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Published on: March 26, 2015
Fulminant demyelinating diseases
Megan R Rahmlow1, Orhun Kantarci
1Mayo Clinic, Rochester, MN, USA.
Abstract:
Fulminant demyelinating disease is a heading that covers acute disseminated encephalomyelitis and its variant acute hemorrhagic leukoencephalitis (Hurst disease), severe relapses of multiple sclerosis (MS), variants of MS (tumefactive MS, Marburg variant, Balo concentric sclerosis, myelinoclastic diffuse sclerosis), and neuromyelitis optica-spectrum disorders associated with aquaporin autoimmunity. These categories of inflammatory demyelinating disease often prompt hospital admission and many necessitate intensive care monitoring due to the aggressive nature of the illness and associated neurologic morbidity. In this review, we highlight the discriminating clinical, radiographic, and pathologic features of these disorders. Acute management is often accomplished with use of high-dose intravenous steroids and plasma exchange. Aggressive disease may respond to immunosuppression. Prognosis for recovery varies among the disorders but most patients improve. Factors influencing outcome are also discussed.
Insights
Fulminant demyelinating diseases, including acute disseminated encephalomyelitis and multiple sclerosis variants, require intensive care. Early diagnosis and treatment with steroids, plasma exchange, and immunosuppression improve patient outcomes.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Fulminant demyelinating diseases encompass severe inflammatory conditions affecting the central nervous system.
- These include acute disseminated encephalomyelitis, variants of multiple sclerosis, and neuromyelitis optica-spectrum disorders.
- Their aggressive nature often necessitates intensive care due to significant neurologic morbidity.
Purpose of the Study:
- To review the clinical, radiographic, and pathologic features of fulminant demyelinating diseases.
- To discuss current management strategies and factors influencing patient prognosis.
Main Methods:
- Literature review focusing on clinical presentation, diagnostic imaging, and histopathology.
- Analysis of treatment modalities including corticosteroids, plasma exchange, and immunosuppressive therapies.
Main Results:
- Discriminating features for each disorder are highlighted.
- High-dose intravenous steroids and plasma exchange are primary acute management strategies.
- Immunosuppression may be required for aggressive disease presentations.
Conclusions:
- Fulminant demyelinating diseases are severe but treatable neurological conditions.
- Prompt diagnosis and appropriate management are crucial for improving patient outcomes.
- Prognosis varies, but most patients show improvement with timely intervention.
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