Treatment of myelopathy in Sjögren syndrome with a combination of prednisone and cyclophosphamide.
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.


OBJECTIVE
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.


METHODS
Case report and literature review.


METHODS
University hospital.


METHODS
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.
Williams
C S
CS
Department of Neurology, Wilford Hall Medical Center, Lackland Air Force Base, Texas, USA. christopher.williams@59MDW.WHMC.af.mil
Butler
E
E
Román
G C
GC
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009188:Myelitis, Transverse / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology*; D009901:Optic Nerve Diseases / Q000209:etiology; D011241:Prednisone / Q000627:therapeutic use*; D012859:Sjogren's Syndrome / Q000150:complications*
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone