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Sjögren's syndrome with acute transverse myelopathy as the initial manifestation
1Department of Neurology, Okayama University Medical School, 2-5-1 Shikata-cho, 700-8558, Okayama, Japan. manabe@cc.okayama-u.ac.jp
Abstract:
This is the first report of a patient with acute transverse myelopathy as the initial manifestation of primary Sjögren's syndrome (SS). The patient developed tetraparesis and sensory disturbance within 6 days of onset. Spinal MRI showed an extensive intraparenchymal lesion with high T2-weighted signal intensity, gadolinium enhancement, and cord swelling. Although the patient did not show clinical sicca symptoms, primary SS was diagnosed on the basis of clinical tests on lacrimal and salivary glands which showed high levels of autoantibodies. Treatment with prednisone improved motor and sensory symptoms and resulted in improvement of MRI findings. The present case suggests that acute transverse myelopathy can occur as an initial symptom of SS.
Insights
Acute transverse myelopathy, a spinal cord disorder, can be the first sign of primary Sjögren
Area of Science:
- Neurology
- Rheumatology
- Immunology
Background:
- Primary Sjögren's syndrome (SS) is a chronic autoimmune disease primarily affecting exocrine glands.
- Neurological manifestations in SS can occur but are typically diagnosed after the onset of sicca symptoms.
Observation:
- A patient presented with acute transverse myelopathy, characterized by rapid onset tetraparesis and sensory disturbances.
- Spinal MRI revealed an extensive intraparenchymal lesion with significant cord swelling and enhancement.
- The patient initially lacked typical sicca symptoms.
Findings:
- Despite the absence of overt sicca symptoms, the diagnosis of primary SS was confirmed via autoantibody levels and lacrimal/salivary gland tests.
- Treatment with prednisone led to significant improvement in both motor/sensory deficits and MRI findings.
- This case represents the first report of acute transverse myelopathy as an initial manifestation of primary SS.
Implications:
- Acute transverse myelopathy should be considered in the differential diagnosis of unexplained myelopathy, even without classic SS symptoms.
- Early diagnosis and treatment of SS-associated myelopathy can lead to favorable neurological outcomes.
- This case highlights the diverse neurological presentations of primary Sjögren's syndrome.