[A case of Sjögren's syndrome with subacute transverse myelitis as the initial manifestation]

Abstract

We report a case of subacute transverse myelitis associated with Sjögren's syndrome free of xerosis. A 62-year-old man was admitted due to dysesthesia of both lower extremities and the left trunk, weakness of the left leg, and urinary disturbance. Neurological examination showed myelopathy at the Th7 level. CSF had increased protein (82 mg/dl) and IgG (23.4 mg/dl) and IgG index (1.03) but an almost normal cell count (7/mm3). T2-weighted MRI showed a high signal intensity lesion at the sixth and seventh thoracic levels. Although he was free of xerosis, typical sialographic findings, as well as the presence of anti-SSA antibody, are consistent with the diagnostic criteria for Sjögren's syndrome decided by the Japanese research group on Sjögren's syndrome. The patient was treated with prednisolone, 60 mg/day, which completely cured his muscle weakness and difficulty in walking, and sensory disturbance was gradually alleviated. Spinal MRI detected a marked reduction in the size of T2-weighted high signal intensity lesion during prednisolone treatment. In Western countries, central nervous system complications are reported in up to 20% of Sjögren's syndrome patients, but myelopathy is a very rare condition. Only 12 cases, including ours, have been reported. The clinical manifestations of myelopathy in Sjögren's syndrome include acute or subacute transverse myelitis (6 cases, including ours), chronic progressive myelopathies (2 cases.), relapsing and remitting cord syndromes (4 cases) and Brown-Séquard syndrome (none). Ten patients were women. In 9 of 12 cases there were sicca symptoms. The level of the myelopathies in 6 of 10 cases was between the third to eighth thoracic level, consistent with the region vulnerable to ischemia. Eight patients were treated successfully with steroids. We speculate that ischemia due to vasculitis is important in the genesis of myelopathy associated with Sjögren's syndrome. In the case of myelopathy, especially in the thoracic cord, it is necessary to look for evidence of Sjögren's syndrome even when xerosis is unremarkable.