Singer Mike A, Kojan Suleiman, Barohn Richard J, Herbelin Laura, Nations Sharon P, Trivedi Jaya R, Jackson Carlayne E, Burns Dennis K, Boyer Philip J, Wolfe Gil I
From the Departments of *Neurology and daggerPathology, University of Texas Southwestern Medical Center, Dallas, TX; double daggerDepartment of Neurology, University of Kansas Medical Center, Kansas City, KS; and section signDepartment of Neurology, University of Texas Health Science Center, San Antonio, TX.
J Clin Neuromuscul Dis. 2005 Sep;7(1):1-9. doi: 10.1097/01.cnd.0000176974.61136.45.
: The objective of this study was to characterize the clinical features and natural history of primary lateral sclerosis (PLS).
: PLS is a motor neuron disorder defined by corticospinal and corticobulbar tract dysfunction without clinically significant lower motor neuron involvement.
: We collected data from 25 patients with PLS seen in 2 academic neurology departments over a 5-year period.
: The PLS population represented approximately 3% of acquired motor neuron disease cases seen during that period. Twenty-three patients (92%) presented with lower limb weakness, spasticity, or difficulty with ambulation. None presented with upper limb symptoms. Eleven patients (44%) developed bulbar symptoms. All patients had hyperreflexia and increased muscle tone. Muscle weakness was observed in 15 patients (60%) and tended to be mild and asymmetric. Needle electromyography (EMG) was normal or showed only fasciculations in 15 patients (60%); 10 patients had features of mild active denervation, consisting of fibrillation or positive sharp wave potentials, but the extent of these findings did not satisfy World Federation of Neurology electrophysiological criteria for the diagnosis of amyotrophic lateral sclerosis. Fourteen patients (52%) continued independent ambulation. Of the 10 patients with active denervation on EMG, 6 (60%) required a walker, scooter, or wheelchair at a mean follow up of 6.2 years. There were no fatalities over the 5-year period.
: Our experience supports the observation that PLS progresses more slowly than other forms of acquired motor neuron disease, particularly amyotrophic lateral sclerosis. Follow-up data suggest that patients with active denervation changes develop greater disability.
本研究的目的是描述原发性侧索硬化症（PLS）的临床特征和自然病程。
PLS是一种运动神经元疾病，其定义为皮质脊髓束和皮质延髓束功能障碍，而临床上无明显的下运动神经元受累。
我们收集了在5年期间于2个学术性神经科就诊的25例PLS患者的数据。
PLS患者约占该时期获得性运动神经元疾病病例的3%。23例患者（92%）出现下肢无力、痉挛或行走困难。无一例出现上肢症状。11例患者（44%）出现延髓症状。所有患者均有反射亢进和肌张力增高。15例患者（60%）观察到肌肉无力，且往往较轻且不对称。针极肌电图（EMG）在15例患者（60%）中正常或仅显示肌束震颤；10例患者有轻度失神经活动的特征，包括纤颤或正锐波电位，但这些发现的程度不符合世界神经病学联合会诊断肌萎缩侧索硬化症的电生理标准。14例患者（52%）仍能独立行走。在EMG显示有失神经活动的10例患者中，6例（60%）在平均6.2年的随访中需要使用助行器、踏板车或轮椅。在5年期间无死亡病例。
我们的经验支持以下观察结果，即PLS的进展比其他形式的获得性运动神经元疾病，尤其是肌萎缩侧索硬化症更为缓慢。随访数据表明，有失神经活动改变的患者残疾程度更高。
