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戈特弗雷森综合征——复发性颅底软骨肉瘤 6 年随访:病例报告及文献复习。

Godtfredsen syndrome - recurrent clival chondrosarcoma with 6 years follow up: a case report and literature review.

机构信息

Ophthalmology Department, Kuala Lumpur Hospital, Kuala Lumpur, Malaysia.

Ophthalmology Department, Ampang Hospital, Ampang Jaya, Malaysia.

出版信息

BMC Neurol. 2022 Apr 11;22(1):134. doi: 10.1186/s12883-022-02654-w.

Abstract

BACKGROUND

We report a rare case of Godtfredsen syndrome caused by clival chondrosarcoma and perform a review of literatures. This article also explains the clinico-anatomical correlation of this rare neurological syndrome.

CASE PRESENTATION

A 22-year-old gentleman presented with binocular diplopia. Clinical examination revealed an isolated right abducent nerve and right hypoglossal nerve palsy, with other cranial nerves intact. Neuroimaging revealed a right clival mass. Supraorbital craniotomy and tumour debulking were done in the same year. Histopathological examination showed low-grade chondrosarcoma. After 5-years of default, he came back with the tumour enlarged. He underwent a right orbitozygomatic craniotomy and tumour excision with 33 cycles of radiotherapy. Despite two surgeries and radiotherapy, the abducent nerve and hypoglossal nerve did not improve throughout 6 years of follow-up. Cranial nerve VI palsy is not always a false localizing sign, in Godtfredsen syndrome it serves as a localizing sign.

CONCLUSION

To the best of our knowledge, this is the first case report of Godtfredsen Syndrome secondary to clival chondrosarcoma. Cranial nerve VI and XII palsy with no involvement of other cranial nerves, most likely the pathology is located at the clivus.

摘要

背景

我们报告了一例罕见的由颅底软骨肉瘤引起的 Godtfredsen 综合征,并对文献进行了回顾。本文还解释了这种罕见的神经综合征的临床解剖相关性。

病例介绍

一名 22 岁男性出现双眼复视。临床检查显示右侧展神经和右侧舌下神经麻痹,其他颅神经完好。神经影像学显示右侧颅底肿块。同年进行了眶上颅骨切开术和肿瘤切除术。组织病理学检查显示低度软骨肉瘤。5 年后肿瘤增大,他回来就诊。他接受了右侧眶颧颅底切开术和肿瘤切除术,并接受了 33 个周期的放疗。尽管进行了两次手术和放疗,但在 6 年的随访中,展神经和舌下神经没有改善。颅神经 VI 麻痹并不总是假性定位体征,在 Godtfredsen 综合征中,它是定位体征。

结论

据我们所知,这是首例由颅底软骨肉瘤引起的 Godtfredsen 综合征病例报告。颅神经 VI 和 XII 麻痹而无其他颅神经受累,最有可能的病变位于颅底。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/56f9/8996403/4fa9fa116d5d/12883_2022_2654_Fig2_HTML.jpg

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