Objective ·To study the clinical features of two primary Sjögren's syndrome (pSS) patients with neurological symptoms as initial manifestations and review the related literature. Methods ·The clinical data, response to treatment as well as prognosis of 2 cases were analyzed and followed up. Results ·Case 1, female, initially presented progressive gait instability and distal sensory impairment at the age of 50. Clinical manifestations included spastic gait, sensory ataxia, conduction fascicular sensory impairment, as well as urination and defecation dysfunction. At the age of 58, this patient was unable to walk independently. The laboratory findings revealed a positive result for anti-nuclear antibody with a titer of 1∶1 000, anti-Sjögren's syndrome A (SSA)/Ro-52, anti-SSA/Ro-60, and anti-centromere antibodies. Labial salivary gland biopsy showed lymphocytes and plasma cells infiltration into the glandular tissues, interstitium, and lobules, with 2 foci/4 mm2 (lymphocytes >50). The cranial magnetic resonance imaging exhibited bilateral symmetric hyperintensity in the brainstem, characterized by the "snake-eye sign". Electromyography examination revealed axonal impairment of the right peroneal nerve. The patient was treated with high-dose corticosteroid therapy in combination with immunosuppressants. She experienced remarkable improvement. After three months, she was able to walk with aids and take care of herself in daily life. Case 2, female, presented spasmodic torticollis since the age of 81 with unknown reason. She had a history of mild mucosal dryness of mouth and eyes, and painful knee for one year. Laboratory findings revealed positive results for anti-SSA/Ro-52 and anti-SSA/Ro-60 antibodies, as well as hemoglobin of 86 g/L. Labial salivary gland biopsy demonstrated partial atrophy of the acini and the presence of 2 foci/4 mm2 of lymphocytes and plasma cells infiltration into the stroma (lymphocytes>50). Electromyography examination showed reduced conduction velocity in the right median nerve. She got a significant relief after the treatment of immunosuppressants, antispasmodics, and muscle relaxants. Conclusion ·Patients presenting initial symptoms such as complex forms of spastic paraplegia and cervical dystonia expand the clinical spectrum of pSS. In clinical practice, it is important to distinguish neurological involvement secondary to pSS from other primary neurological disorders. For patients with neurological impairments but without apparent etiology, it is crucial to screen relevant series of autoimmune antibodies.
YAO Li, TIAN Wotu, CAO Li. Two cases of primary Sjögren's syndrome with neurological impairment as initial symptom. Journal of Shanghai Jiao Tong University (Medical Science)[J], 2024, 44(6): 795-800 doi:10.3969/j.issn.1674-8115.2024.06.016
回顾性分析安徽医科大学附属宿州医院和上海交通大学医学院附属第六人民医院神经内科分别于2023年2月和7月收治的以神经系统损伤为首发症状的2例pSS患者临床资料。2例患者诊断均符合2016年美国风湿病学会（American Rheumatism Association，ARA）和欧洲抗风湿病联盟（European League Against Rheumatism，EULAR）制定的pSS诊断标准［16］。根据评分准则，唇腺活检灶性指数2个灶/4 mm2为3分，血清抗干燥综合征抗原A抗体（anti-Sjögren's syndrome A antibody，anti-SSA antibody）阳性为3分；2例患者均为6分，确诊pSS。
Note：A. "Snake-eye sign" in the brainstem on the T2 Flair-weighted image, which presents symmetrical hyperintensity in the corticospinal tract regions. B. Patchy hyperintensity in the right prefrontal lobe on the T2 Flair-weighted image.
YAO Li contributed to data collection and analysis and drafted the manuscript. TIAN Wotu and CAO Li contributed to the study design, data acquisition, interpretation of data, and manuscript revision. All the authors have read the last version of paper and consented for submission.
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COMPETING INTERESTS
All authors disclose no relevant conflict of interests.
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... 回顾性分析安徽医科大学附属宿州医院和上海交通大学医学院附属第六人民医院神经内科分别于2023年2月和7月收治的以神经系统损伤为首发症状的2例pSS患者临床资料.2例患者诊断均符合2016年美国风湿病学会（American Rheumatism Association，ARA）和欧洲抗风湿病联盟（European League Against Rheumatism，EULAR）制定的pSS诊断标准［16］.根据评分准则，唇腺活检灶性指数2个灶/4 mm2为3分，血清抗干燥综合征抗原A抗体（anti-Sjögren's syndrome A antibody，anti-SSA antibody）阳性为3分；2例患者均为6分，确诊pSS. ...
