CENTRAL MYELIN AND DEMYELINATION

2021 ◽  
pp. 77-79
Author(s):  
Moses P Moorthy ◽  
A.V. Srinivasan ◽  
S. Balasubramaniyan
Keyword(s):  
Multiple Sclerosis ◽  
Vital Signs ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Cervical Cord ◽  
Hyperintense Lesion ◽  
Csf Analysis ◽  
Central Demyelination ◽  
Demyelinating Disorders ◽  
Whole Spine

INTRODUCTION:CNS demyelinating disorders are common neurological illness that affects the brain. This causes signicant disability and mortality if not properly identied and treated. CNS demyelinating diseases includes multiple sclerosis, acute disseminated encephalomyelitis, NMO spectrum disorders and transverse myelits. Here we are reporting a case of central demyelination. CASE SUMMARY: 44yrs female admitted with subacute onset of weakness of all four limbs, more on left side and sensory disturbance in the form of numbness and tingling in all four limbs, more on left side, associated with urinary urgency and constipation. Patient had right optic neuritis 3 years back. General examination was normal. Vital signs were stable. On nervous system examination, patient had decresed visual acuity in right eye, with relative afferent pupillary defect and impaired red green colour vision, fundus was normal. Motor system examination, Patient had normal bulk, increased tone, brisk deep tendon reexes, power of 4 on right and 4- on left side with extensor planter on both sides. Patient had decresed sensation below C5 spinal cord level, predominantly joint position and vibration sense with positive lhermitte's sign. Other systems were normal. Routine lab investigation ,ECG,CXR were normal. CSF analysis revealed elevated protein of 72mg. MRI BRAIN WITH WHOLE SPINE SCREENING(contrast) revealed Multiple smallT2/FLAIR hyperintense lesion in bilateral juxta cortical, subcortical(involving the temporal lobes and callososeptal interface), subtle patchy enhancement in the left peritrigonal lesion and in body of corpus callosum. Mild atrophy of cervical cord from C3 to C5 with T2 hyperintense involving both hemicord from C2 to C5 level, focal Hyperintensity subtle contrast enhancement in Dorsal cord at the level of D11, Features suggestive of demyelination likely Multiple Sclerosis.

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....

Multiple Sclerosis and Related Disorders

2014 ◽  
Author(s):  
J William Lindsey
Keyword(s):  
Multiple Sclerosis ◽  
Differential Diagnosis ◽  
Neuromyelitis Optica ◽  
Subacute Sclerosing Panencephalitis ◽  
Large Diameter ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Pathologic Features ◽  
Relapsing Remitting

Multiple sclerosis (MS) is a relatively common cause of neurologic symptoms and disability in young adults. The distinguishing pathologic features of MS are loss of myelin and inflammation in the central nervous system (CNS). The myelin sheath is essential for rapid conduction of nerve signals along large-diameter axons. Oligodendrocytes produce and maintain myelin in the CNS, and Schwann cells produce and maintain myelin in the peripheral nerves. In addition to MS, there are a number of related disorders causing demyelination, inflammation, or both in the CNS. This chapter discusses MS and related disorders, including neuromyelitis optica, optic neuritis, acute disseminated encephalomyelitis, transverse myelitis, Behçet syndrome, neurosarcoidosis, inherited demyelinating diseases (leukodystrophies, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy [CADASIL]), and virus-induced demyelination (progressive multifocal leukoencephalopathy, subacute sclerosing panencephalitis). The section on MS covers epidemiology, etiology/genetics, pathogenesis, diagnosis, differential diagnosis, management, and prognosis. Figures include organization of the microenvironment of larger-diameter axons, typical magnetic resonance imaging findings in MS and neuromyelitis optica, postgadolinium images of the cervical spine in MS, and an approach to treatment of relapsing-remitting MS. Tables list MS and related disorders, distribution of neurologic deficits at the onset of MS, differential diagnosis of MS, disease-modifying therapies for relapsing-remitting MS, and selected leukodystrophies, as well as diagnostic criteria and selected symptomatic therapies for MS. This review contains 3 highly rendered figures, 7 tables, and 82 references.

