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A case report of Sj?gren syndrome manifesting bilateral basal ganglia lesions

机译:Sj?gren综合征双侧基底神经节病变的一例报道

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Rationale: Peripheral neurological complications in primary Sj?gren's syndrome (pSS) seem the most common, however the involvement of central nervous system (CNS) remains unclear. While abnormalities in pSS revealed by brain magnetic resonance imaging (MRI) are usually small discrete hyperintense areas in the white matter on T2-FLAIR weighted MRI, massive brain lesions have been rarely reported, particularly in bilateral basal ganglia. Patient concerns: A 51-year-old woman exhibited dizziness, slurred speech and hemiplegia as a manifestation of pSS. Brain MRI revealed bilateral and symmetrical lesions extending into the basal ganglia, corona radiata and corpus callosum. Diagnoses: Primary Sj?gren's syndrome was diagnosed on the basis of clinical features, abnormal Schirmer's test and tear break-up time (BUT) findings, high levels of anti-Sj?gren's-syndrome-related antigen A (anti-SSA) (Ro) and anti-Sj?gren's-syndrome-related antigen B (anti-SSB) (La) antibodies, and positive labial minor salivary gland biopsy results. Interventions: She was treated with intravenous methylprednisolone and discharged on oral steroid therapy of prednisolone acetate. Outcomes: The patient had an excellent response to steroid therapy. Lessons: The present case suggests that symmetry bilateral lesions can occur as a symptom of pSS, which could be induced by an autoimmune mechanism.
机译:理由:原发性干燥综合征(pSS)的周围神经系统并发症似乎最为常见,但是中枢神经系统(CNS)的受累程度仍不清楚。尽管脑磁共振成像(MRI)揭示的pSS异常通常是T2-FLAIR加权MRI上白质中离散的小高信号区,但很少报道大规模脑损伤,特别是在双侧基底神经节中。患者关注:一名51岁女性表现出头晕,言语不清和偏瘫,这是pSS的表现。脑部MRI显示双侧和对称性病变扩展到基底神经节,电晕放射线和。体。诊断:原发性干燥综合征是根据临床特征,Schirmer检验异常和泪液破裂时间(BUT)发现,高水平的抗Sjgren综合征相关抗原A(anti-SSA)诊断的( Ro)和抗Sjgren's综合征相关抗原B(抗SSB)(La)抗体,以及阴唇小唾液腺阳性活检结果。干预措施:她接受了静脉注射甲基泼尼松龙治疗,并接受口服泼尼松龙醋酸酯类固醇激素治疗后出院。结果:患者对类固醇疗法有很好的反应。经验教训:本病例表明,对称性双侧病变可能是pSS的症状,可能是由自身免疫机制引起的。

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