Juvenile primary Sjögren's syndrome; a rare case report

Primary Sjögren"s syndrome is a rare collagen vascular disorder in children. It can be primary or secondary to other connective tissue disorders mostly systemic lupus erythematous. Keratoconjunctivitis sicca, xerostomia and inflammatory rheumatoid arthritis or collagenosis can be seen in this syndrome. Neurologic involvement such as dysarthria, extrapyramidal signs, aphasia, hemiparesis, sensory symptoms, brainstem and cerebellar involvement (some of which are similar to those seen in systemic lupus erythematous) has also been reported specially in primary form. In this study a 9-year-old girl with the diagnosis of primary Sjögren"s syndrome with neurologic manifestations including decreased level of consciousness, restlessness, aphasia, ataxia, headache and paralysis is presented. Other symptoms such as dry mouth and parotiditis had also existed although they were less prominent in our patient. It is notable that Sjögren"s syndrome does not always manifest typically as dry mouth or eyes and may present with less common symptoms including neurologic ones.