Journal of Neurology, Neurosurgery & Psychiatry
Fecha de publicación: 6 February 2020
DOI: http://dx.doi.org/10.1136/jnnp-2019-322541
Autores: Martin R Turner, Richard J Barohn, Philippe Corcia, John K Fink, Matthew B Harms, Matthew C Kiernan, John Ravits, Vincenzo Silani, Zachary Simmons,Jeffrey Statland, Leonard H van den Berg, Delegates of the 2nd International PLS Conference, Hiroshi Mitsumoto
Background: 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.