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Tumefactive Demyelination

Last Updated: October 1, 2018

Open Table of Contents: Tumefactive Demyelination

Figure 1: T1 hypointense (top row left) oval, subcortical lesion with corresponding FLAIR hyperintense signal (top row right) and faint rim of incomplete (horse-shoe) enhancement (bottom row left). Notice the mildly increased cerebral blood volume on MR perfusion imaging (bottom row right) along the medial margin. The incomplete rim of enhancement and low T1 signal is fairly classic for a tumefactive, demyelinating lesion. While perfusion can be mildly increased along the periphery of the lesion, it is often much less than would be expected with a high grade glioma.

Figure 2: Tumefactive demyelinating lesions are often large and mass-like, demonstrating FLAIR hyperintensity throughout the lesion (top row left – sagittal, top row right, bottom row left - axial). These lesions commonly involve the corpus callosum (top row left, top row right - arrow) and are often confused for lymphoma or glioblastoma. As is common for lesions of demyelination, there is vague incomplete peripheral enhancement on post-contrast imaging (bottom row right).

Description

  • Probable autoimmune-mediated demyelination in which environmental factors affect genetically susceptible individuals

Pathology

  • Activated T-cells attack myelinated axons

Clinical Features

  • Symptoms
    • Variable; often initial impaired/double vision of acute optic neuritis
    • Weakness, numbness, tingling, gait disturbances, multiple cranial nerve palsies
    • Spinal cord symptoms in most patients
  • Age of onset
    • Peak: 20-40
  • Gender
    • Females twice as likely to be affected than men

Imaging

  • General
    • Callososeptal T2 hyperintensities radiating perpendicularly
    • Can have involvement throughout the CNS
    • Tumefactive MS
      • Locally aggressive form of demyelination manifesting as one or many lesions without significant mass effect or surrounding edema
    • Revised McDonald criteria
      • Dissemination in time
        • Either new T2 or enhancing lesions on followup MR
        • Or simultaneous presence of asymptomatic enhancing and nonenhancing lesions at any time
      • Dissemination in space
        • In at least 2 of the following 4 areas
          • Periventricular, juxtacortical, infratentorial, spinal cord
  • CT
    • Iso to slightly hypodense with variable degrees of (usually peripheral) enhancement if active
  • MR
    • T1
      • Hypo to isointense
      • Markedly hypointense lesions in the chronic stage
    • T2/FLAIR
      • Hyperintense, linear foci radiating from ventricles
    • DWI
      • Uncommonly, restriction associated with acute plaques
    • Contrast
      • Peripheral or small nodular enhancement suggests active demyelination
      • Incomplete rim of enhancement with tumefactive lesions
    • MRS
      • Decreased NAA and increased choline, sometimes overlapping with findings of neoplasm
    • Perfusion MR
      • Mean CBV within tumefactive lesions said to be less than in high-grade gliomas and lymphomas
  • Imaging Recommendations
    • MR with contrast. Include sagittal FLAIR and a fat saturated coronal sequence to assess for optic neuritis
    • Spectroscopy and Perfusion imaging may be helpful
  • Mimic
    • Most commonly mimics Glioblastoma and Lymphoma, particularly when it involves the corpus callosum. Carefully inspect the enhancement pattern as MS often has a smooth, incomplete ring. If the history of the patient or pattern of enhancement doesn’t help, can consider perfusion imaging which should be normal or decreased.

For more information, please see the corresponding chapter in Radiopaedia.

Contributor: Sean Dodson, MD

DOI: https://doi.org/10.18791/nsatlas.v1.03.02.28

References

Cha S, et al. Dynamic contrast-enhanced T2*-weighted MR imaging of tumefactive demyelinating lesions. AJNR. 2001; 22(6):1109-16.

Checrallah A, et al. Multiple sclerosis mimicking brain tumor: an unusual presentation. J Med Liban. 2005; 53(1):45-9.

Huisman T. Tumor-like lesions of the brain. Cancer Imaging. 2009; 9:S10-S13.

Iwamoto K, et al. Late-onset multiple sclerosis mimicking brain tumor: a case report. Brain Tumor Pathol. 2004; 21(2)83-6.

Kim DS, et al. Distinguishing Tumefactive Demyelinating Lesions from Glioma or Central Nervous System Lymphoma: Added Value of Unenhanced CT Compared with Conventional Contrast-enhanced MR imaging. Radiology. 2009; 251(2):467-75.

Saindane AM, et al. Proton MR Spectroscopy of Tumefactive Demyelinating Lesions. AJNR. 2002; 23:1378-86.

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