Chen San-Feng, Chiu Ming-Jang, Su Jen-Jen
Department of Neurology, National Taiwan University Hospital.
Acta Neurol Taiwan. 2011 Mar;20(1):35-41.
We present a case report and a comprehensive review of the literature concerning aseptic meningoencephalitis and Sjögren's syndrome (SS).
We report a 44-year-old woman of primary SS with initial presentation of aseptic meningoencephalitis and a reversible magnetic resonance image (MRI) lesion in the medulla. The diagnosis of primary SS based on ocular dryness, lacrimal hyposecretion, secretory and excretory dysfunction from sialocintigraphy, and positive anti-SS-A antibodies. Corticosteroid and cyclophosphamide therapies reversed the neurological deficits and the MRI lesion.
Primary SS may have variable manifestations in the central nervous system which may precede the classic sicca symptoms. SS should be investigated in cases of aseptic meningoencephalitis even without clinical signs of xerostomia or xerophthalmia. MRI is useful in demonstrating brain lesions and in evaluating treatment efficacy of the SS.
我们报告一例病例，并对有关无菌性脑膜脑炎和干燥综合征（SS）的文献进行全面综述。
我们报告一名44岁原发性干燥综合征女性患者，最初表现为无菌性脑膜脑炎，延髓出现可逆性磁共振成像（MRI）病变。根据眼干、泪液分泌减少、唾液腺闪烁造影显示的分泌和排泄功能障碍以及抗SS - A抗体阳性，诊断为原发性干燥综合征。皮质类固醇和环磷酰胺治疗使神经功能缺损和MRI病变得到逆转。
原发性干燥综合征在中枢神经系统可能有多种表现，这些表现可能先于典型的口干和眼干症状出现。即使没有口干或眼干的临床体征，在无菌性脑膜脑炎病例中也应排查干燥综合征。MRI有助于显示脑部病变并评估干燥综合征的治疗效果。
