Central nervous system Sjogren's syndrome in a child: Case report and review of the literature

We describe a case of pediatric Sjogren's syndrome with progressive neurolo gic involvement. At age 4 years, she had been diagnosed with Melkersson-Ros enthal syndrome. After being stable with facial diplegia and swelling for 5 years, she acutely presented with diplopia, vertigo, and ataxia. Cranial m agnetic resonance imaging (MRI) showed a left dorsal midbrain lesion. Serol ogic and histopathologic findings confirmed primary Sjogren's syndrome. She responded well to intravenous methylprednisolone, with subsequent clinical improvement and MRI resolution. This report reviews the pediatric literatu re and underscores the importance of considering Sjogren's syndrome in a ch ild with unexplained facial weakness and in the differential diagnosis of p ediatric stroke.