PMID: 19928688Nov 26, 2009Paper

A 25-year-old woman with primary Sjögren syndrome who developed optic neuritis and encephalomyelitis associated with an anti-aquaporin 4 antibody

Rinshō shinkeigaku = Clinical neurology
Hirotaka KatoMitsuru Kawamura

Abstract

We report a 25-year-old woman who developed optic neuritis and encephalomyelitis following primary Sjögren's syndrome (SjS). SjS began with Sicca syndrome when she was 8 years old, and neurological involvement subsequently developed at the age of 10 with right hemiparesis. Based on clinical symptoms, serum positive for SS-A and SS-B antibodies and pathological findings of the salivary gland, we confirmed a diagnosis of primary SjS. Magnetic resonance imaging (MRI) revealed multiple lesions in the brain and the spinal cord. These led diagnosis of SjS with central nervous system involvement (CNS-SjS) and initiated steroid therapy. At the age of 25, the patient developed left visual loss due to retrobulbar optic neuritis, left lower quadrantic hemianopia, numbness of the right upper limb, and weakness of both legs. Laboratory examinations showed that her serum was positive for SS-A and SS-B antibodies, and her cerebrospinal fluid had elevated levels of total protein and myelin basic protein without pleocytosis. Her brain MRI revealed multiple T2-high-intensity lesions bilaterally in the frontal subcortical white matter and in the right temporo-parietal subcortical white matter. The lesions included a tumefactive lesion and an acti...Continue Reading

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Citations

Apr 28, 2011·Arthritis Care & Research·Jason R KolfenbachSterling G West
Aug 28, 2014·Case Reports in Neurological Medicine·Michele ColaciClodoveo Ferri

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