TY - JOUR 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 SP - 1365 LP - 1371 DO - 10.1136/jnnp-2013-306020 VL - 84 IS - 12 AU - Ryoichi Nakamura AU - Naoki Atsuta AU - Hazuki Watanabe AU - Akihiro Hirakawa AU - Hirohisa Watanabe AU - Mizuki Ito AU - Jo Senda AU - Masahisa Katsuno AU - Fumiaki Tanaka AU - Yuishin Izumi AU - Mitsuya Morita AU - Kotaro Ogaki AU - Akira Taniguchi AU - Ikuko Aiba AU - Koichi Mizoguchi AU - Koichi Okamoto AU - Kazuko Hasegawa AU - Masashi Aoki AU - Akihiro Kawata AU - Koji Abe AU - Masaya Oda AU - Masaaki Konagaya AU - Takashi Imai AU - Masanori Nakagawa AU - Shoji Tsuji AU - Ryuji Kaji AU - Imaharu Nakano AU - Gen Sobue Y1 - 2013/12/01 UR - http://jnnp.bmj.com/content/84/12/1365.abstract N2 - 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. ER -