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肉芽肿性多血管炎合并颅内动脉瘤1例

2018-01-21 佚名 中国微侵袭神经外科杂志

男,67岁

【一般资料】

男,67岁

【主诉】

因“左侧头痛3个月余,左耳鸣伴听力下降2个月余,左眼视力下降2周”入本院肿瘤科。

【现病史】

病人2016年5月出现左侧头痛,在当地按照“鼻炎”治疗,1个月后出现左侧耳鸣,伴左侧听力下降,在外院行经鼻内镜手术,内镜见:鼻咽左侧壁黏膜稍增厚,病理提示:鼻咽黏膜慢性炎症,术后予止痛治疗。2016年8月中旬出现左侧上睑下垂伴左眼视力、听力渐进性下降,外院MRI:左侧上颌窦、筛窦及乳突炎症,鼻咽顶部片状异常信号增高影,病灶侵犯斜坡、颅底周围肌群,考虑鼻咽癌可能。病人自发病以来体质量减轻7.5kg。

【体格检查】

左侧上睑下垂,左眼视力丧失,左侧眼球固定,左眼瞳孔直径约5mm,直接对光反射消失,间接光反射迟钝;右眼视力正常,右眼瞳孔直径约3mm,直接对光反射正常,间接对光反射消失。本院再行“鼻咽黏膜活检”,病理示:黏膜显慢性炎。

【辅助检查】

腰椎穿刺压力75mmH2O,性质:细胞学呈淋巴-单核细胞反应型。抗核抗体(-),血沉:52mm/h(正常参考值0~28mm/h),C-反应蛋白:26.6mg/L(正常参考值0~6mg/L)。头颅MRI示鼻咽顶后部等T1、长T2信号,向前、向上侵犯左侧海绵窦区、左侧视神经管,增强后见明显斑片状强化。手术行左侧翼点入路病变活检术。术后复查病变部分切除。病理结果:左蝶骨嵴占位,镜下见骨小梁间纤维组织增生活跃;病变硬脑膜组织显急性及慢性肉芽肿性炎症改变。

【初步诊断

诊断为肉芽肿性多血管炎(granulomatosiswithpolyangiitis,GPA)。

【治疗】

术后给予激素、人免疫球蛋白、环磷酰胺治疗,左侧头痛症状明显改善,左侧听力恢复正常。治疗4周后病变体积变小。在药物治疗过程中,病人突然出现剧烈头痛,后意识状态转为嗜睡状,复查头颅CT提示蛛网膜下腔出血,颅内动脉瘤破裂可能性大。


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    2018-01-23 jyzxjiangqin

    肉芽肿性多血管炎.

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