Rosemberg S, Amaral L C, Kliemann S E, Arita F N
Department of Pediatrics, Santa Casa of São Paulo School of Medicine, Brasil.
Neuropediatrics. 1992 Dec;23(6):310-5. doi: 10.1055/s-2008-1071364.
Two patients with acute encephalopathy with bilateral striatal necrosis are presented and the literature on the subject is reviewed. The disease is characterized by abrupt onset following a systemic infectious illness, with disturbance of consciousness, absence of speech, dystonic movements of the limbs, general stiffness, opisthotonus, tremor, facial grimacing, and stereotyped reaction to painful stimuli. After a variable period of time, there is gradual improvement of the neurological status with clearing of consciousness and recovery of motor functions. Mild CSF pleocytosis is the only abnormal laboratory test encountered. Cranial imaging shows from the beginning of the illness, bilateral involvement of the striatum that may persist indefinitely. The pathogenesis of this disorder remains unknown although an infectious or para-infectious mechanism seems to be the most likely possibility.
本文报告了2例患有双侧纹状体坏死性急性脑病的患者，并对该主题的文献进行了综述。该疾病的特征是在全身性感染性疾病后突然发病，伴有意识障碍、失语、肢体张力障碍性运动、全身僵硬、角弓反张、震颤、面部怪相以及对疼痛刺激的刻板反应。经过一段可变的时间后，神经状态逐渐改善，意识清醒，运动功能恢复。轻度脑脊液淋巴细胞增多是唯一遇到的异常实验室检查结果。头颅影像学检查显示，从疾病开始时起，双侧纹状体均受累，且这种情况可能会无限期持续。尽管感染或感染后机制似乎是最有可能的原因，但这种疾病的发病机制仍然未知。
