Abstract:

Objective  To investigate the clinical and imaging manifestations of secondary central nervous system vasculitis caused by primary Sjögren's syndrome (PSS). Methods  Thirty-five inpatients diagnosed as central nervous system involvement secondary vasculitis by Sjögren's syndrome were retrospectively analyzed in Beijing Tiantan Hospital. Results  Thirty females and five males were enrolled in this study. The range age was from 18 to 76 (52±14) years old. Twenty-four patients were involved the central nervous system as initial presentations of PSS. The clinical manifestations of acute cerebral vessel disease were 8 patients (22.86%), including 2 subarachnoid hemorrhage (5.71%), 2 transient ischemic attack (5.71%), 2 arteriovenous fistula (5.71%), 1 cerebral hemorrhage (2.86%), 1 venous sinus thrombosis (2.86%). Other clinical manifestations were including 10 cerebral white matter damage, 6 neuromyelitis optica, 5 spinal cord inflammations, 3 Parkinson syndrome, 2 trigeminal neuralgia, 1 encephalomyelitis. The imaging findings were respectively involved in 3 cases of aneurysm (2 cases of subarachnoid hemorrhage) (8.57%), 2 cases of temporal parietal occipital arteriovenous fistula (5.71%), 2 case of cerebral arterial stenosis (5.71%) and 1 case of superior sagittal sinus thrombosis (2.86%), 20 cases of paraventricular white matter, 16 cases of subcortical white matter, 8 cases of brainstem, 11 cases of spinal cord, 2 cases of slightly larger ventricle. The single lesion stages (no less than 3) of spinal cord/brainstem were 10 patients.

Conclusion  The onset of central nervous system involvement in PSS could be form of acute cerebrovascular disease, but the cerebral white matter lesions and spinal cord inflammation are most common. The specific imaging characteristic is single lesion involving muti-stages.

Key words: Primary Sjögren's syndrome; Second central nervous system vasculitis; Imaging

摘要：

目的   探讨原发性干燥综合征继发中枢神经系统血管炎的临床和影像特征。 方法   回顾性分析35例原发性干燥综合征继发中枢神经系统血管炎患者临床表现和影像学资料。 结果  男性5例，女性30例；年龄18～76［平均（52±14）］岁；中枢神经系统首发症状24例（68.57%）。其中以急性脑血管病发病患者8例（22.86%），表现为蛛网膜下腔出血2例（5.71%），短暂性脑缺血发作2例（5.71%），动静脉瘘2例（5.71%），脑出血1例（2.86%），静脉窦血栓1例（2.86%），其余的患者临床表现形式分别为：脑白质病变10例（28.57%），视神经脊髓炎6例（17.14%），脊髓炎5例（14.29%），帕金森综合征3例（5.57%），三叉神经痛2例（5.71%），脑脊髓炎1例（2.86%）。影像学表现动脉瘤3例（8.57%）（2例蛛网膜下腔出血）；动脉狭窄2例（5.71%），其中椎动脉狭窄1例（2.86%），大脑中动脉狭窄1例（2.86%）；颞顶枕动静脉瘘2例（占5.71%，三叉神经痛合并动静脉瘘1例）；上矢状窦血栓1例（2.86%）。室旁白质受累20例（57.14%），皮层下白质受累16例（45.71%），脑干8例（22.86%），脊髓11例（31.43%）；脑室扩大2例（5.71%）；其中脊髓/脑干单病灶累及多个脊髓阶段（≥3个脊髓阶段）患者10例（28.57%）。 结论  中枢神经系统原发性干燥综合征可以急性脑血管病形式起病，但以急慢性小血管受累的脑白质病和脊髓病最常见；单病灶多个脊髓阶段是脊髓病变的影像特点。

关键词: 原发性干燥综合征; 继发性中枢神经系统血管炎; 影像学