Anagnostou Evangelos, Papageorgiou Sokratis G, Potagas Constantinos, Alexakis Theodoros, Kalfakis Nikolaos, Anastasopoulos Dimitri
Department of Neurology, Eginition Hospital, University of Athens, Greece.
Clin Neurol Neurosurg. 2008 Mar;110(3):286-90. doi: 10.1016/j.clineuro.2007.10.008. Epub 2007 Dec 19.
Central nervous system (CNS) involvement in Langerhans' cell histiocytosis (LCH) has been described as a progressive neurological disorder marked by motor and cognitive decline. Detailed analysis of ocular motor abnormalities is lacking. We report on a 60-year-old male with histologically confirmed LCH who developed oscillopsia and gait ataxia over a 1-year period. Eye movements recorded with infrared oculography revealed a high rate of square-wave jerks (SWJ) with frequencies of 41 min(-1) on average and amplitudes between 1 degrees and 7 degrees , as well as marked impairment of smooth tracking of sinusoidally moving targets. Furthermore, static posturography disclosed increased body sway, with an abnormally high sway path. The initial brain MRI was unremarkable. Due to the presumed cerebellar dysfunction we performed a second MRI 1 year later that disclosed deep cerebellar lesions compatible with LCH relapse within the CNS. The abnormal high SWJ rate and the impaired smooth pursuit performance correctly heralded later involvement of the cerebellum anticipating lesion appearance in the MRI.
朗格汉斯细胞组织细胞增多症（LCH）累及中枢神经系统（CNS）被描述为一种以运动和认知功能衰退为特征的进行性神经疾病。目前缺乏对眼球运动异常的详细分析。我们报告了一名60岁男性，经组织学确诊为LCH，在1年时间里出现了视振荡和步态共济失调。通过红外眼动图记录的眼球运动显示，方波急跳（SWJ）发生率很高，平均频率为41次/分钟，幅度在1度至7度之间，同时对正弦运动目标的平滑跟踪明显受损。此外，静态姿势描记法显示身体摆动增加，摆动路径异常高。最初的脑部MRI检查无明显异常。由于推测存在小脑功能障碍，我们在1年后进行了第二次MRI检查，结果显示深部小脑病变，符合CNS内LCH复发。异常高的SWJ发生率和平滑跟踪性能受损正确地预示了小脑随后会受累，并在MRI中提前出现病变。
