Opsoclonus as a dominant sign in primary Sjogren's syndrome

Primary Sjogren's syndrome (PSS) is often associated with nervous system ab normalities, although opsoclonus as the dominant sign has not been reported before. In a 69-year-old woman with acute-onset opsoclonus as the dominant sign, ataxia, dysarthria, vertigo, depression, anxiety, and limb jerking, PSS was diagnosed based on xerophthalmia, xerostomia, increased anti-nuclea r antibodies, a positive Schirmer's test, and a pathologic salivary gland s cintigraphy. Cerebral MRI revealed multiple, hyperintense basal ganglia and brainstem lesions. Cerebral HMPAO-SPECT showed unilateral cerebellar hypom etabolism. Muscle/nerve biopsy was compatible with axonal polyneuropathy. C orticosteroids improved the patient's condition considerably. In conclusion , opsoclonus may be the dominant sign in PSS and responds well to corticost eroids.