[Primary Sjogren syndrome with involvement of the nervous system. Description of 4 cases]

Four patients, 2 males and 2 females, with primary Sjögren's syndrome (PSS) and neurologic involvement are described. The first patient presented a progressive axonal polyneuropathy due to endo-epineural vasculitis. The second patient developed an acute and focal lymphocytic meningoencephalitis with increased IgG synthesis and excellent response to corticosteroids. The remaining 2 patients had previously been diagnosed as having definite multiple sclerosis (MS), but after a standardized study both fulfilled PSS diagnostic criteria. These cases illustrate the polymorphism of the neurological manifestations in PSS. Pathogenetic relationships between PSS and MS are briefly reviewed.