Primary leptomeningeal lymphomas are rare, and usually fellow a rapid
clinical course with early systemic involvement. A 60-year-old woman p
resented with a 3-year history of worsening seizures and memory loss.
Neuroimaging showed widespread meningeal calcification. After extensiv
e investigations a meningeal biopsy revealed a low-grade B-cell lympho
ma categorized as an extranodal marginal zone B-cell lymphoma, attribu
ted to the same histological group as the MALT (mucosa-associated lymp
hoid tissue) lymphomas described in the stomach, thyroid, salivary gla
nds and orbit. There was no evidence of systemic involvement. The exte
nsive meningeal calcification would appear to be a novel finding in pr
imary leptomeningeal lymphoma whereas the unusually long clinical hist
ory in this case is possibly related to the particular histological ty
pe of low-grade B-cell lymphoma.