伴有强直的进行性脑脊髓炎：一例报告
Progressive encephalomyelitis with rigidity: a case report.
作者信息
Baraba Ranka, Jusić Anica, Sruk Ana
机构信息
Department of Neurology, General Hospital "Sveti Duh", Zagreb, Croatia.
出版信息
J Spinal Cord Med. 2010;33(1):73-6. doi: 10.1080/10790268.2010.11689677.
BACKGROUND/OBJECTIVE: The most prominent clinical features of progressive encephalomyelitis with rigidity (PER) are painful spasms and rigidity accompanied by clinical signs of brainstem and spinal cord involvement. In initial reports, PER had fatal outcome. Later, clinical improvement related to corticosteroid therapy has been described in some cases. The objective of this study was to signify a reputed clinical significance of corticosteroid therapy in PER.
METHODS
Case report.
RESULTS
A 50-year-old man developed progressive syndrome of tonic extensor spasms. Magnetic resonance imaging (MRI) showed areas of signal changes in cervical spinal cord and lower brainstem, whereas cerebrospinal fluid analysis indicated subacute encephalomyelitis. His condition dramatically improved on oral corticosteroid therapy. Clinical improvement was accompanied by normalization of MRI findings.
CONCLUSION
For this patient with PER, corticosteroid therapy was a dramatically effective and life-saving treatment, although initiated rather late in the course of the disease.
背景/目的：进行性脑脊髓炎伴强直（PER）最突出的临床特征是疼痛性痉挛和强直，并伴有脑干和脊髓受累的临床体征。在最初的报告中，PER的预后是致命的。后来，在一些病例中描述了与皮质类固醇治疗相关的临床改善情况。本研究的目的是阐明皮质类固醇治疗在PER中的显著临床意义。
方法
病例报告。
结果
一名50岁男性出现进行性强直性伸肌痉挛综合征。磁共振成像（MRI）显示颈脊髓和脑桥下部有信号改变区域，而脑脊液分析提示亚急性脑脊髓炎。口服皮质类固醇治疗后，他的病情显著改善。临床改善伴随MRI表现正常化。
结论
对于该PER患者，尽管在疾病过程中开始治疗较晚，但皮质类固醇治疗是一种极其有效且挽救生命的治疗方法。
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