Multiple Sclerosis and Related Inflammatory Demyelinating Diseases

2018 ◽  
pp. 172-185
Author(s):  
Hans Lassmann ◽  
Danielle Seilhean
Keyword(s):  
Multiple Sclerosis ◽  
Neuromyelitis Optica ◽  
Gray Matter ◽  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Scar Formation ◽  
Demyelinating Diseases ◽  
Primary Demyelination ◽  
Similarities And Differences ◽  
Progressive Stage

This chapter describes the basic neuropathology of multiple sclerosis and other inflammatory demyelinating diseases. It specifically addresses the nature of multiple sclerosis lesions, characterized by inflammation, selective primary demyelination with partial preservation of axons, remyelination, and reactive astroglial scar formation. Attention is paid to the importance of demyelinated lesions in the gray matter, and it discusses the differences of the pathology between the relapsing and the progressive stage of the disease. Finally, it summarizes the similarities and differences between multiple sclerosis and other inflammatory demyelinating diseases, such as acute multiple sclerosis, Baló concentric sclerosis, neuromyelitis optica, myelin oligodendrocyte glycoprotein antibody-associated disease and acute disseminated encephalomyelitis.

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.

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.

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.

Multiple Sclerosis and Related Inflammatory Demyelinating Diseases

2013 ◽  
pp. 161-172
Author(s):  
Hans Lassmann ◽  
Raymond A. Sobel ◽  
Danielle Seilhean
Keyword(s):  
Multiple Sclerosis ◽  
Chronic Inflammation ◽  
Neuromyelitis Optica ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Selective Destruction ◽  
Myelin Sheaths ◽  
Morphologic Changes

This chapter describes and illustrates the morphologic changes observed in inflammatory demyelinating diseases. These are defined by the selective destruction of myelin sheaths and oligodendrocytes, arising in a background of acute or chronic inflammation. The spectrum of inflammatory demyelinating diseases includes multiple sclerosis, acute disseminated encephalomyelitis, Balo concentric sclerosis, and neuromyelitis optica. The etiology and pathogenesis of the diseases are discussed and criteria for the diagnosis of inflammatory demyelinating diseases on brain biopsies are provided.

Epidemiology of Demyelinating Diseases in Korean Pediatric Patients

2020 ◽  
Vol 36 (2) ◽  
pp. 141-147
Author(s):  
Hye Hyun Yoon ◽  
Ji Young Park ◽  
Su Yeong Kim ◽  
Na Mi Lee ◽  
Dae Yong Yi ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Neuromyelitis Optica ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Guillain Barre Syndrome ◽  
Korean Children ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

The epidemiology of demyelinating diseases in the Korean pediatric population has not been reported to date. This study aimed to identify the epidemiology of demyelinating diseases in Korean children by using big data. The subjects were children (0-17 years old) diagnosed with acute-disseminated encephalomyelitis, multiple sclerosis, neuromyelitis optica, and Guillain-Barré syndrome enrolled in the Korean Health Insurance Review and Assessment Service (HIRA) from January 2010 to December 2017. Of 1722 enrolled children, 553 (32.1%) had acute-disseminated encephalomyelitis, 170 (9.9%) had multiple sclerosis, 68 (3.9%) had neuromyelitis optica, and 931 (54.1%) had Guillain-Barré syndrome. The male-female ratios were 1.47:1 in acute-disseminated encephalomyelitis, 1.43:1 in Guillain-Barré syndrome, 1:1.66 in multiple sclerosis, and 1:1.62 in neuromyelitis optica. Demyelinating diseases were most prevalent in summer. The prevalence differed by region, with 545 (31.6%) in Seoul and 298 (17.3%) in Gyeonggi. This study is the first to identify the incidence of demyelinating diseases in South Korea.

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.

