Tumefactive demyelination

Case contributed by Vivien Jones
Diagnosis probable

Presentation

Patient presented to the emergency room with a one week history of left lower limb weakness with gait difficulty and recurrent tripping. Patient also notes a few day history of left hand weakness.

Patient Data

Age: 25 years
Gender: Female
mri

There is a 1.5 x 1.2 x 1.1 cm ring enhancing mass in the white matter of the right posterior frontal lobe. There is ring enhancement and mild edema with no significant mass effect on surrounding structures.

Case Discussion

The MRI brain findings, along with the clinical presentation, raised suspicion for a primary neoplasm. She had no prior history of malignancy and a CT thorax abdomen pelvis revealed no lesions. A brain biopsy and lumbar puncture were performed (results below).

Pathology results

  • brain biopsy: hypercellular brain parenchyma with abundant macrophages, dense perivascular inflammatory infiltrates, and reactive astrocytes

  • lumbar puncture: myelin basic protein elevated, zero oligoclonal bands

Differential

  • CNS lymphoma

  • tumefactive multiple sclerosis

  • stroke

Plan

  • repeat MRIs to evaluate for dissemination in time and dissemination in space

  • serial lumbar punctures to rule out CNS lymphoma

Of note, she received IV dexamethasone for 1.5 weeks before the brain biopsy, which could have affected the biopsy results.

Key learning points

  1. although pathology supports a demyelinating process, the absence of oligoclonal bands and other white matter lesions does not match this conclusion

  2. the brain biopsy showed no signs of CNS lymphoma but this must be reviewed knowing that the patient had already received 1.5 weeks of IV dexamethasone

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