title: Prediction of prognosis of ALS: Importance of active denervation findings of the cervical-upper limb area and trunk area.
abstract: Amyotrophic lateral sclerosis (ALS) is a motor neuron disease characterized by serious muscle atrophy and weakness. The purpose of this study was to find prognostic factors in patients with mild ALS using application forms for the Specified Disease Treatment Research Program in Japan. We classified ALS as mild, moderate and severe. The subjects consisted of 363 patients with mild ALS who underwent needle electromyography at registration and were followed for more than one year. Time to progression to severe ALS and time to deterioration of activities of daily living such as speech dysfunction, upper limb dysfunction, and walking disability were used as outcomes. Cox proportional hazards model analysis was performed to identify prognostic factors. Of the patients with initially mild ALS, 38.3% (139/363) had progressed severe ALS at the last follow-up. In multivariate analysis of time to progression to severe ALS, bulbar onset (hazard ratio [95% confidence interval]: 1.68 [1.13-2.49], p = 0.010), tongue atrophy (1.69 [1.14-2.51], p = 0.009), dyspnea (1.57 [1.02-2.41], p = 0.042) and active denervation findings (ADFs) of the cervical-upper limb area (1.81 [1.25-2.63], p = 0.002) emerged as prognostic factors. Furthermore ADFs in the trunk area were prognostic factors for upper limb dysfunction and walking disability (1.72 [1.05-2.81], p = 0.031, and 1.97 [1.09-3.59], p = 0.026). In conclusion ADFs of the cervical-upper limb area and trunk area were prognostic factors in ALS patients.
authors.affiliation: Department of Immunotherapeutics, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan ; Center for Public Health Informatics, National Institute of Public Health, Saitama, Japan.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Sato
authors.affiliation: Translational Research Informatics Center, Foundation for Biomedical Research and Innovation, Kobe, Japan.
authors.forename: Eiji
authors.initials: E
authors.lastname: Nakatani
authors.affiliation: Division of Neurology, Department of Brain and Neurosciences, School of Medicine, Faculty of Medicine, Tottori University, Yonago, Japan.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Watanabe
authors.affiliation: Translational Research Informatics Center, Foundation for Biomedical Research and Innovation, Kobe, Japan.
authors.forename: Masanori
authors.initials: M
authors.lastname: Fukushima
authors.affiliation: Division of Neurology, Department of Brain and Neurosciences, School of Medicine, Faculty of Medicine, Tottori University, Yonago, Japan.
authors.forename: Kenji
authors.initials: K
authors.lastname: Nakashima
authors.affiliation: Department of Immunotherapeutics, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.
authors.forename: Mari
authors.initials: M
authors.lastname: Kannagi
authors.affiliation: Department of Health Crisis Management, National Institute of Public Health, Saitama, Japan.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Kanatani
authors.affiliation: Center for Public Health Informatics, National Institute of Public Health, Saitama, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Mizushima
keywords: Amyotrophic lateral sclerosis; bulbar onset; denervation findings; needle electromyography; prognostic factors