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Marburg's variant multiple sclerosis

Case contributed by: Dr Praveen Jha

Presentation:

A 50 year old male presented with sequential weakness progressing from hemiparesis to quadriparesis. Patient could not talk properly initially and later, he stopped obeying commands. His tendon jerks were exaggerated. He doesn't have any history of fever and his CSF study was normal.

Patient Data:

Age: 50 years
Gender: Male
Modality: MRI

A large left frontal lobe mass is present with incomplete peripheral enhancement. 

Case Discussion:

Initial MRI revealed multiple 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.

MRI was done subsequently (shown above), which 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's variant) was considered.

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