伴CNS炎性脱髓鞘的原发性干燥综合征8例临床分析

Clinical analysis of 8 cases of primary Sjgren's syndrome with CNS inflammatory demyelination

目的探讨伴有CNS炎性脱髓鞘的原发性干燥综合征(p SS)的临床特点。方法回顾性分析8例诊断明确的伴有CNS炎性脱髓鞘的p SS患者的临床资料。结果 8例患者中7例以CNS症状表现为首发症状。在3例出现脑损害症状的患者中,1例表现为头晕、视物重影、饮水呛咳、行走困难,其病灶位于延髓至颈髓C2段脱髓鞘病灶;1例表现为听力下降、耳鸣,病灶位于双侧额颞顶深部白质脱髓鞘病灶;1例表现为反应迟钝、听力下降,头颅MRI示双侧额顶叶深部多发脱髓鞘病灶。其余5例均为脊髓损害症状患者,单纯颈髓受累1例,单纯胸髓受累1例,颈胸腰骶髓受累1例,颈胸髓同时受累1例,胸腰段受累伴左侧颞极病灶1例。2例脊髓炎患者行血清水通道蛋白4(AQP4)抗体检测为阳性。8例患者中1例患者放弃治疗,7例患者接受治疗,其中5例好转,2例无明显疗效。结论在CNS脱髓鞘的患者中应注意筛查是否合并系统性疾病如p SS等,在p SS患者中若出现视神经脊髓炎典型表现应查AQP4抗体以尽早诊断是否二者共病。

Objective To evaluate the clinical features of primary Sjogren＇s syndrome （pSS） patients with CNS inflammatory demyelination. Methods The clinical data of 8 pSS patients with CNS inflammatory demyelination were analyzed retrospectively. Results Neurological symptoms preceded the diagnosis of pSS in 7 patients. Three patients had brain involvement and 5 patients had spinal cord involvement. Among the 3 patients with brain involvement, one patients who presented with dizziness, diplopia, dysphagia and difficult walking had demyelination lesions from medulla to C2. One patient with hearing loss and tinnitus had demyelination lesions in deep white matter of frontotemporal and parietal lobe. One patient presented with slow mental reaction and hearing loss, whose MR showed demyelination lesions in deep white matter of bilateral frontal and parietal lobe. Five patients had spinal cord involvement and the lesions predominantly located in cervical and thoracic cords. Two patients with blurred vision and myelitis underwent serum and cerebrospinal fluid inspection for anti-aquaporin-4 （AQP4） antibody, which showed positive results. Among the 8 cases, except for one abandoning treatment, seven cases received treatment, five of them had good response while the other two had no obvious response. Conclusions Patients with CNS inflammatory demyelination should be screen for systemic autoimmune disease, such as pSS. If pSS patients present typical optic neuritis and longitudinal extensive transverse myelitis, AQP4 antibody is supposed to be done for diagnosis of neuroinyelitis optica.