Progressive leptomeningeal fibrosis: a clinico-pathological case report

A female patient developed persistent facial pain beginning at age 19 years. Intermittent motor and sensory disturbances referable to one hemisphere began nine years later and by the age of 41 she had developed signs of increased intracranial pressure. Exploratory craniotomy revealed replacement of the leptomeninges by thick, fibrous tissue. The histological appearance was that of a chronic, benign and minimally infiltrative process with a mild, non-specific inflammatory component, underlying cortical ischaemic changes, and white matter oedema. The lesion resembled nodular fasciitis, a soft tissue process. No cause of the reactive fibrosis of the meninges in this case is known.