原发性侧索硬化临床及影像特征分析

Clinical and imaging analysis of primary lateral sclerosis

关键词:  原发性侧索硬化  运动神经元病  肌萎缩侧索硬化

Key words:  primary lateral sclerosis  motor neuron disease  amyotrophic lateral sclerosis

摘要:

目的　探讨原发性侧索硬化（PLS）的临床、影像特点及其鉴别诊断。方法与结果　收集2012年10月至2017年5月于海军总医院神经内科住院治疗的4例PLS患者的临床及影像学资料。其中男性3例，女性1例，主要表现为缓慢进展的下肢无力，查体发现双侧锥体束征。头颅MRI可有皮质脊髓束走行区长T2异常信号，脊髓MRI示脊髓正常或变细，2例患者肌电图正常，2例轻度异常。4例患者均长期口服丁苯肽、维生素B1、甲钴胺、辅酶Q10治疗，随访显示该病进展缓慢。结论　对于以上运动神经元损害为主且进展缓慢的患者，肌电图（EMG）可轻度异常，在排除其它疾病后应高度怀疑PLS，仔细询问病史、细致的神经系统查体以及必要的电生理检查对于诊断至关重要。

Abstract:

Objective To investigate the clinical and imaging features and differential diagnosis of primary lateral sclerosis.[WTHZ]Methods and results We collected clinical and imaging data of 4 PLS patients, who were hospitalized in the Department of Neurology of Navy General Hospital from October 2012 to May 2017, including 3 men and 1 woman.They presented with slow progression of lower extremity weakness and bilateral pyramidal sign findings. The cranial MRI had corticospinal tract long T2 abnormal signal; spinal MRI showed normal or thin spinal cord. Twopatients hadnormal EMG, and 2 with mild abnormality. All patients were treated with Butylphthalide, Vitamin B1, Methylcobalamin，and Coenzyme Q10.The disease progressed slowly during follow-up.[WTHZ]Conclusion For patients with predominantly upper motor neuron damage and slow progression, EMG can be mildly abnormal. PLS should be highly suspected in the exclusion of other diseases.Careful history, detailed neurologic examination and necessary electrophysiological examination are critical.