Patterns of Weakness, Classification of Motor Neuron Disease, and Clinical Diagnosis of Sporadic Amyotrophic Lateral Sclerosis.
Patterns of Weakness, Classification of Motor Neuron Disease, and Clinical Diagnosis of Sporadic Amyotrophic Lateral Sclerosis.
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DOI：
10.1016/j.ncl.2015.07.006
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发表时间：
2015-11
影响因子：
2.4
通讯作者：
Dimachkie MM
中科院分区：
文献类型：
--
作者：
Statland JM;Barohn RJ;McVey AL;Katz JS;Dimachkie MM
当接近疑似运动神经元疾病（MND）的患者时，检查时的无力模式有助于将MND与其他周围神经、神经肌肉接头或肌肉疾病区分开来。MND是在神经影像学或血清学检测没有其他异常的情况下，由电诊断检测结果支持的临床诊断。MND存在于一个频谱上：从纯的下运动神经元;到混合的上和下运动神经元;再到纯的上运动神经元变体以及局限于臂、腿或延髓区域的区域变体。肌萎缩侧索硬化症（ALS）是一种进行性混合性上下运动神经元疾病，最常见的是散发性（约85%），这总是致命的。FDA唯一批准的ALS治疗方法是利鲁唑，它可以使生命延长约3个月，而美沙芬/奎尼丁可以缓解假性延髓影响（不适当的大笑或哭泣）的症状。在这里，我们描述了一个模式的方法来识别运动神经元疾病，和散发性ALS的临床特征。
When approaching the patient with suspected motor neuron disease (MND) the pattern of weakness on exam helps distinguish MND from other diseases of peripheral nerves, the neuromuscular junction, or muscle. MND is a clinical diagnosis supported by findings on electrodiagnostic testing, in the absence of other abnormalities on neuroimaging or serological testing. MNDs exist on a spectrum: from a pure lower motor neuron; to mixed upper and lower motor neuron; to a pure upper motor neuron variant in addition to regional variants restricted to the arms, legs or bulbar region. Amyotrophic lateral sclerosis (ALS) is a progressive mixed upper and lower motor neuron disorder, most commonly sporadic (~85%), which is invariably fatal. The only FDA approved treatments for ALS are riluzole, which prolongs life by about 3 months, and dextromethorphan/quinidine which provides symptomatic relief for pseudobulbar affect (inappropriate bouts of laughter or crying). Here we describe a pattern approach to identifying motor neuron disease, and clinical features of sporadic ALS.