PT  - JOURNAL ARTICLE
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
TI  - Neck weakness is a potent prognostic factor in sporadic amyotrophic lateral sclerosis patients
AID  - 10.1136/jnnp-2013-306020
DP  - 2013 Dec 01
TA  - Journal of Neurology, Neurosurgery & Psychiatry
PG  - 1365--1371
VI  - 84
IP  - 12
4099  - http://jnnp.bmj.com/content/84/12/1365.short
4100  - http://jnnp.bmj.com/content/84/12/1365.full
SO  - J Neurol Neurosurg Psychiatry2013 Dec 01; 84
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.