Multiple Sclerosis and Related Disorders

2015 ◽  
Author(s):  
J William Lindsey
Keyword(s):  
Multiple Sclerosis ◽  
Differential Diagnosis ◽  
Neuromyelitis Optica ◽  
Subacute Sclerosing Panencephalitis ◽  
Large Diameter ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Pathologic Features ◽  
Relapsing Remitting

Multiple sclerosis (MS) is a relatively common cause of neurologic symptoms and disability in young adults. The distinguishing pathologic features of MS are loss of myelin and inflammation in the central nervous system (CNS). The myelin sheath is essential for rapid conduction of nerve signals along large-diameter axons. Oligodendrocytes produce and maintain myelin in the CNS, and Schwann cells produce and maintain myelin in the peripheral nerves. In addition to MS, there are a number of related disorders causing demyelination, inflammation, or both in the CNS. This chapter discusses MS and related disorders, including neuromyelitis optica, optic neuritis, acute disseminated encephalomyelitis, transverse myelitis, Behçet syndrome, neurosarcoidosis, inherited demyelinating diseases (leukodystrophies, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy [CADASIL]), and virus-induced demyelination (progressive multifocal leukoencephalopathy, subacute sclerosing panencephalitis). The section on MS covers epidemiology, etiology/genetics, pathogenesis, diagnosis, differential diagnosis, management, and prognosis. Figures include organization of the microenvironment of larger-diameter axons, typical magnetic resonance imaging findings in MS and neuromyelitis optica, postgadolinium images of the cervical spine in MS, and an approach to treatment of relapsing-remitting MS. Tables list MS and related disorders, distribution of neurologic deficits at the onset of MS, differential diagnosis of MS, disease-modifying therapies for relapsing-remitting MS, and selected leukodystrophies, as well as diagnostic criteria and selected symptomatic therapies for MS.   This chapter contains 3 highly rendered figures, 7 tables, 82 references, 1 teaching slide set, and 5 MCQs.

International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 definitions

2013 ◽  
Vol 19 (10) ◽  
pp. 1261-1267 ◽  
Author(s):  
Lauren B Krupp ◽  
Marc Tardieu ◽  
Maria Pia Amato ◽  
Brenda Banwell ◽  
Tanuja Chitnis ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Acute Disseminated Encephalomyelitis ◽  
Study Group ◽  
Pediatric Multiple Sclerosis ◽  
Immune Mediated ◽  
International Pediatric ◽  
Demyelinating Disorders ◽  
Multiple Sclerosis Study Group

Background: There has been tremendous growth in research in pediatric multiple sclerosis (MS) and immune mediated central nervous system demyelinating disorders since operational definitions for these conditions were first proposed in 2007. Further, the International Pediatric Multiple Sclerosis Study Group (IPMSSG), which proposed the criteria, has expanded substantially in membership and in its international scope. Objective: The purpose of this review is to revise the 2007 definitions in order to incorporate advances in delineating the clinical and neuroradiologic features of these disorders. Methods: Through a consensus process, in which input was sought from the 150 members of the Study Group, criteria were drafted, revised and finalized. Final approval was sought through a web survey. Results: Revised criteria are proposed for pediatric acute disseminated encephalomyelitis, pediatric clinically isolated syndrome, pediatric neuromyelitis optica and pediatric MS. These criteria were approved by 93% or more of the 56 Study Group members who responded to the final survey. Conclusions: These definitions are proposed for clinical and research purposes. Their utility will depend on the outcomes of their application in prospective research.

scholarly journals Differential diagnosis of suspected multiple sclerosis: a consensus approach

2008 ◽  
Vol 14 (9) ◽  
pp. 1157-1174 ◽  
Author(s):  
DH Miller ◽  
BG Weinshenker ◽  
M Filippi ◽  
BL Banwell ◽  
JA Cohen ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Differential Diagnosis ◽  
Evidence Base ◽  
Demyelinating Diseases ◽  
Future Research ◽  
International Panel ◽  
The Central Nervous System ◽  
Definition Of ◽  
Demyelinating Disorders ◽  
Diagnosis Criteria

