Tien R D, Ashdown B C, Lewis D V, Atkins M R, Burger P C
Department of Radiology, Duke University Medical Center, Durham, NC 27710.
AJR Am J Roentgenol. 1992 Jun;158(6):1329-32. doi: 10.2214/ajr.158.6.1590136.
Rasmussen's encephalitis is a devastating disease of childhood causing progressive neurologic deficits and intractable seizure activity. Patients frequently have episodes of epilepsia partialis continua and, much less frequently, generalized status epilepticus. The seizures are intractable despite aggressive medical management. In advanced cases, hemispherectomy appears to be the only option to control the seizures. Permanent physical and mental impairments are inevitable. The cause of this disease is unknown, although pathologic specimens demonstrate nonspecific changes that are compatible with viral encephalitis. The progressive brain damage is typically so insidious in onset and gradual in course that it is difficult to make an accurate diagnosis on the basis of clinical evidence. We retrospectively evaluated the CT, xenon CT, positron emission tomographic, and MR neuroimaging findings in four young patients with pathologically suspected Rasmussen's encephalitis (three patients had CT scans, two had xenon CT scans, two had MR scans, and one had a positron emission tomogram). All studies showed abnormalities of the involved cerebral hemisphere: CT and MR revealed nonspecific atrophy, xenon CT showed decreased cerebral blood flow, and positron emission tomography revealed a hypometabolic state. Rasmussen's encephalitis is a diagnosis of exclusion; however, the information obtained from neuroimaging studies in combination with the clinical course should suggest this disorder.
拉斯穆森脑炎是一种严重的儿童疾病，可导致进行性神经功能缺损和顽固性癫痫发作。患者常出现持续性部分性癫痫发作，较少出现全身性癫痫持续状态。尽管积极进行药物治疗，癫痫发作仍难以控制。在晚期病例中，半球切除术似乎是控制癫痫发作的唯一选择。永久性的身体和智力损伤不可避免。尽管病理标本显示出与病毒性脑炎相符的非特异性变化，但该病的病因尚不清楚。进行性脑损伤通常起病隐匿、病程缓慢，仅凭临床证据很难做出准确诊断。我们回顾性评估了4例病理怀疑为拉斯穆森脑炎的年轻患者的CT、氙CT、正电子发射断层扫描和磁共振神经影像学检查结果（3例患者进行了CT扫描，2例进行了氙CT扫描，2例进行了磁共振扫描，1例进行了正电子发射断层扫描）。所有检查均显示受累脑半球异常：CT和磁共振显示非特异性萎缩，氙CT显示脑血流量减少，正电子发射断层扫描显示代谢减低状态。拉斯穆森脑炎是一种排除性诊断；然而，神经影像学检查获得的信息结合临床病程应提示该病。
