Eye twist and tongue twist: a rare neurological syndrome

Thapa, Lekhjung; Paudel, Raju; Chhetri, Pradip; Rana, Pvs

doi:10.1136/bcr.06.2011.4366

Cited by 3 publications

(2 citation statements)

References 4 publications

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“…The causes for the 5 cases of "Clival Syndromes" were a case of local chordoma, 3 cases of distant metastases from prostate, pancreas and ?ovary, and a case of ?self-limited cranial polyneuropathy. Thapa et al [3] described a case with similar presentation secondary to metastasis from rectal adenocarcinoma. Amalnath [4] described a similar case due to retro-clival subdural hematoma.…”

Section: Discussionmentioning

confidence: 96%

Case Report of Combined Sixth and Twelfth Cranial Nerve Palsy: A Rare Case of Clival Syndrome Arising from Thymoma

Chan,

Liew,

Jamih

et al. 2023

CRCM

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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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Section: Discussionmentioning

confidence: 96%

Case Report of Combined Sixth and Twelfth Cranial Nerve Palsy: A Rare Case of Clival Syndrome Arising from Thymoma

Chan,

Liew,

Jamih

et al. 2023

CRCM

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“…A literature review found 9 cases in the past 20 years (Table 1 ). 55.5% (5/9) of the cases are secondary to metastatic diseases at clivus [ 2 , 5 – 8 ]. Amongst the 9 cases, 66.7% (6/9) are tumour origin.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2022

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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.

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Godtfredsen syndrome

Sharma¹

2021

Radiopaedia.org

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Eye twist and tongue twist: a rare neurological syndrome

Cited by 3 publications

References 4 publications

Case Report of Combined Sixth and Twelfth Cranial Nerve Palsy: A Rare Case of Clival Syndrome Arising from Thymoma

Case Report of Combined Sixth and Twelfth Cranial Nerve Palsy: A Rare Case of Clival Syndrome Arising from Thymoma

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

Godtfredsen syndrome

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