PROGRESSIVE systemic sclerosis (PSS) or systemic scleroderma is a disease characterized pathologically by alterations in collagen tissue and clinically by diffuse induration of the skin. In the majority of cases there is evidence of dysfunction of visceral structures, particularly the gastrointestinal tract, kidneys, heart, and lungs. Vasomotor and endocrine disturbances may also be present.
Unlike other collagen diseases, such as systemic lupus erythematosus,1 thrombotic thrombocytopenic purpura,2 and periarteritis nodosa,3 the nervous system is rarely involved in PSS. Little has been written in the large literature on PSS of the neurologic manifestations which do occur in this disease. The purpose of this communication is to review and discuss the neurologic problems occurring in systemic scleroderma as determined from the charts of patients with PSS at the Mount Sinai Hospital, New York.
Materials and Methods
Between 1935 and 1966, 155 patients with the diagnosis of scleroderma were admitted