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中央桥脑髓鞘溶解症的皮质脊髓束和桥脑小脑纤维

Corticospinal tract and pontocerebellar fiber of central pontine myelinolysis.

作者信息

Min Yong, Park Sung-Hee, Hwang Seung-Bae

机构信息

Department of Physical Medicine and Rehabilitation, Institute for Medical Sciences, Chonbuk National University Medical School, Research Institute of Clinical Medicine, Chonbuk National University Hospital, Jeonju 561-180, Korea.

出版信息

Ann Rehabil Med. 2012 Dec;36(6):887-92. doi: 10.5535/arm.2012.36.6.887. Epub 2012 Dec 28.

Abstract

Central pontine myelinolysis is a rare neurologic disorder that is defined by demyelination of longitudinally descending tracts and transversly crossing fibers in the basis pontis. Frequently observed clinical manifestations of this disorder include sudden weakness, dysphagia, loss of consciouness and locked-in syndrome. However, there have been a few studies that reported a benign course of this disease, which include cerebellar signs, such as ataxia, intention tremor, and dysarthria. Here we report on a 53-year-old male with a history of liver cirrhosis who showed the cerebellar type of central pontine myelinolysis. The patient was diagnosed with central pontine myelinolysis based on clinical presentations and magnetic resonance imaging findings after a liver transplantation. Conventional magenetic resonance imaging (MRI) revealed the preservation of the corticospinal tract and abnormal pontocerebellar fibers. However, these findings were not sufficient to define the pathophysiology of our patient. Electrophysiologic analysis and diffusion tensor imaging (DTI) were performed to investigate cerebellar signs in this case. Delayed central motor conduction time (CMCT) to the tibialis anterior muscle with transcranial magnetic stimulation (TMS) was observed, which indicated demyelination of the corticospinal tract. Also, diffusion tensor imaging showed abnormal pontocerebellar fibers, which might have been caused by cerebellar dysfunction in our patient. A combination of TMS and DTI was also used to determine the pathophysiology of this disease.

摘要

中央桥脑髓鞘溶解症是一种罕见的神经系统疾病,其定义为桥脑基底部纵向下行纤维束和横向交叉纤维的脱髓鞘。该疾病常见的临床表现包括突发无力、吞咽困难、意识丧失和闭锁综合征。然而,有少数研究报道了该疾病的良性病程,其中包括小脑体征,如共济失调、意向性震颤和构音障碍。在此,我们报告一例53岁患有肝硬化病史的男性,其表现为小脑型中央桥脑髓鞘溶解症。该患者在肝移植后根据临床表现和磁共振成像结果被诊断为中央桥脑髓鞘溶解症。传统磁共振成像(MRI)显示皮质脊髓束保留,桥小脑纤维异常。然而,这些发现不足以明确我们患者的病理生理学机制。进行了电生理分析和扩散张量成像(DTI)以研究该病例中的小脑体征。经颅磁刺激(TMS)观察到胫前肌的中枢运动传导时间(CMCT)延迟,这表明皮质脊髓束脱髓鞘。此外,扩散张量成像显示桥小脑纤维异常,这可能是由我们患者的小脑功能障碍引起的。TMS和DTI联合使用也用于确定该疾病的病理生理学机制。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a08e/3546196/e24fea6d6748/arm-36-887-g001.jpg

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