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Research paper
Neck weakness is a potent prognostic factor in sporadic amyotrophic lateral sclerosis patients
  1. Ryoichi Nakamura1,
  2. Naoki Atsuta1,
  3. Hazuki Watanabe1,
  4. Akihiro Hirakawa2,
  5. Hirohisa Watanabe1,
  6. Mizuki Ito1,
  7. Jo Senda1,
  8. Masahisa Katsuno1,
  9. Fumiaki Tanaka3,
  10. Yuishin Izumi4,
  11. Mitsuya Morita5,
  12. Kotaro Ogaki6,
  13. Akira Taniguchi7,
  14. Ikuko Aiba8,
  15. Koichi Mizoguchi9,
  16. Koichi Okamoto10,
  17. Kazuko Hasegawa11,
  18. Masashi Aoki12,
  19. Akihiro Kawata13,
  20. Koji Abe14,
  21. Masaya Oda15,
  22. Masaaki Konagaya16,
  23. Takashi Imai17,
  24. Masanori Nakagawa18,
  25. Shoji Tsuji19,
  26. Ryuji Kaji4,
  27. Imaharu Nakano5,
  28. Gen Sobue1
  1. 1Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan
  2. 2Biostatistics Section, Center for Advanced Medicine and Clinical Research, Nagoya University Graduate School of Medicine, Nagoya, Japan
  3. 3Department of Neurology, Yokohama City University Graduate School of Medicine, Yokohama, Japan
  4. 4Department of Clinical Neuroscience, Institute of Health Biosciences, University of Tokushima Graduate School, Tokushima, Japan
  5. 5Division of Neurology, Department of Internal Medicine, Jichi Medical University, Tochigi, Japan
  6. 6Department of Neurology, Juntendo University School of Medicine, Tokyo, Japan
  7. 7Department of Neurology, Mie University Graduate School of Medicine, Tsu, Japan
  8. 8Department of Neurology, National Hospital Organization Higashinagoya National Hospital, Nagoya, Japan
  9. 9Department of Neurology, Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan
  10. 10Department of Neurology, Gunma University Graduate School of Medicine, Gunma, Japan
  11. 11Division of Neurology, National Hospital Organization, Sagamihara National Hospital, Sagamihara, Japan
  12. 12Department of Neurology, Tohoku University School of Medicine, Sendai, Japan
  13. 13Department of Neurology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan
  14. 14Department of Neurology, Okayama University Graduate School of Medicine, Okayama, Japan
  15. 15Department of Neurology, Vihara Hananosato Hospital, Miyoshi, Japan
  16. 16Department of Neurology, National Hospital Organization, Suzuka National Hospital, Suzuka, Japan
  17. 17Division of Neurology, National Hospital Organization, Miyagi National Hospital, Miyagi, Japan
  18. 18Director of North Medical Center, Kyoto Prefectural University of Medicine, Kyoto, Japan
  19. 19Department of Neurology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
  1. Correspondence to Professor Gen Sobue, Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466–8550 Japan; sobueg{at}


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.

  • ALS
  • Neuroepidemiology
  • Clinical Neurology

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