The primary Sjogren-syndrome (PSS) is a chronic autoimmune disorder with a prevalence of 0.2% and 2% that is manifestated clinically by xerophthalmia with reduced lacrimation (pathological Schirmer's test), xerostomia with reduced salivary flow (pathological salivary scintigraphy), antibodies to Ro/SSA and La/SSB, labial salivary gland biopsy with mononuclear infiltrations. In a considerable percentage, PSS is associated with neurological complications which may affect the peripheral nervous system (sensory polyneuropathy, autonomic polyneuropathy, cranial nerve neuropathy) and to a lesser extent the central nervous system (acute myelitis, progressive myelopathy, optic neuritis, ischemic infarction, psychiatric disturbances). Since neurological findings may dominate the clinical picture, PSS should be considered frequently as differential diagnosis.