Tumefactive multiple sclerosis

Case contributed by Yahya Baba
Diagnosis almost certain

Presentation

Presents with confusion and memory disorders for a week.

Patient Data

Age: 70 years
Gender: Male

Day 01

ct

Mild hazy hypodensity of the splenium of the corpus callosum.

The lesion was not initially seen, and the CT report was normal, so the patient went back home.

The symptoms persisted and the patient presents a month later with the onset of gait disturbances.

Day 30

mri

There is a hyperintense T2 lesion involving the splenium of the corpus callosum and the subcortical white matter around the occipital horns of the lateral ventricles. This lesion demonstrates enhancement after Gadolinium injection.

The differential diagnosis discussed for this lesion was butterfly glioma or primary CNS lymphoma.

The decision was to refer the patient for a stereotactic brain biopsy and corticosteroid treatment to reduce cerebral edema.

Day 65

mri

This MRI was done after the biopsy and the steroid treatment:

  • marked decrease of the hyperintense lesion involving the corpus callosum

  • marked decrease in the cerebral edema

  • biopsy sequelae

The pathological report was non-conclusive, and re-biopsy was advised.

The second biopsy was non-conclusive as well.

The short corticosteroid therapy (two months) led to a total regression of symptoms without clinical or radiological relapse so far.

5 years later

mri

Imaging follow-up of an undetermined corpus callosum lesion:

  • atrophy of the splenium of the corpus callosum

  • there are no new lesions or increase of the FLAIR hyperintensities around the occipital horns of the lateral ventricles

The patient presents with ataxia of the right lower limb, MR to rule out medullary lesions.

5 Years + 4 months

mri
  • unique anterior intramedullary lesion at the level of T6-T7-T8

  • this lesion is T2 hyperintense, and T1 isointense with moderate enhancement after Gadolinium injection

  • no blood product on gradient echo images

  • no syrinx

8 months later (6 years from the initial episode), he presents with left brachiofacial hemiparesis and minor hemisphere major syndrome.

MRI to rule out a relapse of his initial lesion.

6 years

mri
  • onset of a new subcortical right-sided frontoparietal lesion demonstrating irregular concentric areas alternating iso/hypointense and hyperintense signal, surrounded with vasogenic edema, measuring 27 x 35 mm on axial images

  • peripheral ring enhancement after gadolinium injection and no restricted diffusion

  • there is no neoangiogenesis on perfusion sequences

6 years + 1 month

mri
  • increased size of the right frontoparietal lesion measuring 77 x 46 mm in axial sequences

  • increased mass effect with no significant midline shift

  • persistence of the peripheral enhancement

6 years + 1 month

ct
  • increased size of the right frontoparietal lesion and of the associated vasogenic edema

  • increased mass effect with no significant midline shift

The patient responded favorably to a SOLUMEDROL bolus initially but relapsed shortly after.

This new lesion was also biopsied and was negative, it did not demonstrate lymphomatous cells. A lumbar puncture did not reveal intrathecal IgG synthesis.

A treatment with Rituximab was started along with corticosteroid therapy.

6 years + 9 months

mri

Complete regression of the right fronto-parietal lesion and onset of cortical and subcortical white matter artophy.

Biopsy sequelae in the right parietal region with a hematoma in the biopsied lesion.

Case Discussion

Features of tumefactive multiple sclerosis, with no definite pathological proof, in fact, several biopsies were performed but were non-conclusive.

The diagnosis was made based on the evolution with only corticosteroid treatment, the associated medullary demyelinating lesion, and the absence of aggressiveness during 6 years of evolution.

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