Acute malignant Marburg type multiple sclerosis

Case contributed by Dr Mohammad A. ElBeialy

Presentation

Severe progressive headache in the occiptal region with gait disturbance since three weeks.

Patient Data

Age: 25 years
Gender: Female

A right medial cerebellar hemisphere 2.3 X 2.2 X 2.1 cm mass lesion is seen, the lesion shows heterogeneously hypointense T1 and hyperintense T2 signal intensity with heterogeneous predominantly marginal and nodular enhancement with central necrosis. The lesion is surrounded by mild to moderate vasogenic brain oedema with some compression of the 4th ventricle and slight downward displacement of the cerebellar tonsils. 

At left splenium of the corpus callosum small enhancing lesion is seen with slight hyperintense T1 and hyperintense T2 / FLAIR signal intensity. It measures 1.4 X 0.8 cm. 

A small left cerebellar and adjacent middle cerebellar peduncle demyelinating focus of high T2 / FLAIR signal intensity and faint post-contrast enhancement is seen.

A small right posterior parietal periventricular demyelinating focus of low T1 / FLAIR and high T2 signal is seen with no post-contrast enhancement. 

 

MR spectroscopic examination of the right cerebellar lesion shows:

  • Elevated choline (Cho) as well as myoinositol (MI) suggesting demyelination. 
  • Decreased N-acetyl aspartate (NAA) denoting axonal loss and dysfunction with increased Cho / NAA and Cho / Creatine ratios. 
  • Elevated lipid / lactate peak is noted.
  • MR spectroscopic examination of the perifocal oedema shows normal metabolites’ ratio and peaks.

Case Discussion

The above described constellation of findings are those brain demyelinating process with the right cerebellar hemisphere mass lesion conventional and spectroscopic criteria are very suggestive of tumefactive variant of multiple sclerosis (Marburg’s variant of MS).

Marburg multiple sclerosis is a sub-type of MS with only one lesion may be present. This lesion tends to be very large, and it can resemble a brain tumor or abscess. The associated brain demyelinating foci particularly those periventricular and corpus callosal lesions as well as the MRS findings in a young age female are very suggestive of the diagnosis of Marburg's variant of MS. 

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Case Information

rID: 22851
Case created: 28th Apr 2013
Last edited: 13th Mar 2017
Inclusion in quiz mode: Included

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