Idiopathic inflammatory demyelining diseases: optimization of early diagnosis, predictors of the course

Objective: to describe the clinical and epidemiological features of idiopathic inflammatory demyelinating diseases and to determine the factors influencing their course. Materials and methods: the study included 803 patients with idiopathic inflammatory demyelinating diseases using patient questionnaires and scales, laboratory and instrumental research methods. Statistical processing of the results was carried out using a point biserial coefficient and programs for analyzing large data arrays and machine learning. Results: a dynamic increase in the prevalence of some forms of idiopathic inflammatory demyelinating diseases was revealed, the difficulties of differential diagnosis of rare forms of demyelination and the need to create a unified version of their classification are reflected. In the studied population, the effectiveness of liquorological examination in the diagnosis of multiple sclerosis in the early stages of the disease was shown. It has been shown that the likelihood of developing highly active multiple sclerosis is influenced by both a genetic factor and concomitant inflammatory, allergic and autoimmune diseases, surgical interventions, dietary habits, childhood infections, and a history of pregnancy. Conclusions: given the complexity of the differential diagnosis of idiopathic inflammatory demyelinating diseases and the appointment of modifying therapy in multiple sclerosis in the early stages, it is necessary to create a combined classification and maintain a common register.

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Multiple Sclerosis and Related Disorders

10.2310/fm.6040 ◽

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.

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Multiple Sclerosis and Related Disorders

10.2310/neuro.6040 ◽

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.

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Differential diagnosis of suspected multiple sclerosis: a consensus approach

Multiple Sclerosis Journal ◽

10.1177/1352458508096878 ◽

2008 ◽

Vol 14 (9) ◽

pp. 1157-1174 ◽

Cited By ~ 392

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.

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Multiple Sclerosis and Related Disorders

10.2310/psych.6040 ◽

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.

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Central vein sign for differential diagnosis of demyelinating diseases of CNS

10.47183/mes.2021.021 ◽

2021 ◽

Author(s):

SE Belov ◽

IL Gubsky ◽

VG Lelyuk ◽

AN Boyko

Keyword(s):

Multiple Sclerosis ◽

Systemic Lupus Erythematosus ◽

Differential Diagnosis ◽

Lupus Erythematosus ◽

Demyelinating Diseases ◽

Central Vein ◽

Systemic Lupus ◽

Multiple Sclerosis Case ◽

Systemic Disorder ◽

Secondary Demyelination

The search for highly sensitive and highly specific biomarkers of MS, including neuroimaging biomarkers, continues. One of such biomarkers is the central vein sign detectable on SW and T2-weighted MR images. The sensitivity and specificity of methods used for central vein sign detection vary. This article describes two clinical cases of patients with similar neurological symptoms which required making differential diagnosis between multiple sclerosis and secondary demyelination in the presence of a systemic disorder (systemic lupus erythematosus). In addition to routine MR sequences, we used SWI generated by a 3T scanner. The lesions with the central vein sign were counted; the proportion of perivenular lesions was determined. In the multiple sclerosis case, all the lesions were perivenular; the proportion of lesions with the central vein sign in the patient with secondary demyelination in the presence of systemic lupus erythematosus was 16.7%. The use of SW images improved the informative value of the analysis.

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Multiple sclerosis: etiology, pathogenesis, clinic, diagnosis, differential diagnosis, treatment (clinical lecture)

East European Journal of Neurology ◽

10.33444/2411-5797.2017.5(17).27-37 ◽

2017 ◽

pp. 27-37

Author(s):

G. Chuprina

Keyword(s):

Multiple Sclerosis ◽

Nervous System ◽

Differential Diagnosis ◽

Demyelinating Disease ◽

Social Consequences ◽

Demyelinating Diseases ◽

Cerebral Stroke ◽

Partial Restoration ◽

Primary Progressive ◽

The Central Nervous System

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.

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Magnetic Resonance Imaging in the Differential Diagnosis of Multiple Sclerosis and Other Demyelinating Diseases

Journal of radiology and nuclear medicine ◽

10.20862/0042-4676-2019-100-4-229-236 ◽

2019 ◽

Vol 100 (4) ◽

pp. 229-236

Author(s):

I. A. Krotenkova ◽

V. V. Bryukhov ◽

R. N. Konovalov ◽

M. N. Zakharova ◽

M. V. Krotenkova

Keyword(s):

Magnetic Resonance Imaging ◽

Multiple Sclerosis ◽

Differential Diagnosis ◽

Magnetic Resonance ◽

Correct Diagnosis ◽

Laboratory Data ◽

Demyelinating Diseases ◽

Resonance Imaging ◽

Confirmatory Tests ◽

Magnetic Resonance Imaging Mri

The diagnosis of multiple sclerosis (MS) is quite complicated, which is associated with its clinical features and the lack of unique confirmatory tests. Magnetic resonance imaging (MRI) is one of the ways to confirm the diagnosis and also makes it possible to establish a differential diagnosis with other demyelinating diseases and to exclude diseases that mimic MS. This review presents not only MRI criteria for MS and other diseases similar to the MRI pattern, but also additional clinical and laboratory data, without which it is impossible to make a correct diagnosis.

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