Comparison of deep gray matter lesions on magnetic resonance imaging among adults with acute disseminated encephalomyelitis, multiple sclerosis, and neuromyelitis optica

2013 ◽  
Vol 20 (4) ◽  
pp. 418-423 ◽  
Author(s):  
Lei Zhang ◽  
Aimin Wu ◽  
Bingjun Zhang ◽  
Suqin Chen ◽  
Xuejiao Men ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Multiple Sclerosis ◽  
Magnetic Resonance ◽  
Neuromyelitis Optica ◽  
Gray Matter ◽  
Acute Disseminated Encephalomyelitis ◽  
Morphological Features ◽  
Adult Patients ◽  
Resonance Imaging ◽  
Deep Gray Matter

Background: Deep gray matter lesions have been reported in patients with acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), and neuromyelitis optica (NMO). Objectives: The purpose of this study was to compare the features of deep gray matter lesions on magnetic resonance imaging (MRI) among adult patients with ADEM, MS, and NMO. Methods: Ninety-five adult patients with ADEM ( n=12), MS ( n=60), and NMO ( n=23) who had deep gray matter lesions on MRI were enrolled. Morphological features of deep gray matter lesions among these patients were assessed. Results: Putamen involvement was more common in patients with ADEM than in patients with MS and NMO. Differing from children, thalamus involvement might not be helpful in differentiating ADEM from MS in adults. Hypothalamus involvement was more common in patients with NMO than in patients with ADEM and MS. More importantly, bilateral hypothalamus involvement was more helpful in differentiating NMO from MS. The diameter of the thalamus lesions in patients with ADEM was larger than that in patients with NMO. Conclusions: Morphological features of deep gray matter lesions vary among adult patients with ADEM, MS, and NMO, and may be helpful in distinguishing these diseases.

scholarly journals Multiple Sclerosis in Malaysia: Demographics, Clinical Features, and Neuroimaging Characteristics

2013 ◽  
Vol 2013 ◽  
pp. 1-10 ◽  
Author(s):  
S. Viswanathan ◽  
N. Rose ◽  
A. Masita ◽  
J. S. Dhaliwal ◽  
S. D. Puvanarajah ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Neuromyelitis Optica ◽  
Demyelinating Disease ◽  
Age At Onset ◽  
Positive Family History ◽  
Disease Onset ◽  
Demyelinating Diseases ◽  
Lesion Length ◽  
Relapsing Remitting ◽  
Spectrum Disorders

Background. Multiple sclerosis (MS) is an uncommon disease in multiracial Malaysia. Diagnosing patients with idiopathic inflammatory demyelinating diseases has been greatly aided by the evolution in diagnostic criterion, the identification of new biomarkers, and improved accessibility to neuroimaging in the country.Objectives. To investigate the spectrum of multiple sclerosis in Malaysia.Methods. Retrospective analysis with longitudinal follow-up of patients referred to a single tertiary medical center with neurology services in Malaysia.Results. Out of 245 patients with idiopathic inflammatory demyelinating disease, 104 patients had multiple sclerosis. Female to male ratio was 5 : 1. Mean age at onset was 28.6 ± 9.9 years. The Malays were the predominant racial group affected followed by the Chinese, Indians, and other indigenous groups. Subgroup analysis revealed more Chinese having neuromyelitis optica and its spectrum disorders rather than multiple sclerosis. Positive family history was reported in 5%. Optic neuritis and myelitis were the commonest presentations at onset of disease, and relapsing remitting course was the commonest disease pattern observed. Oligoclonal band positivity was 57.6%. At disease onset, 61.5% and 66.4% fulfilled the 2005 and 2010 McDonald’s criteria for dissemination in space. Mean cord lesion length was 1.86 ± 1.65 vertebral segments in the relapsing remitting group as opposed to 6.25 ± 5.18 vertebral segments in patients with neuromyelitis optica and its spectrum disorders.Conclusion. The spectrum of multiple sclerosis in Malaysia has changed over the years. Further advancement in diagnostic criteria will no doubt continue to contribute to the evolution of this disease here.

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