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Lt Col Christopher S. Williams, USAF, MC, SFS; Elizabeth Butler, MD; Gustavo C. Román, MD

Arch Neurol. 2001;58:815-819.

Background  Peripheral neuropathy is a common complication of primary Sjögren syndrome, but central nervous system involvement also occurs and may be the only extraglandular manifestation. Sicca symptoms may also be minimal. Combinations of lesions along with relapses and remissions can suggest multiple sclerosis in the proper clinical setting, making the correct diagnosis elusive.

Objectives  To report a case of progressive transverse myelopathy with previous optic neuropathy in primary central nervous system Sjögren syndrome (CNS-SS), and to review 17 previously reported cases and the patient's responses to various therapies.

Design  Case report and literature review.

Setting  University hospital.

Patient  A 63-year-old Hispanic woman with a 10-month history of progressive spastic paraparesis associated with optic neuropathy and a T10 sensory level. Magnetic resonance imaging demonstrated multifocal, contrast-enhancing lesions in the spinal cord. The patient was diagnosed as having CNS-SS because of the presence of sicca symptoms, abnormal serological test results, and salivary gland biopsy results, which fulfilled San Diego criteria for "definite" Sjögren syndrome. She responded to treatment with a combination of prednisone and cyclophosphamide.

Conclusions  Diagnosis of primary CNS-SS requires a high index of suspicion and specialized clinical testing. Treatment with pulse doses of corticosteroids alone may be suboptimal, but results of treatment with a combination of corticosteroids and either cyclophosphamide or chlorambucil have been encouraging.

From the Departments of Neurology, Wilford Hall Medical Center, Lackland Air Force Base, Tex (Dr Williams), and the University of Texas Health Sciences Center at San Antonio (Drs Butler and Román).

Corresponding author and reprints: Lt Col Christopher S. Williams, USAF, MC, SFS, Department of Neurology, 59 MDOS/MMCNN, 2200 Bergquist Dr, Suite 1, Lackland Air Force Base, TX 78236-5300 (e-mail: christopher.williams{at}59MDW.WHMC.af.mil).

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