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Journal of Child Neurology
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Central Nervous System Sjögren's Syndrome in a Child: Case Report and Review of the Literature

Jay A. Gottfried, MD, PhD

Division of Neurology

Terri H. Finkel, MD, PhD

Division of Rheumatology

Jill V. Hunter, MBBS

Department of Radiology

David F. Carpentieri, MD

Department of Pathology

Richard S. Finkel, MD

Division of Neurology The Children's Hospital of Philadelphia The University of Pennsylvania Philadelphia, Pennsylvania

We describe a case of pediatric Sjögren's syndrome with progressive neurologic involvement. At age 4 years, she had been diagnosed with Melkersson-Rosenthal syndrome. After being stable with facial diplegia and swelling for 5 years, she acutely presented with diplopia, vertigo, and ataxia. Cranial magnetic resonance imaging (MRI) showed a left dorsal midbrain lesion. Serologic and histopathologic findings confirmed primary Sjögren's syndrome. She responded well to intravenous methylprednisolone, with subsequent clinical improvement and MRI resolution. This report reviews the pediatric literature and underscores the importance of considering Sjögren's syndrome in a child with unexplained facial weakness and in the differential diagnosis of pediatric stroke. (J Child Neurol 2001;16:683-685).

Journal of Child Neurology, Vol. 16, No. 9, 683-685 (2001)
DOI: 10.1177/088307380101600911
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