Tumefactive demyelination

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  • An inflammatory demyelinating
  • Presents as a solitary large (> 2 cm) focus of demyelination within a cerebral hemisphere with associated edema that may simulate neoplasm or abscess.
  • Presentation is acute (≤ 3 weeks) with headache, seizures, and focal neurologic deficits.
    • Often it may be a monophasic episode of disease without recurrence,
    • Some may evolve into relapsing-remitting MS.
  • Imaging features in 50% have contrast enhancement in the form of an incomplete ring, without enhancement at junctions with gray matter (or basal ganglia depending on orientation), and there is usually minimal mass effect.
      notion image
  • Advanced MR imaging techniques may be useful, and the rCBV values are significantly lower than for high-grade glial neoplasms.
  • Management
    • High-dose corticosteroid therapy.
      • Radiation or surgical excision of lesions misdiagnosed as tumour will cause additional irreversible neurologic deficits.
  • Differential diagnosis
    • Ring-enhancing lesions
      • Neoplasia
      • Abscess
      • MS
      Differentiating
      MRI
      MRS
      MR perfusion
      Tumefactive demyelination
      - 50% show enhancement, usually an open ring with in complete portion facing grey matter
      - Mildy increased diffusion (unlike abscess)
      - Elevation glutamate/glutamine peaks
      - Reduced NAA
      - Inc cho, lipis, lactate
      - No elevation in rCBV
      High grade glioma
      - Peripheral, heterogenous enhancement with nodules and necrosis
      - Can be ring enhancing
      Solid parts diffusion restriction
      - Reduce NAA, Myoinositol
      - Inc. Lipid, choline and lactate
      - Marked elevation rCBV
      Primary CNS lymphoma
      - Homogenous enhancement common
      - Ring enhancing in HIV/immunocompromise
      - Restricted diffusion (lower ADC then metastasis or HGG)
      - Large Choline peak
      - Reversed Cho/Cr Ratio
      - Markedly reduced NAA
      - Lactate peak possible
      - Modest elevation rCBV