Progressive systemic sclerosis and nervous system involvement. A review of 14 cases

Nervous system involvement in progressive systemic sclerosis (PSS) has been considered rare compared to other collagen diseases. We present 14 additional cases of PSS with neurological manifestations. Primary involvement of the peripheral nerves could be detected in 4 of 14 patients and is documented by electromyo- and electroneurographical examinations. Central nervous system (CNS) manifestations directly related to PSS are a rarity, which may reflect the lack of collagen in the brain, histological differences between cerebral and other arteries and the immunological particularity of the brain. There may have been a direct relationship between CNS involvement and PSS in only one patient presenting with an overlap-syndrome.