it has a remitting, less progressive nature of the course, often leads to disability. Multiple sclerosis is the most famous and widespread throughout the globe demyelinating diseases of the nervous system. According to the prevalence, it ranks fourth after cerebral stroke, epilepsy and parkinsonism, and is among the so-called "Four riders of the neurological apocalypse", according to their medical consequences and social consequences. The main variants of the development of multiple sclerosis are remitting, secondary and primary-progressive flow. The remitting flow (85-90% of patients in the early stages of the disease) is characterized by pronounced exacerbations followed by complete or partial restoration of impaired functions without signs of progression during remission periods. With secondary progressive scattered sclerosis, the remitting nature changes with a gradual increase in neurological symptoms with or without rare exacerbations, with minor stabilization or remission periods. A similar transformation is observed 10 years after the onset of the disease in almost 50% of patients, and in 25 years - in 80% of patients. At the primary progressive scattered (10-15%) progression is noted from the very beginning with isolated periods of stabilization and temporary minor improvement. Etiology, pathogenesis, clinic, diagnostics, differential diagnosis, treatment of multiple sclerosis are considered.


2021 ◽  
Author(s):  
MV Melnikov ◽  
VS Rogovskii ◽  
AV Lopatina ◽  
AA Sviridova ◽  
AI Volkov ◽  
...  

Multiple sclerosis (MS) is a chronic demyelinating disease of the central nervous system involving autoimmune mechanisms. MS has been treated as a disorder mediated mainly by T cells for a long time. However, recent findings demonstrate that B lymphocytes are of crucial pathogenetic significance in MS. In patients with MS, B cells can possess both pro-inflammatory and anti-inflammatory effects. The paper reports the main mechanisms of B lymphocyte involvement in the pathogenesis of MS. Diagnostic value of assessing humoral immune parameters in individuals with demyelinating diseases and modern possibility of B cell function modulation are discussed.


2020 ◽  
Vol 21 (21) ◽  
pp. 8409
Author(s):  
Marina Boziki ◽  
Styliani-Aggeliki Sintila ◽  
Panagiotis Ioannidis ◽  
Nikolaos Grigoriadis

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.


2021 ◽  
pp. 531-539
Author(s):  
Claudia F. Lucchinetti ◽  
Yong Guo

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.


2020 ◽  
pp. 6026-6042
Author(s):  
Alasdair Coles ◽  
Siddharthan Chandran

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.


2013 ◽  
Vol 71 (9B) ◽  
pp. 685-688 ◽  
Author(s):  
Sandro Luiz de Andrade Matas ◽  
Felipe von Glehn ◽  
Gustavo Bruniera Peres Fernandes ◽  
Carlos Augusto Senne Soares

The central nervous system demyelinating diseases are a group of disorders with different etiologies, characterized by inflammatory lesions that are associated with loss of myelin and eventually axonal damage. In this group the most studied ones are multiple sclerosis (MS), neuromyelitis optic (NMO) and acute disseminated encephalomyelitis (ADEM). The cerebrospinal fluid is essential to differentiate between these different syndromes and to define multiple sclerosis, helping to assess the probability of Clinical Isolated Syndrome turn into multiple sclerosis.


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