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Autoimmune demyelinating diseases of the central nervous system (CNS) encompass a broad spectrum of inflammatory disorders characterized by immune-mediated damage to myelin. This chapter provides a comprehensive overview of key diseases including acute disseminated encephalomyelitis (ADEM), acute hemorrhagic leukoencephalitis (AHLE), autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy, Baló’s concentric sclerosis, combined central and peripheral demyelination (CCPD), idiopathic acute transverse myelitis (IATM), Marburg’s variant of multiple sclerosis (MS), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), neuromyelitis optica spectrum disorders (NMOSD), solitary sclerosis, or tumefactive MS. For each condition, the chapter addresses epidemiology, pathophysiology, clinical features, neuroimaging findings, cerebrospinal fluid profiles, and differential diagnoses. Detailed attention is given to radiologic red flags, such as lesion distribution and enhancement patterns, which aid in distinguishing these diseases from MS, MOGAD, and NMOSD. Specific diagnostic challenges are emphasized, including overlapping clinical and radiologic features, the role of antibody testing (e.g., anti-AQP4, anti-MOG, anti-GFAP, and neurofascin-155), and the evolution of disease classification as novel biomarkers emerge. The chapter also summarizes current therapeutic strategies—ranging from corticosteroids and plasma exchange to long-term immunosuppression—and outlines prognostic considerations. Although some diseases, such as ADEM and GFAP astrocytopathy, are typically monophasic with favorable outcomes, others may follow a relapsing or fulminant course requiring aggressive treatment. The importance of early recognition and tailored management is underscored throughout, particularly for rare but severe conditions such as AHLE and Marburg’s variant of MS. This chapter serves as a practical and up-to-date reference for clinicians working in the complex field of autoimmune demyelinating diseases of the CNS.

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Autoimmune Demyelinating Diseases of Central Nervous System (CNS)

  • 章节

  • 发表日期：

    2025年

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  • Tomas Uher

摘要

Autoimmune demyelinating diseases of the central nervous system (CNS) encompass a broad spectrum of inflammatory disorders characterized by immune-mediated damage to myelin. This chapter provides a comprehensive overview of key diseases including acute disseminated encephalomyelitis (ADEM), acute hemorrhagic leukoencephalitis (AHLE), autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy, Baló’s concentric sclerosis, combined central and peripheral demyelination (CCPD), idiopathic acute transverse myelitis (IATM), Marburg’s variant of multiple sclerosis (MS), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), neuromyelitis optica spectrum disorders (NMOSD), solitary sclerosis, or tumefactive MS. For each condition, the chapter addresses epidemiology, pathophysiology, clinical features, neuroimaging findings, cerebrospinal fluid profiles, and differential diagnoses. Detailed attention is given to radiologic red flags, such as lesion distribution and enhancement patterns, which aid in distinguishing these diseases from MS, MOGAD, and NMOSD. Specific diagnostic challenges are emphasized, including overlapping clinical and radiologic features, the role of antibody testing (e.g., anti-AQP4, anti-MOG, anti-GFAP, and neurofascin-155), and the evolution of disease classification as novel biomarkers emerge. The chapter also summarizes current therapeutic strategies—ranging from corticosteroids and plasma exchange to long-term immunosuppression—and outlines prognostic considerations. Although some diseases, such as ADEM and GFAP astrocytopathy, are typically monophasic with favorable outcomes, others may follow a relapsing or fulminant course requiring aggressive treatment. The importance of early recognition and tailored management is underscored throughout, particularly for rare but severe conditions such as AHLE and Marburg’s variant of MS. This chapter serves as a practical and up-to-date reference for clinicians working in the complex field of autoimmune demyelinating diseases of the CNS.