格局打开!一则在风湿免疫科确诊的神经梅毒病例分享

2022-04-09 木木 MedSci原创

新英格兰杂志在2022年2月10日报道了这样一则在风湿免疫科确诊神经梅毒的临床病例。

新英格兰杂志在2022年2月10日报道了这样一则在风湿免疫科确诊神经梅毒的临床病例。

患者,男,55岁,因双侧听力丧失和眼睛发红前来风湿免疫科就诊。情回顾

8周前

出现左侧太阳穴疼痛,15年前的一次带状疱疹发作期间也发生过类似的头痛。当时医生推测此次太阳穴疼痛是由带状疱疹引起的,遂开具了为期一周的伐昔洛韦疗程的处方。此过程中无皮肤症状,头痛也得到了缓解。

4周前

在飞机飞行期间出现左耳听力损失。飞行后听力损失持续存在,患者开始出现左耳间歇性耳鸣和双眼发红。遂至耳鼻喉科就诊。患者无眩晕、耳漏、鼻炎或喉咙痛。

查体:两眼发红,左右耳廓和外耳道正常,右鼓膜也正常,左侧鼓膜回缩,无中耳积液。韦伯检验的结果不一致。双侧 Rinne 试验阳性,气导大于骨导。无窦性压痛。鼻咽镜检查显示中隔偏左;鼻咽后部正常。其余检查,包括颅神经检查,均正常。听力图显示双耳有倾斜的轻度至重度感音神经性听力损失(图1A)。结果在低频时不对称,在 125 至 2000 Hz 范围内,左耳的听力损失比右耳更严重。右耳的单词识别率为 96%,左耳的单词识别率为 84%(参考范围,80 到 100)。双耳的鼓室压测量结果正常。

患者接受了为期 12 天的口服泼尼松逐渐减量疗程的处方。

 

图片

治疗4 周后

耳鼻喉科复诊结果:听力损失有所进展,开始泼尼松治疗后眼睛发红最初有所减轻,但在泼尼松治疗停止后又复发

查体:双眼发红,无自发性眼球震颤或步态共济失调。正弦注视跟踪正常。左侧的头部推力试验呈阳性,提示左侧前庭功能减退。Romberg 试验结果正常。福田台阶试验阳性,患者向左旋转提示左侧前庭功能减退。鼓室测量结果正常(图1B)。该患者被转诊进行紧急风湿病评估。

3天后

三天后,患者于风湿科门诊就诊。

主诉:双耳听力持续下降,双眼发红。近2 个月感觉疲劳和间歇性身体疼痛,并且在 6 个月内体重减轻了 5 公斤。无口腔或生殖器病变史;6 周前,他在接种流感疫苗后躯干出现无瘙痒性斑片状皮疹,口服抗组胺药后迅速消退。

肠易激综合征、憩室病、胃炎、过敏性鼻炎和哮喘病史。药物包括鼻内和吸入氟替卡松、维生素 B12和奥美拉唑。没有已知的药物过敏。病人和他的妻子住在新英格兰的一个市区。无冶游史。患者是一名退休的建筑工人。他不吸烟,很少喝酒,也没有使用违禁药物。 

经检查,患者表现良好。体温 36.9°C,血压 115/79 mm Hg,心率 95 次/分钟,呼吸频率 15 次/分钟,呼吸环境空气时的氧饱和度为 100%。两眼发红。手指摩擦测试显示双耳听力严重下降。Romberg 试验呈阳性,提示前庭或小脑功能减退。

其余检查,包括皮肤、关节和神经系统评估,均正常。电解质和葡萄糖的血液水平正常,肝功能和肾功能检查结果也正常。分类计数的全血细胞计数正常。红细胞沉降率为每小时 55 毫米(参考范围,0 ~ 13),和 C 反应蛋白水平 12.3 mg/L(参考值,<8.0)。

 在眼科评估中,患者报告没有视力模糊、飞蚊症、眼痛、瘙痒或畏光。经检查,双眼视力正常。右侧角膜有微量的针状上皮沉积,周围有一个点状上皮糜烂环,中央有几个小的点状上皮糜烂汇合区,无浸润。左侧角膜周围有一圈点状上皮糜烂,中央有数个点状上皮糜烂汇合区,无浸润。有前葡萄膜炎的证据,右前房每个高倍视野 7 个细胞,左前房每个高倍视野 6 至 7 个细胞。

患者描述的症状示意图

图片

 

这位 55 岁的男性患者的主要症状为进行性听力丧失、失衡和眼部炎症。患者有 6 个月的体重减轻史、2 个月的身体疼痛和疲劳史以及躯干上出现非瘙痒性皮疹史,8周的涉及左侧太阳穴的头痛。在评估期间,红细胞沉降率和C反应蛋白水平升高。

在这一系列发现的基础上,将把鉴别诊断重点放在有可能引起颞部疼痛、不对称感音神经性听力损失和眼部炎症的疾病上。

图片

图片

图片

 

总之,与眼睛和耳朵受累以及全身体征和症状相关的全身性疾病的可能性有很多。最终考虑患者的诊断为Cogan 综合征或梅毒。考虑到梅毒的诊断的可行性,选择用脑脊液 (CSF) 分析进行腰椎穿刺以寻找细胞增多症,并进行性病研究实验室 (VDRL) 测试和荧光密螺旋体抗体吸收 (FTA-ABS) 测试。

测试结果

发光微粒免疫测定:IgM(+) 和 IgG(+);患者的快速血浆反应素 (RPR) 滴度为1:512, 呈阳性,非梅毒螺旋体试验也为阳性。

患者的脑脊液分析显示无色不混浊液体,每立方毫米有 21 个有核细胞,54% 中性粒细胞,22% 淋巴细胞,24% 单核细胞,总蛋白水平为 83 g/dL。CSF VDRL 测试在 1:2 时呈反应性;该结果支持神经梅毒的诊断。

答案已经很明显了,患者的诊断为:神经梅毒。

那为什么神经梅毒会导致患者的听力损失呢?可能的病理生理过程是什么?

有证据表明,梅毒螺旋体通过血行播散到全身,并可能进入颞骨。在梅毒的晚期阶段,当 CSF 通过内耳道或耳蜗导水管时,它会进入内耳。内耳具有免疫反应性,梅毒螺旋体应通过促炎细胞因子和 CD4+ 和 CD8+ T 细胞引起体液抗体反应并激活先天免疫。这种反应的所有必要成分都位于内淋巴囊区域的内耳中。

梅毒诊治后继续使用糖皮质激素会增加听力恢复的可能性吗?

哈里斯博士:有各种形式的快速进展性感觉神经性听力损失的症状的患者通常在早期就会接受大剂量糖皮质激素治疗,有时在鼓室内进行糖皮质激素治疗,这可能会逆转听力损失和头晕。单独使用抗生素治疗神经梅毒可能会导致感染的控制,但需要糖皮质激素来减少内耳的炎症反应并防止不可逆的纤维化和成骨。

因此,如果患者接受了符合疾病控制和预防中心建议的长期抗生素治疗,将考虑后续使用糖皮质激素治疗。

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    2022-06-04 ms173570681524994

    神经梅毒有各种表现,需注意

    0

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    2022-04-10 4977194

    风湿免疫科

    0

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