| 제목 |
A case report of Klippel-Feil syndrome with multiple cerebral infarctions |
| 소속 |
Ulsan University Hospital, Department of Physical Medicine and Rehabilitation1 |
| 저자 |
Woo-Ram Koo1,1*, Park Dae Kwon1,1, Chung Reen Kim1,1† |
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Klippel-Feil syndrome is a rare bone disorder characterized by cervical vertebral fusion and the multiple congenital anomalies of cardiovascular, renal or endocrine organs, Although there has been a few cases of the vertebral artery dissection and posterior circulation infarction secondary to hypermobility adjacent to fused vertebrae, anterior circulation infarction related to Klippel-Feil syndrome was not yet reported, In this report, we presented a case of Klippel-Feil syndrome with a concurrent anterior and posterior circulation stroke. A 47-year-old male was admitted to the department of neurology because of sudden left side weakness and diplopia. The magnetic resonance imaging showed multiple acute infarctions in right middle cerebral artery territory and left medulla and cerebellum. And the magnetic resonance arteriography revealed occlusion of the left vertebral artery. After the patient was treated with antiplatelet agents, his weakness and diplopia gradually improved. However, the patient also had the abnormal short neck and forward head posture, and suffered from severe neck and shoulder pains since long ago. Interestingly the cervical spine X-ray showed the fusion of multiple cervical vertebrae (C3 through C7), and then he was also diagnosed with Klippel-Feil syndrome type I. About the cause of posterior circulation stroke, the vertebral arterial occlusion might explain his left medullary and cerebellar infarction. However the cause of right middle cerebral artery infarction was unclear. Although the results of some cardiac work ups were normal, the cerebral embolism of cardiac origin was suspected, because Klippel-Feil syndrome often accompanies cardiac anomalies including the cardiac septal defect, and the level of cardiac enzymes were significantly elevated at admission. But it would be also possible that unrevealed cerebrovascular abnormalities might induce the anterior circulation stroke of this patient. |
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Fig 1. Diffusion weighted magentic resonance imagings of the brain. The white arrows indicate right cerebral and left cerebellar infarctions.
Fig 2. Lateral cervical spine X-ray. The white arrows indicate massive cervical spine fusion.
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