@article {Nakamura1365, author = {Ryoichi Nakamura and Naoki Atsuta and Hazuki Watanabe and Akihiro Hirakawa and Hirohisa Watanabe and Mizuki Ito and Jo Senda and Masahisa Katsuno and Fumiaki Tanaka and Yuishin Izumi and Mitsuya Morita and Kotaro Ogaki and Akira Taniguchi and Ikuko Aiba and Koichi Mizoguchi and Koichi Okamoto and Kazuko Hasegawa and Masashi Aoki and Akihiro Kawata and Koji Abe and Masaya Oda and Masaaki Konagaya and Takashi Imai and Masanori Nakagawa and Shoji Tsuji and Ryuji Kaji and Imaharu Nakano and Gen Sobue}, editor = {Yuasa, Tatsuhiko and Kano, Takahiro and Sasaki, Hidenao and Kato, Masaaki and Ishihara, Tomohiko and Nishizawa, Masatoyo and Ikeda, Masaki and Shibuya, Kazumoto and Kuwabara, Satoshi and Hayashi, Hideaki and Takahashi, Yuji and Tomiyama, Hiroyuki and Hattori, Nobutaka and Aizawa, Hitoshi and Kano, Osamu and Iwasaki, Yasuo and Nagasaka, Takamura and Takiyama, Yoshihisa and Sakai, Motoko and Shiga, Kensuke and Yamashita, Hirofumi and Takahashi, Ryosuke and Fujita, Takuji and Yamashita, Toru and Hiji, Masanori and Watanabe, Yasuhiro and Nakashima, Kenji and Hayashi, Shintaro and Kira, Jun-ichi}, title = {Neck weakness is a potent prognostic factor in sporadic amyotrophic lateral sclerosis patients}, volume = {84}, number = {12}, pages = {1365--1371}, year = {2013}, doi = {10.1136/jnnp-2013-306020}, publisher = {BMJ Publishing Group Ltd}, abstract = {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.}, issn = {0022-3050}, URL = {https://jnnp.bmj.com/content/84/12/1365}, eprint = {https://jnnp.bmj.com/content/84/12/1365.full.pdf}, journal = {Journal of Neurology, Neurosurgery \& Psychiatry} }