Background and objective: Primary lateral sclerosis (PLS) is a neurodegenerative disease characterized by progressive upper motor neuron dysfunction. Because PLS patients represent only 1 to 4% of patients with adult motor neuron diseases, there is limited information about the disease’s natural history. The objective of this study was to establish a large multicenter retrospective longitudinal registry of PLS patients seen at Northeast ALS Consortium (NEALS) sites to better characterize the natural progression of PLS. Methods: Clinical characteristics, electrophysiological findings, laboratory values, disease-related symptoms, and medications for symptom management were collected from PLS patients seen between 2000 and 2015. Results: The NEALS registry included data from 250 PLS patients. Median follow-up time was 3 years. The mean rate of functional decline measured by ALSFRS-R total score was −1.6 points/year (SE:0.24, n = 124); the mean annual decline in vital capacity was −3%/year (SE:0.55, n = 126). During the observational period, 18 patients died, 17 patients had a feeding tube placed and 7 required permanent assistive ventilation. Conclusions: The NEALS PLS Registry represents the largest available aggregation of longitudinal clinical data from PLS patients and provides a description of expected natural disease progression. Data from the registry will be available to the PLS community and can be leveraged to plan future clinical trials in this rare disease.

Original languageEnglish
Pages (from-to)74-81
Number of pages8
JournalAmyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Issue numberS1
StatePublished - 2020
Externally publishedYes


FundersFunder number
Amylyx Therapeutics
Fund for Primary Lateral Sclerosis Research and Care at Massachusetts General Hospital
MGH ALS Therapy Fund
Minnesota Regenerative Medicine PartnershipRMM 11215 CT002
Otsuka America, Inc.
Revalesio Inc.
U.S. Department of Veterans Affairs Office of Research and DevelopmentIK2CX001595-02
National Institutes of HealthCA 211887
Centers for Disease Control and Prevention
National Institute of Environmental Health SciencesK23ES027221
Amyotrophic Lateral Sclerosis Association
Harvard NeuroDiscovery Center
Agency for Toxic Substances and Disease Registry
American Academy of Neurology
Salah Foundation
Spastic Paraplegia Foundation


    • PLS
    • disability
    • outcome measures
    • survival
    • upper motor neurons


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