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Dysexecutive Syndrome: A Specific Pattern of Cognitive Impairment in Systemic Sclerosis

Yilmaz, Neslihan MD*; Mollahasanoglu, Aynur PhD; Gurvit, Hakan MD; Can, Meryem MD*; Tuncer, Nese MD; İnanc, Nevsun MD*; Yavuz, Sule MD*

Cognitive and Behavioral Neurology: June 2012 - Volume 25 - Issue 2 - p 57–62
doi: 10.1097/WNN.0b013e3182593c75
Original Studies

Background and Purpose: Systemic sclerosis (SSc) is a connective-tissue disorder characterized by microvascular damage and tissue fibrosis. Although overt nervous system involvement is unusual in SSc, imaging studies have shown cerebral hypoperfusion. We evaluated cognitive functions in patients with SSc who had no history of neurological involvement, to seek cognitive impairment caused by the suggested cerebral hypoperfusion.

Methods: We performed a comprehensive neuropsychological test battery on 31 patients with SSc and on 2 groups of age-adjusted, sex-adjusted, and education-adjusted controls: 15 patients with rheumatoid arthritis and 20 healthy volunteers.

Results: The patients with SSc scored significantly worse on most of the measures of executive function than the 2 control groups (P<0.05). However, both patient groups did worse than the healthy controls on measures of attention and memory (P<0.005).

Conclusions: Our results suggest that patients with SSc have a specific pattern of cognitive impairment: the dysexecutive syndrome. Attentional and memory problems, however, may arise from other confounders such as disease duration and chronic medication use. SSc may be a rare cause of vascular cognitive impairment.

Departments of *Rheumatology

Neurology, Faculty of Medicine, Marmara University, Selamicesme/Istanbul

Department of Neurology, Behavioral Neurology and Movement Disorders Unit, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey

S.Y., H.G., N.Y., and N.T. conceived of and designed the study. N.Y., A.M., M.C., and N.I. collected the data. S.Y., N.Y., and H.G. analyzed and interpreted the data.

The authors declare no conflicts of interest.

Reprints: Sule Yavuz, MD, Department of Rheumatology, Faculty of Medicine, University of Marmara, Bagdat Cad. Trak Ap, No:149/3, Selamicesme/Istanbul, Turkey 34750 (e-mail:

Received August 13, 2011

Accepted January 18, 2012

© 2012 Lippincott Williams & Wilkins, Inc.