title: Targeted assessment of lower motor neuron burden is associated with survival in amyotrophic lateral sclerosis.
abstract: Estimating survival in amyotrophic lateral sclerosis (ALS) is challenging due to heterogeneity in clinical features of disease and a lack of suitable markers that predict survival. Our aim was to determine whether scoring of upper or lower motor neuron weakness is associated with survival. With this objective, 161 ALS subjects were recruited from two tertiary referral centres. Scoring of upper (UMN) and lower motor neuron (LMN) signs was performed, in addition to a brief questionnaire. Subjects were then followed until the censorship date. Univariate analysis was performed to identify variables associated with survival to either non-invasive ventilation (NIV) or death, which were then further characterized using Cox regression. Results showed that factors associated with reduced survival included older age, bulbar and respiratory involvement and shorter diagnostic delay (all p < 0.05). Whole body LMN score was strongly associated with time to NIV or death (p ≤0.001) whereas UMN scores were poorly associated with survival. In conclusion, our results suggest that, early in disease assessment and in the context of other factors (age, bulbar, respiratory status), the burden of LMN weakness provides an accurate estimate of outcome. Such a scoring system could predict prognosis, and thereby aid in selection of patients for clinical trials.
authors.affiliation: a Department of Neurology , Royal Brisbane and Women's Hospital , Herston , QLD .|b School of Medicine , The University of Queensland , St Lucia , QLD .
authors.forename: Matthew S
authors.initials: MS
authors.lastname: Devine
authors.affiliation: c QIMR Berghofer Medical Research Institute , Herston , QLD , and.
authors.forename: Emma
authors.initials: E
authors.lastname: Ballard
authors.affiliation: c QIMR Berghofer Medical Research Institute , Herston , QLD , and.
authors.forename: Peter
authors.initials: P
authors.lastname: O'Rourke
authors.affiliation: d Brain and Mind Research Institute, ForeFront , Sydney , NSW , Australia.
authors.forename: Matthew C
authors.initials: MC
authors.lastname: Kiernan
authors.affiliation: a Department of Neurology , Royal Brisbane and Women's Hospital , Herston , QLD .|b School of Medicine , The University of Queensland , St Lucia , QLD .
authors.forename: Pamela A
authors.initials: PA
authors.lastname: Mccombe
authors.affiliation: a Department of Neurology , Royal Brisbane and Women's Hospital , Herston , QLD .|b School of Medicine , The University of Queensland , St Lucia , QLD .
authors.forename: Robert D
authors.initials: RD
authors.lastname: Henderson
mesh_terms: D000367:Age Factors; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000175:diagnosis* / Q000401:mortality*; D004185:Disability Evaluation; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000145:classification / Q000473:pathology / Q000502:physiology*; D063087:Noninvasive Ventilation; D012044:Regression Analysis; D012120:Respiration Disorders / Q000209:etiology; D012189:Retrospective Studies; D011795:Surveys and Questionnaires
keywords: Biomarker; amyotrophic lateral sclerosis; clinical phenotype; prognostic; survival