Age of onset differentially influences the progression of regional dysfunction in sporadic amyotrophic lateral sclerosis

Daichi Yokoi, Naoki Atsuta, Hazuki Watanabe, Ryoichi Nakamura, Akihiro Hirakawa, Mizuki Ito, Hirohisa Watanabe, Masahisa Katsuno, Yuishin Izumi, Mitsuya Morita, Akira Taniguchi, Masaya Oda, Koji Abe, Kouichi Mizoguchi, Osamu Kano, Satoshi Kuwabara, Ryuji Kaji, Gen Sobue

Research output: Contribution to journalArticlepeer-review

21 Citations (Scopus)


The clinical courses of sporadic amyotrophic lateral sclerosis (ALS) show extensive variability. Our objective was to elucidate how age of onset influences the progression of regional symptoms and functional losses in sporadic ALS. We included 648 patients with sporadic ALS from a multicenter prospective ALS cohort. We investigated the distribution of initial symptoms and analyzed the time from onset to events affecting activities of daily living (ADL) as well as the longitudinal changes in each regional functional rating score among four groups with different ages of onset. The frequencies of dysarthria and dysphagia as initial symptoms were higher in the older age groups, whereas weakness of upper limbs was the most common initial symptom in the youngest age group. The survival times and the times from onset to loss of speech and swallowing were significantly shorter in the older age group (p < 0.001), although the times from onset to loss of upper limb function were not significantly different among the age groups. According to joint modeling analysis, the bulbar score declined faster in the older age groups (<50 vs. 60–69 years: p = 0.029, <50 vs. ≥70 years: p < 0.001), whereas there was no significant correlation between the age of onset and decline in the upper limb score. Our results showed that age of onset had a significant influence on survival time and the progression of bulbar symptoms, but had no influence on upper limb function in sporadic ALS.

Original languageEnglish
Pages (from-to)1129-1136
Number of pages8
JournalJournal of Neurology
Issue number6
Publication statusPublished - Jun 1 2016


  • Age of onset
  • Bulbar upper limb function
  • Sporadic ALS

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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