A Case of Fulminant Recurrent Tumefactive Demyelinating Lesions

Background: Tumefactive demyelinating lesions in the central nervous system present diagnostic challenges. Assumed to be a mass, they are often biopsied, and histopathology shows evidence of demyelination. These lesions usually follow a monophasic course, and if the patient goes on to develop multiple sclerosis, the lesions that follow are more typical in appearance for the demyelinating lesions seen in MS. Here we present a case report of a patient with recurrent CNS tumefactive demyelinating lesions that have followed a relatively fulminant course which has resulted in the rapid onset of disability. These cases are uncommonly reported in the literature.

Case Report: A 46 year old African American male presented to an affiliated neurosurgery clinic after the onset of impaired spatial orientation, bumping into objects, and fatigue. He was found to have a large right parietal lesion which was concerning initially for a glioma. Biopsy was confirmatory of demyelination. Following the biopsy, the patient was readmitted to the hospital for continued worsening despite intravenous steroids, and given plasma exchange. He improved to baseline and resumed work several months later. He remained stable for 18 months following, with the interval development of complex partial seizures that were well controlled with antiepileptic drugs. He subsequently developed a second large lesion in the left temporal lobe. The patient was treated with plasma exchange and steroids again with good recovery and subsequently started on dimethyl fumarate. He developed a third tumefactive lesion in the right frontal lobe several months later with cognitive changes, requiring hospital admission. He then rapidly developed fourth and fifth lesions in the brainstem and thalamus, and he was started on treatment with cyclophosphamide. He has had a rapid downward progression in his clinical status over the last 3-4 months, with severe cognitive deficits.

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Conclusions: Here we report a case of tumefactive CNS demyelination that has followed a fulminant course, resistant to disease modifying therapy and requiring aggressive immunosuppression. These cases are relatively rare, and there is little data to guide the treatment for episodes of recurrent tumefactive CNS demyelination. Further research is needed.