Inflammatory demyelinating pseudotumor

Case contributed by Stephen Stuckey
Diagnosis probable

Presentation

Right facial droop, right arm and leg weakness.

Patient Data

Age: 45 years
Gender: Male

Large area of low attenuation involves the left frontal lobe with mass effect. 

Mass-like T2 signal change in the left frontal lobe, extending into the corpus callosum and right frontal lobe. Poorly defined, incomplete peripheral enhancement. A few associated punctate foci of diffusion restriction and susceptibility. 

Case Discussion

Inflammatory Demyelinating Pseudotumor

As the name suggests is a demyelinating process, typically acute and fulminant with mass effect. The condition mimics tumor on imaging and pathology. 

The key to diagnosis is the enhancement pattern - peripheral, incomplete, and often poorly defined or 'wispy'.

Pathological features include:

  • Well defined borders
  • Uniform distribution of foamy macrophages
  • No coagulative necrosis
  • Sheets of gemistocytic astrocytes with well formed processes
  • Perivascular chronic inflammatory cell infiltration
  • Absence of myelin with preservation of axons

 

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