第六、第十二脑神经联合麻痹1例:罕见胸腺瘤引起的斜坡综合征

Yew Chung Chan, Chin Aun Liew, Darcy Jamih, Yin Chin Chan, Mei Dree Lim, Angeline Madatang, Sandhya AP Rajaintharan
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引用次数: 0

摘要

Godtfredsen综合征或斜坡综合征是一种罕见的外展神经和舌下神经麻痹综合征(分别是颅神经第6和第12),它定位于斜坡肿块。这种临床表现的病例报道很少。本病例报告的目的是描述这种罕见的表现,观察到妇女与斜坡转移引起胸腺瘤。先前身体健康的34岁当地妇女,因视力模糊和头痛病史1个月来到马来西亚沙巴一家地区医院就诊。脑神经检查显示右侧展神经麻痹(右侧第六神经中枢)和右侧舌下神经麻痹(右侧第十二神经中枢)。最初的脑部CT显示一个微妙的轴外高密度肿块靠近斜坡,常规胸片显示一个纵隔肿块。进一步的胸部成像显示右侧前纵隔肿块,然后进行视频辅助胸腔镜手术(VATS)指导下的活检。活检结果与胸腺瘤B2型一致。然后她被诊断出患有侵袭性胸腺瘤,不幸的是已经转移到骨、肺、肝和脑。尽管在诊断时的预后是谨慎的,她仍然选择接受化疗。尽管完成了6个周期的化疗,她的病情仍在恶化,最终她死于疾病。总之,第6和第12麻痹合并的存在应该提醒临床医生注意斜坡肿块或转移的可能性,因此早期的检查和适当的影像学检查可以导致早期诊断和更好的治疗结果。
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Case Report of Combined Sixth and Twelfth Cranial Nerve Palsy: A Rare Case of Clival Syndrome Arising from Thymoma
Godtfredsen syndrome or clival syndrome is a rare syndrome of abducens and hypoglossal nerve palsies (cranial nerve 6th and 12th respectively) that localizes to a clival mass. There are few reported cases of this clinical presentation. The aim of this case report is to describe this rare manifestation observed in a woman with clival metastases arising from a thymoma. A previously well 34-year-old native lady presented to a district hospital in Sabah, Malaysia, with history of blurring of vision and headache for 1 month. Cranial nerve examination reveals right abducens nerve palsy (right 6th CN) and right hypoglossal nerve palsy (right 12th CN). Initial imaging with CT brain reveals a subtle extra-axial hyperdense mass adjacent to the clivus and a routine chest x-ray reveals a mediastinal mass. Further imaging of thorax showed right anterior mediastinal mass, which then proceeded with Video-Assisted Thoracoscopic Surgery (VATS) guided biopsy. The biopsy result was consistent with the finding of thymoma, type B2. She was then diagnosed with aggressive form of thymoma, which unfortunately has metastasized to the bone, lung, liver and brain. Despite prognosis at the time of diagnosis is guarded, she still opted to undergo chemotherapy. Despite the completion of 6 cycles of chemotherapy, her disease progressed, and she eventually succumbed to the illness. In short, the presence of combined 6th and 12th palsy should alert clinician to the possibility of clival mass or metastases and hence could earlier workup with appropriate imaging can lead to earlier diagnosis and better treatment outcome.
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