摘要
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.
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.
作者
Yew Chung Chan
Chin Aun Liew
Darcy Jamih
Yin Chin Chan
Mei Dree Lim
Angeline Madatang
Sandhya A/P Rajaintharan
Yew Chung Chan;Chin Aun Liew;Darcy Jamih;Yin Chin Chan;Mei Dree Lim;Angeline Madatang;Sandhya A/P Rajaintharan(Department of Internal Medicine, Hospital Duchess of Kent, Sandakan, Sabah, Malaysia;Department of Internal Medicine, Hospital Kuala Lumpur, Kuala Lumpur, Malaysia;Department of Pathology, Queen Elizabeth Hospital I, Kota Kinabalu, Sabah, Malaysia)