Marburg acute multiple sclerosis

Marburg acute multiple sclerosis, also known as Marburg variant of multiple sclerosis or Marburg disease, is an extremely rare and fulminant form of multiple sclerosis (MS) characterized by a rapidly progressive course leading to severe disability or death within weeks to months of onset. Unlike typical relapsing-remitting or progressive forms of MS, Marburg variant follows a monophasic or rapidly progressive course with extensive, destructive demyelination of the central nervous system (CNS), primarily affecting the brain and spinal cord white matter. The disease presents with acute and severe neurological symptoms including rapid cognitive decline, altered consciousness progressing to stupor or coma, hemiparesis or quadriparesis, seizures, visual loss, aphasia, and brainstem dysfunction. MRI typically reveals large, tumefactive, ring-enhancing demyelinating lesions that may be mistaken for brain tumors or acute disseminated encephalomyelitis (ADEM). Pathologically, the lesions show massive macrophage infiltration, acute axonal destruction, and severe demyelination, often with necrotic features that distinguish them from typical MS plaques. There is no established standard treatment for Marburg acute MS. Management is largely empirical and may include high-dose intravenous corticosteroids, plasma exchange (plasmapheresis), intravenous immunoglobulin (IVIG), and aggressive immunosuppressive therapies such as cyclophosphamide or mitoxantrone. Despite treatment, the prognosis remains very poor, with many patients succumbing to the disease or experiencing devastating neurological sequelae. Some case reports have described partial responses to aggressive early immunotherapy, but evidence is limited to individual cases and small case series. The underlying cause remains unknown, though it is believed to involve an aberrant and severe autoimmune attack against CNS myelin.

Common questions about Marburg acute multiple sclerosis