Dysexecutive Syndrome A Specific Pattern of Cognitive Impairment in Systemic Sclerosis PDF Print E-mail
Sunday, 07 July 2013 20:31
Systemic sclerosis (SSc), also called scleroderma, is a connective-tissue disorder characterized by obliterative microvascular lesions and diffuse interstitial fibrosis. SSc damages the small and medium-sized vessels serving the skin, joints (especially fingers and toes), and internal organs such as the lungs, gastrointestinal tract, kidneys, and heart. In most patients, neurological involvement is limited, and localized to the peripheral nervous system. Raynaud phenomenon (RP) is a prominent early vascular feature of SSc, affecting 90% of patients during their disease course and, in some, progressing
to severe ischemia.

Although the typical manifestation of neurological involvement in SSc is mononeuropathy or polyneuropathy, some imaging studies have reported cerebral hypoperfusion and acute cerebral vasculitis. Cognitive dysfunction is one of the important signs of neurological involvement in systemic lupus erythematosus, affecting up to 80% of patients. In most, the cognitive dysfunction is attributed to RP or antiphospholipid antibodies.

Although RP is a well-known feature of SSc, little is known about cognitive functioning in SSc. Limited data from small series suggest possible impairment. In our study, we aimed to evaluate cognitive functioning in patients with SSc who had no manifest sign of neurological involvement and to determine whether they showed a specific pattern of cognitive impairment that might be caused by cerebral hypoperfusion.

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