Tumefactive multiple sclerosis

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By Reem F Bunyan MD and Claudia F Lucchinetti MD

Tumefactive multiple sclerosis is characterized by one or more large, swollen brain lesions that can mimic brain tumors. Tumefactive multiple sclerosis is also known as or subsumes Pseudotumoral multiple sclerosis. -ed.

Multiple sclerosis presenting with clinical and radiographic features similar to a brain tumor is referred to as tumefactive multiple sclerosis. This often poses a diagnostic challenge for the neurologist, neurosurgeon, radiologist, and pathologist. In this clinical article, Drs. Reem Bunyan and Claudia Lucchinetti of the Mayo Clinic Department of Neurology discuss the spectrum of central nervous system inflammatory demyelinating disease that can have a tumefactive clinical or radiographic presentation, including the Marburg variant of multiple sclerosis, Balo concentric sclerosis, acute disseminated encephalomyelitis, and neuromyelitis optica.

Key points

  • A spectrum of disorders causes inflammatory demyelination of the central nervous system; the disorders are collectively referred to as CNS idiopathic inflammatory demyelinating diseases.
  • Any type of CNS idiopathic inflammatory demyelinating disease may present clinically and radiographically as a tumefactive lesion.
  • Multiple sclerosis is the most common CNS idiopathic inflammatory demyelinating disease.
  • Pathological features of multiple sclerosis lesions include focal demyelination, variable inflammation, gliosis, and relative axonal preservation.
  • Two thirds of patients who present with tumefactive demyelinating lesions subsequently develop multiple sclerosis, with a relapsing-remitting disease course.
  • The interval between initial tumefactive presentation and subsequent multiple sclerosis-defining clinical exacerbation is longer than for prototypic multiple sclerosis (approximately 5 years).
  • MRI features associated with tumefactive demyelination include multifocal lesions with at least a single dominant lesion larger than 2.0 cm, variable presence of mass effect or edema, and ring enhancement.
  • High-dose intravenous corticosteroids are the first line management for tumefactive relapses.
  • Aggressive supportive management in the acute phase is crucial because the predicted long-term outcome of many patients is good.