Ararat Kerime, Berrios Idanis, Hannoun Anas, Ionete Carolina
Department of Medicine, Neurology, University of Massachusetts Medical School, Worcester, Massachusetts, USA.
Department of Neurology, University of Massachusetts School of Medicine, Worcester, Massachusetts, USA.
BMJ Case Rep. 2018 Jun 4;2018:bcr-2017-223468. doi: 10.1136/bcr-2017-223468.
There are only six cases in literature that describe development of dystonia with Sjogren's syndrome (SS). We describe a case of a 43-year-old woman who presented with symptoms including movement disorder, sensory neurogenic bladder, sensory loss and neuropathic pain, migraine like headaches, musculoskeletal pain, Raynaud's phenomenon and dysautonomia. Symptoms started in 2000, with weakness that progressed to dystonia in 2003. Diagnostic work-up was inconclusive with negative inflammatory serologies, cerebrospinal fluid and MRI for many years. After patient developed sicca syndrome with dry eyes and mouth in 2009, her rheumatoid factor titre was elevated (550 IU/mL), erythrocyte sedimentation rate, anti-Sjogrens syndrome-related antigen A (anti-Ro/SSA) and anti-SSB/La: anti-Sjogrens syndrome-related antigen B (anti-La/SSB) became positive. Lip biopsy confirmed diagnosis of SS. She was diagnosed with primary SS with neurological involvement. Her symptoms responded well to intravenous methylprednisolone. Symptoms stabilised with trials of immune-suppressive therapy. This is a case that demonstrates the delay of diagnosing SS with preceding unique neurological association.
文献中仅有6例描述了干燥综合征(SS)伴发肌张力障碍的病例。我们报告1例43岁女性,其症状包括运动障碍、感觉神经性膀胱、感觉丧失、神经性疼痛、偏头痛样头痛、肌肉骨骼疼痛、雷诺现象和自主神经功能障碍。症状始于2000年,2003年出现无力并进展为肌张力障碍。多年来,炎症血清学、脑脊液和MRI检查均无结论且结果为阴性。2009年患者出现干眼症和口干的干燥综合征后,类风湿因子滴度升高(550 IU/mL),红细胞沉降率、抗干燥综合征相关抗原A(抗Ro/SSA)和抗SSB/La:抗干燥综合征相关抗原B(抗La/SSB)呈阳性。唇活检确诊为SS。她被诊断为原发性SS伴神经系统受累。她的症状对静脉注射甲泼尼龙反应良好。免疫抑制治疗试验使症状稳定。这是1例显示伴有独特神经系统关联的SS诊断延迟的病例。