Background and objectives Diagnosis of multiple sclerosis (MS) requires exclusion of diseases that could better explain the clinical and paraclinical findings. A systematic process for exclusion of alternative diagnoses has not been defined. An International Panel of MS experts developed consensus perspectives on MS differential diagnosis. Methods Using available literature and consensus, we developed guidelines for MS differential diagnosis, focusing on exclusion of potential MS mimics, diagnosis of common initial isolated clinical syndromes, and differentiating between MS and non-MS idiopathic inflammatory demyelinating diseases. Results We present recommendations for 1) clinical and paraclinical red flags suggesting alternative diagnoses to MS; 2) more precise definition of “clinically isolated syndromes” (CIS), often the first presentations of MS or its alternatives; 3) algorithms for diagnosis of three common CISs related to MS in the optic nerves, brainstem, and spinal cord; and 4) a classification scheme and diagnosis criteria for idiopathic inflammatory demyelinating disorders of the central nervous system. Conclusions Differential diagnosis leading to MS or alternatives is complex and a strong evidence base is lacking. Consensus-determined guidelines provide a practical path for diagnosis and will be useful for the non-MS specialist neurologist. Recommendations are made for future research to validate and support these guidelines. Guidance on the differential diagnosis process when MS is under consideration will enhance diagnostic accuracy and precision.

scholarly journals A Case of Acute Encephalopathy Due to Autoimmune Overload

2020 ◽  
Vol 5 (6) ◽  
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Pediatric Population ◽  
Autoimmune Disorder ◽  
Acute Disseminated Encephalomyelitis ◽  
Acute Encephalopathy ◽  
Demyelinating Disorder ◽  
Demyelinating Disorders ◽  
Brain And Spinal Cord ◽  
The Brain

Acute disseminated encephalomyelitis, also known as postinfectious encephalomyelitis, is considered an autoimmune disorder that causes inflammation of the brain and spinal cord. It was seen mainly in pediatric population possibly due to vaccination but there have been cases identified in adult [1-9]. Acute disseminated encephalomyelitis can be challenging to diagnose owing to fact that there have been many overlapping symptoms among other demyelinating disorder such multiple sclerosis and Neuromyelitis Optica. In this case report, we will discuss a case about a patient that presented due to acute encephalopathy and was noted to have an atypical MRI of the brain that was not consistent with results of the lumbar puncture [10-12]. Knowledge gained from this case will help bring awareness to the diagnose of acute disseminated encephalomyelitis and how imaging in context with the clinical picture can help us differentiate between the various demyelinating disorders; thereby, giving a better understanding of managing these patients as management can affect prognosis and outcomes.

The Importance and Utility of Cerebrospinal Fluid Evaluation in the Diagnosis of Central Demyelinating Diseases

2016 ◽  
Author(s):  
Mark S. Freedman ◽  
Mohammad Abdoli
Keyword(s):  
Multiple Sclerosis ◽  
Cerebrospinal Fluid ◽  
Prognostic Value ◽  
Clinically Isolated Syndrome ◽  
Demyelinating Diseases ◽  
Response To Treatment ◽  
Disease Subtype ◽  
Csf Analysis ◽  
Diagnostic And Prognostic Value ◽  
Csf Findings

This chapter aims to highlight the diagnostic and prognostic value of cerebrospinal fluid (CSF) findings in multiple sclerosis with a special consideration of distinguishing it from neuromyelitis optica (NMO) and NMO spectrum disorder. Interpretation of CSF findings in daily clinical practice in patients with MS is thoroughly explained. New advances in CSF analysis and recently identified biomarkers may be helpful for diagnosis, help elucidate disease subtype and activity, or aid in prognosis and monitoring of the response to treatment. Characteristics of CSF changes in different subtypes of multiple sclerosis, clinically isolated syndrome (CIS), and radiologically isolated syndrome (RIS) are discussed. CSF findings in NMO spectrum disease as a diagnostic and differentiating marker are explained separately.

