Primary lateral sclerosis (PLS) is a neurodegenerative disorder of the adult motor system. Characterised by a slowly progressive upper motor neuron syndrome, the diagnosis is clinical, after exclusion of structural, neurodegenerative and metabolic mimics. Differentiation of PLS from upper motor neuron-predominant forms of amyotrophic lateral sclerosis remains a significant challenge in the early symptomatic phase of both disorders, with ongoing debate as to whether they form a clinical and histopathological continuum. Current diagnostic criteria for PLS may be a barrier to therapeutic development, requiring long delays between symptom onset and formal diagnosis. While new technologies sensitive to both upper and lower motor neuron involvement may ultimately resolve controversies in the diagnosis of PLS, we present updated consensus diagnostic criteria with the aim of reducing diagnostic delay, optimising therapeutic trial design and catalysing the development of disease-modifying therapy.

中文翻译：

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原发性侧索硬化症：共识诊断标准。

原发性侧索硬化症（PLS）是成人运动系统的神经退行性疾病。以缓慢进行性上运动神经元综合征为特征，在排除结构，神经变性和代谢模拟物后，诊断为临床。在这两种疾病的症状早期，将PLS与上运动神经元为主的肌萎缩性侧索硬化相区别仍然是一项重大挑战，有关它们是否形成临床和组织病理学连续性的争论不断。PLS的当前诊断标准可能是治疗发展的障碍，需要在症状发作和正式诊断之间长期延迟。虽然对上，下运动神经元均敏感的新技术最终可以解决PLS诊断方面的争议，