A rare case of cerebellar degeneration due to primary Sjogren's syndrome.

Sjogren's syndrome (SS) is an autoimmune pathology which mainly affects salivary and lacrimal glands. Cerebellar degeneration association with SS is very rare, with only a few cases described. The treatment of SS with central nervous system involvement is not consensual. We present a 48-year-old woman with dysarthria, diplopia and ataxia associated with xerostomia. The brain magnetic imaging revealed bilateral cerebellar atrophy. She had antibody positivity for anti-SSA and anti-SSB and minor salivary glands biopsy revealed lymphocytic infiltration. Methylprednisolone, cyclophosphamide and intravenous immunoglobulin yielded no results. Rituximab was initiated with improvement in dysarthria and coordination.