Multiple Sclerosis and Related Disorders

2015 ◽  
Author(s):  
J William Lindsey
Keyword(s):  
Multiple Sclerosis ◽  
Differential Diagnosis ◽  
Neuromyelitis Optica ◽  
Subacute Sclerosing Panencephalitis ◽  
Large Diameter ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Pathologic Features ◽  
Relapsing Remitting

Multiple sclerosis (MS) is a relatively common cause of neurologic symptoms and disability in young adults. The distinguishing pathologic features of MS are loss of myelin and inflammation in the central nervous system (CNS). The myelin sheath is essential for rapid conduction of nerve signals along large-diameter axons. Oligodendrocytes produce and maintain myelin in the CNS, and Schwann cells produce and maintain myelin in the peripheral nerves. In addition to MS, there are a number of related disorders causing demyelination, inflammation, or both in the CNS. This chapter discusses MS and related disorders, including neuromyelitis optica, optic neuritis, acute disseminated encephalomyelitis, transverse myelitis, Behçet syndrome, neurosarcoidosis, inherited demyelinating diseases (leukodystrophies, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy [CADASIL]), and virus-induced demyelination (progressive multifocal leukoencephalopathy, subacute sclerosing panencephalitis). The section on MS covers epidemiology, etiology/genetics, pathogenesis, diagnosis, differential diagnosis, management, and prognosis. Figures include organization of the microenvironment of larger-diameter axons, typical magnetic resonance imaging findings in MS and neuromyelitis optica, postgadolinium images of the cervical spine in MS, and an approach to treatment of relapsing-remitting MS. Tables list MS and related disorders, distribution of neurologic deficits at the onset of MS, differential diagnosis of MS, disease-modifying therapies for relapsing-remitting MS, and selected leukodystrophies, as well as diagnostic criteria and selected symptomatic therapies for MS.   This chapter contains 3 highly rendered figures, 7 tables, 82 references, 1 teaching slide set, and 5 MCQs.

Highly reactive anti-myelin oligodendrocyte glycoprotein antibodies differentiate demyelinating diseases from viral encephalitis in children

2010 ◽  
Vol 17 (3) ◽  
pp. 297-302 ◽  
Author(s):  
PH Lalive ◽  
MG Häusler ◽  
H Maurey ◽  
Y Mikaeloff ◽  
M Tardieu ◽  
...  
Keyword(s):  
Viral Encephalitis ◽  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Demyelinating Diseases ◽  
Barr Virus ◽  
The Central Nervous System ◽  
Mog Antibodies ◽  
Epstein Barr ◽  
Low Sensitivity ◽  
Demyelinating Disorders

Background: Myelin oligodendrocyte glycoprotein (MOG) may be implicated in the immunopathogenesis of multiple sclerosis (MS) inducing demyelination in the animal model of MS. In adults reported anti-MOG antibody frequencies have been variable across a number of studies and can also be detected in controls. Objective: To measure antibodies against MOG in paediatric patients with demyelinating disorders of the central nervous system and in controls. Methods: Serum antibodies against MOG and myelin basic protein were measured by ELISA, flow cytometry (FACS) and in the liquid phase in 11 children with acute disseminated encephalomyelitis (ADEM), 22 children with MS, seven children with acute viral encephalitis and 13 healthy controls. The serostatus of Epstein–Barr virus (EBV) infections were assessed. Results: Anti-MOG antibodies, measured either by ELISA or FACS were exclusively detected in children with demyelination. In ADEM these antibodies were highly reactive. Anti-MBP reactivity was detectable equally in all groups. The presence of either autoantibodies did not associate with EBV serostatus, age, gender or disease course. Conclusions: This study independently corroborates recently published results of seroprevalence and specificity of the assay. Due to their low sensitivity anti-MOG antibodies will not serve as disease-specific biomarkers, but could help to support the diagnosis of ADEM in difficult cases.

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
Keyword(s):  
Multiple Sclerosis ◽  
Demyelinating Disease ◽  
State Of The Art ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Diagnostic Process ◽  
Imaging Features ◽  
Indonesian Population ◽  
The Central Nervous System ◽  
Medical Advances

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.

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