Systemic sclerosis with central nervous system involvement: brain abnormalities revealed by magnetic resonance imaging

Background : Systemic sclerosis (SSc) is a progressive multisystem connective tissue disease characterised by obliterative microvascular abnormalities and visceral fibrosis. Primary involvement of the brain is thought to be uncommon. The aim of the study was to investigate the prevalence of the brain abnormalities in SSc patients by means of neurological examination and magnetic resonance imaging (MRI) and to correlate MRI images with neurological manifestations.

Material and methods : 21 female patients with SSc aged 23–71, with a disease duration of 1–11 years with neuropsychiatric symptoms and without other relevant systemic diseases were enrolled. All patients underwent brain MRI examinations.

Results : The most common findings in MRI were symptoms of cortical and subcortical atrophy (in 57% of all patients) and focal lesions (62% of patients) predominantly in white matter and < 2 mm in diameter. Among patients with depression, cortical and subcortical atrophy of moderate or significant degree was found in 8 individuals (66.7%), single focal lesions in 6 individuals (50%) and multiple focal lesions in 3 (25%).

Conclusions : These data suggest that brain involvement in patients with systemic sclerosis is not uncommon and magnetic resonance imaging is considered to be a sensitive tool for detection of the central nervous system involvement in patients with SSc.