Pure primary lateral sclerosis - Case reports
Pure primary lateral sclerosis - Case reports
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DOI：
10.1016/j.clineuro.2007.12.002
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发表时间：
2008-04-01
影响因子：
1.9
通讯作者：
Szczudlik, Andrzej
中科院分区：
文献类型：
--
作者：
Tomik, Barbara;Zur, Karnila. Anna;Szczudlik, Andrzej
原发性侧索硬化症（PLS）是否是一个独特的病理实体，或者它是否代表了运动神经元疾病（MND）的一个连续谱的一端，目前仍有争议。在这份报告中，我们提出了四个PLS患者从症状发作（1990-1999）到2007年1月观察。他们都只有上运动神经元（UMN）的迹象和缓慢的临床进展。三名患者出现痉挛性下肢轻瘫。痉挛是主要的临床特征，表现为腱反射亢进、阵挛和巴宾斯基征。1例患者在发病时出现痉挛性构音障碍。在我们报告的系列中，从症状发作到现在的平均病程为11.5年。所有四个PLS患者没有开发下运动神经元（LMN）的迹象，在这段时间的observation.This前瞻性分析，我们的PLS系列与其他研究的数据表明，纯PLS的情况下，有一个长期的病程与高水平的独立性相比，其他MND。(c)2008 Elsevier B. V.保留所有权利。
There is still a debate whether primary lateral sclerosis (PLS) is a distinct pathological entity or whether it represents one end of a continuous spectrum of motor neuron disease (MND).In this report we present four PLS patients who have been observed from the time of symptom onset (1990-1999) through January 2007. All of them have had only upper motor neuron (UMN) signs and slow clinical progression. Three patients have been presented with spastic paraparesis. Spasticity was the main clinical feature in demonstrated cases with hyperactive deep tendon reflexes, clonus, and Babinski signs. One patient was presented with spastic dysarthria at the disease onset. Mean disease duration, measured from symptom onset to the present, was 11.5 years in our reported series. All four PLS patients had not developed lower motor neuron (LMN) signs during this time of observation.This prospective analysis of our PLS series is in agreement with data from other studies suggesting that pure PLS cases have a prolonged course of disease with a high level of independence when compared to other MND. (c) 2008 Elsevier B.V. All rights reserved.