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

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 prospe...

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Bibliographic Details
Published in:Journal of neurology 2016-06, Vol.263 (6), p.1129-1136
Main Authors: Yokoi, Daichi, Atsuta, Naoki, Watanabe, Hazuki, Nakamura, Ryoichi, Hirakawa, Akihiro, Ito, Mizuki, Watanabe, Hirohisa, Katsuno, Masahisa, Izumi, Yuishin, Morita, Mitsuya, Taniguchi, Akira, Oda, Masaya, Abe, Koji, Mizoguchi, Kouichi, Kano, Osamu, Kuwabara, Satoshi, Kaji, Ryuji, Sobue, Gen
Format: Article
Language:English
Subjects:
Activities of Daily Living
Adult
Age groups
Age of Onset
Aged
Aged, 80 and over
Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - epidemiology
Amyotrophic Lateral Sclerosis - physiopathology
Disease Progression
Female
Follow-Up Studies
Hospitals
Humans
Japan - epidemiology
Kaplan-Meier Estimate
Longitudinal Studies
Male
Medical prognosis
Medicine
Medicine & Public Health
Middle Aged
Neurology
Neuroradiology
Neurosciences
Original Communication
Prospective Studies
Respiration
Young Adult
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Summary: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  
ISSN:0340-5354
1432-1459
DOI:10.1007/s00415-016-8109-0