Marburg variant multiple sclerosis

Case contributed by Dr Praveen Jha

Presentation

Sequential weakness progressing from hemiparesis to quadriparesis. Patient could not talk properly initially and later, he stopped obeying commands. He was hyperreflexic. No history of fever and CSF was normal.

Patient Data

Age: 50 years
Gender: Male

A large left frontal lobe mass is present with restricted diffusion and incomplete peripheral enhancement (C-shaped/arc-like enhancement).

Periventricular hyperintense areas and perpendicular extensions from corpus callosum radiating towards cortex (Dawson's fingers) could be observed.

Multiple hyperintense areas in periventricular regions, centrum semiovale and brainstem.

Note: This case has been tagged as "legacy" as it no longer meets image preparation and/or other case publication guidelines.

Case Discussion

Initial MRI (not shown) revealed multiple T2 hyperintensities in brainstem, periventricular regions and centrum semiovale, which also showed restricted diffusion. A provisional diagnosis of acute disseminated encephalomyelitis vs multiple infarcts was made. Patient did not improve on treatment for two months.

Repeat MRI (above) revealed resolution of brainstem lesions, with appearance of a large mass-like lesion in left frontal lobe, multiple hyperintensities in brainstem, centrum semiovale and periventricular regions. A high index of suspicion was kept for possibility of tumefactive demyelinating lesion.

Lesions were typically hypointense on T1W images, hyperintense on T2W images. On T2 sagittal images, hyperintensities radiating from corpus callosum to the cortex were seen. Post-contrast image showed C-shaped/arc-like enhancement in the periphery of mass.

Though CSF oligoclonal bands were absent, a diagnosis of multiple sclerosis (Marburg variant) was considered.

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