Fullam Timothy, Statland Jeffrey
Department of Neurology, University of Kansas Medical Center, Kansas, KS 66160, USA.
Brain Sci. 2021 May 11;11(5):611. doi: 10.3390/brainsci11050611.
Following the exclusion of potentially reversible causes, the differential for those patients presenting with a predominant upper motor neuron syndrome includes primary lateral sclerosis (PLS), hereditary spastic paraplegia (HSP), or upper motor neuron dominant ALS (UMNdALS). Differentiation of these disorders in the early phases of disease remains challenging. While no single clinical or diagnostic tests is specific, there are several developing biomarkers and neuroimaging technologies which may help distinguish PLS from HSP and UMNdALS. Recent consensus diagnostic criteria and use of evolving technologies will allow more precise delineation of PLS from other upper motor neuron disorders and aid in the targeting of potentially disease-modifying therapeutics.
在排除潜在的可逆性病因后，那些以主要的上运动神经元综合征为表现的患者的鉴别诊断包括原发性侧索硬化症（PLS）、遗传性痉挛性截瘫（HSP）或上运动神经元为主型肌萎缩侧索硬化症（UMNdALS）。在疾病的早期阶段区分这些病症仍然具有挑战性。虽然没有单一的临床或诊断测试具有特异性，但有几种正在发展的生物标志物和神经影像学技术可能有助于将PLS与HSP和UMNdALS区分开来。最近的共识诊断标准和不断发展的技术的应用将使PLS与其他上运动神经元疾病的区分更加精确，并有助于靶向潜在的疾病修饰疗法。
