Autoimmunity can manifest clinically in many ways; however, despite the
various efforts to classify autoimmune disorders into specific disease
entities, the borders between these disorders remain, in many cases,
unclear.
In this report we describe a young woman with subclinical Sjogren’s
syndrome and biliary cirrhosis, who presents clinically with symptoms
exclusively from the central nervous system. This neurological syndrome
is consistent with a progressive myelopathy. Although the patient has a
serologically and histologically confirmed multisystemic autoimmune
disorder, she fulfills none of the classification criteria for the
diagnosis of a specific connective tissue disease
