TY - JOUR T1 - Primary lateral sclerosis: consensus diagnostic criteria JF - Journal of Neurology, Neurosurgery & Psychiatry JO - J Neurol Neurosurg Psychiatry SP - 373 LP - 377 DO - 10.1136/jnnp-2019-322541 VL - 91 IS - 4 AU - Martin R Turner AU - Richard J Barohn AU - Philippe Corcia AU - John K Fink AU - Matthew B Harms AU - Matthew C Kiernan AU - John Ravits AU - Vincenzo Silani AU - Zachary Simmons AU - Jeffrey Statland AU - Leonard H van den Berg AU - Delegates of the 2nd International PLS Conference AU - Hiroshi Mitsumoto Y1 - 2020/04/01 UR - http://jnnp.bmj.com/content/91/4/373.abstract N2 - 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. ER -