Neurological involvement in systemic sclerosis. Evidence of a central Raynaud’s phenomenon

Abstract

Objective: To evaluate the neurological involvement in systemic sclerosis and to identify changes of cerebral blood flow after a cold stressor test. Methods: We investigated 16 patients with systemic sclerosis through the SPECT analysis of regional cerebral blood flow to find out whether focal or diffuse perfusion defects might be present and wether new or increased defects might occur after a cold hand test. MRI imaging was obtained to asses the presence of cerebral ischemic lesions. Results: Cerebral perfusion defects on SPECT were found in 7 patients (43.5%). After the cold test, perfusion defects appeared in 1 patient and the perfusion defect detected at baseline worsened in 3 patients. MRI showed cerebral ischemic lesions in 6 patients (37.5%) and cortical atrophy in 10 patients. Conclusions: We observed the appearance of vasospastic features in the central nervous system (CNS) after a cold stressor test in scleroderma patients. A Raynaud’s syndrome may occur in the CNS. Perfusion changes of cerebral blood flow might be due to a reduced vascular reserve or to a vascular-endothelial damage.