A previously healthy 35 year old woman presented with bilateral uveitus ass
ociated with multiple, evolving, non-enhancing white matter lesions consist
ent with a progressive leukoencephalopathy such as multiple sclerosis. Thir
ty months after her initial presentation, she was diagnosed with primary CN
S lymphoma and died 14 months later. The unusual clinical course preceding
the diagnosis suggests that a demyelinating disease may have preceded, and
possibly heralded, the development of primary CNS lymphoma. Cases of "senti
nel lesions" heralding the diagnosis of primary CNS lymphoma have been repo
rted, and this case further corroborates such instances and raises further
issues regarding possible neoplastic transformation occurring in inflammato
ry diseases such as multiple sclerosis.