Neurology:Collet-Sicard综合征和听力丧失伴颈静脉鼓室球瘤

2019-10-30 zyx 神经科病例撷英拾粹

56岁妇女,表现为头痛,呕吐3天,并有右耳听力下降,耳鸣和吞咽困难3月余。查体提示右侧上颚抬高力弱,右胸锁乳突肌,斜方肌和舌肌萎缩,伸舌右偏,提示Collet-Sicard综合征(累及颈静脉孔和舌下神经管的IX–XII颅神经)。

56岁妇女,表现为头痛,呕吐3天,并有右耳听力下降,耳鸣和吞咽困难3月余。查体提示右侧上颚抬高力弱,右胸锁乳突肌,斜方肌和舌肌萎缩,伸舌右偏,提示Collet-Sicard综合征(累及颈静脉孔和舌下神经管的IX–XII颅神经)。听力减退提示耳道延长。影像学检查可见右侧颅底肿块,符合血管球瘤(glomus tumor)(图1-2)。考虑到手术风险,患者行进根治性放疗,阻止肿瘤生长。颈静脉球瘤(glomus jugulare tumors),富血管性副神经节瘤(hypervascular paragangliomas),是颈静脉孔处最常见的肿瘤,可以延伸至中耳(颈静脉鼓室,jugulotympanicum)。



(图1:轴位颞骨成像[A]可见扩张溶解性右侧颈静脉窝病变[红箭],延伸至外耳道[绿箭],可见强化[B],病灶在颈动脉鞘内延伸,颈内动脉向前移位[C];增强颈部CT[D,冠状位]可见病灶沿颈动脉间隙向头尾侧延伸[橙色括号],颈外动脉供血支过度增粗[蓝箭,E,矢状位],以及颈内静脉受侵犯[橙箭,F,冠状)



(图2:轴位T2WI[A]和T1WI[B]可见右侧颈静脉窝病变,呈盐和胡椒粉样外观;增强T1[C]可见病灶强化,通过变宽的舌下神经管延伸至小脑-延髓角[蓝箭];轴位T2WI[D]可见右侧舌部脂肪变性[红箭],右胸锁乳突肌萎缩[绿箭])

原始出处:Ganesh A, Assis Z, Fok D, et al. Teaching NeuroImages: Collet-Sicard syndrome and hearing loss with glomusjugulotympanicum. Neurology. 2019 Oct 1;93(14):e1408-e1409.

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