Until recently, systemic sclerosis (SSc) was thought to spare the central nervous system (CNS). Neurological symptoms secondary to CNS involvement are very rare in SSc patients. Conversely, the prevalence of depression in SSc patients ranges from 17 to 65% and is much higher than that observed in the general population. Cognitive impairment has been reported in SSc patients, but these findings require confirmation in further studies. Brain calcifications and hyperintense white matter signals (leukoaraiosis) have been documented in SSc patients at a higher incidence than in control populations. Severe leukoaraiosis lesions seem to be associated with severe vascular manifestations in SSc. If morphological CNS abnormalities could be linked to neuropsychiatric manifestations, it would be possible to identify neuropsychiatric scleroderma as we can now identify neuropsychiatric systemic lupus erythematosus.
Copyright 2009. Published by Elsevier Masson SAS.