Lymhomatosis cerebri--a rare cause of leukoencephalopathy

Abstract

Background: Lymphomatosis cerebri is a rare variant of primary central nervous system lymphoma. Clinically it presents usually as rapidly progressive dementia and is accompanied by extensive white matter changes in magnetic resonance imaging (MRI).

Case description: A 49-year-old immuno-competent male who complained of dull diffuse headache, rapidly deteriorated in higher functions over a 6 month period. On examination he was globally declined in cognitive functions and had papilledema. MRI showed extensive white matter lesions. He became temporarily alert, and attentive after a course of parenteral steroids. However within three weeks he relapsed and a wedge biopsy of the brain revealed histopathology consistent with lymphomatosis cerebri.

Conclusion: The differential diagnosis of diffuse white matter diseases is constantly expanding. In the background of a rapidly progressing subcortical dementia and extensive white matter disease, neoplastic disorders of the brain especially lymphomatosis cerebri should be considered. Early tissue diagnosis is important for specific treatment and interventions.