Lymhomatosis cerebri--a rare cause of leukoencephalopathy

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).

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.

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.