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Muscle Nerve ; 61(2): 163-172, 2020 02.
Article in English | MEDLINE | ID: mdl-31758557

ABSTRACT

INTRODUCTION: Our research aim was to develop a novel clinimetric scale sensitive enough to detect disease progression in primary lateral sclerosis (PLS). METHODS: A prototype of the PLS Functional Rating Scale (PLSFRS) was generated. Seventy-seven participants with PLS were enrolled and evaluated at 21 sites that comprised the PLSFRS study group. Participants were assessed using the PLSFRS, Neuro-Quality of Life (QoL), Schwab-England Activities of Daily Living (ADL), and the Clinical Global Impression of Change scales. Participants completed telephone assessments at 12, 24, and 48 weeks after enrollment. RESULTS: The PLSFRS demonstrated internal consistency as well as intrarater, interrater, telephone test-retest reliability, and construct validity. Significant changes in disease progression were detected at 6 and 12 months; changes measured by the PLSFRS vs the ALSFRS-R were significantly higher. DISCUSSION: The PLSFRS is a valid tool to assess the natural history of PLS in a shorter study period.


Subject(s)
Motor Neuron Disease/diagnosis , Activities of Daily Living , Adult , Aged , Caregivers , Certification , Disease Progression , Female , Follow-Up Studies , Humans , Male , Middle Aged , Motor Neuron Disease/physiopathology , Motor Neuron Disease/psychology , Observer Variation , Quality of Life , Reproducibility of Results , Telephone
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