Background Central nervous system involvements are the most severe clinical manifestations in connective tissue diseases.

Objectives We estimated prevalence of central nervous system (CNS) involvement in our patients.

Methods They were followed up during ten years period.

Results Among 250 patients with systemic lupus erythematosus (SLE), 17 (7%) had alterations of CNS: cerebrovascular insult (5 pts), seizures (4 pts), psychosis (3 pts), transversal myelitis (2 pts), cranial nerve paralysis (2 pts) and psychoorganic syndrome (1 pt). In 3 pts SLE expressed primarily with CNS manifestations. Thirteen patients of 64 (20%) with Behcet disease had impairment of CNS: aseptic meningoencephalitis (2 pts), pyramid syndrome (2 pts), cerebelar-ataxic syndrome (2 pts), myelopathy (2 pts), cranial nerve paralysis (2 pts), psychosis (1 pt), cerebrovascular insult (1 pt) and idiopathic intracranial hypertension (1 pt). Only two patients of 50 with primary Sjogren syndrome expressed CNS manifestations as cranial nerve paralysis or disseminated demyelinisation disease. We also analysed our 20 patients with primary antiphospholipid syndrome, that is not connective tissue disease, but its immunological-mediated pathogenesis may affects functions of CNS. Among them two patients had multiinfarct dementia.

Conclusion We concluded that CNS involvement is not frequent manifestation in our patients with connective tissue diseases.