RT Journal Article SR Electronic T1 Neck weakness is a potent prognostic factor in sporadic amyotrophic lateral sclerosis patients JF Journal of Neurology, Neurosurgery & Psychiatry JO J Neurol Neurosurg Psychiatry FD BMJ Publishing Group Ltd SP 1365 OP 1371 DO 10.1136/jnnp-2013-306020 VO 84 IS 12 A1 Ryoichi Nakamura A1 Naoki Atsuta A1 Hazuki Watanabe A1 Akihiro Hirakawa A1 Hirohisa Watanabe A1 Mizuki Ito A1 Jo Senda A1 Masahisa Katsuno A1 Fumiaki Tanaka A1 Yuishin Izumi A1 Mitsuya Morita A1 Kotaro Ogaki A1 Akira Taniguchi A1 Ikuko Aiba A1 Koichi Mizoguchi A1 Koichi Okamoto A1 Kazuko Hasegawa A1 Masashi Aoki A1 Akihiro Kawata A1 Koji Abe A1 Masaya Oda A1 Masaaki Konagaya A1 Takashi Imai A1 Masanori Nakagawa A1 Shoji Tsuji A1 Ryuji Kaji A1 Imaharu Nakano A1 Gen Sobue YR 2013 UL http://jnnp.bmj.com/content/84/12/1365.abstract AB Objective To clarify the emergence of muscle weakness in regions of the body that affect survival, and deterioration in activities of daily living (ADL) in amyotrophic lateral sclerosis (ALS) patients. Methods We conducted a multicentre-based prospective cohort study of patients with ALS. We enrolled 401 sporadic patients with ALS. Death or the introduction of invasive ventilation was defined as the primary endpoint, and the time to five clinical markers of ADL deterioration associated with bulbar paralysis or limb weakness were defined as ADL milestones. Muscle weakness was assessed in the neck flexor muscles; the bilateral abductors of the shoulders; the bilateral wrist extensor muscles; the bilateral flexor muscles of the hips; and the bilateral ankle dorsiflexion muscles. We performed Cox proportional hazards regression analyses for the primary endpoint and the five ADL milestones, adjusting for known covariate prognostic factors for ALS. Results The Medical Research Council (MRC) score for the neck flexors was the most significant prognostic factor for the primary endpoint (HR 0.74, p<0.001), loss of speech (HR 0.66, p<0.001), and loss of swallowing function (HR 0.73, p<0.001), and was one of the significant prognostic factors for loss of upper limb function, difficulty turning in bed, and loss of walking ability (p=0.001, 0.002, and 0.008, respectively). The MRC score for the neck flexors was also a significant prognostic factor for covariates of the previously reported prognostic factors. Conclusions Neck weakness is an independent prognostic factor for survival and deterioration in ADL in Patients with ALS.