Manabe Y, Sasaki C, Warita H, Hayashi T, Shiro Y, Sakai K, Kashihara K, Abe K
Department of Neurology, Okayama University Medical School, 2-5-1 Shikata-cho, 700-8558, Okayama, Japan.
J Neurol Sci. 2000 Jun 15;176(2):158-61. doi: 10.1016/s0022-510x(00)00334-8.
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.
这是首例以急性横贯性脊髓炎作为原发性干燥综合征(SS)首发表现的病例报告。该患者在发病6天内出现四肢轻瘫和感觉障碍。脊髓磁共振成像显示实质内广泛病变,T2加权像呈高信号强度,钆增强及脊髓肿胀。尽管患者未表现出临床干燥症状,但基于泪腺和唾液腺的临床检查显示自身抗体水平升高,诊断为原发性SS。泼尼松治疗改善了运动和感觉症状,并使磁共振成像结果得到改善。本病例提示急性横贯性脊髓炎可作为SS的首发症状出现。