Onset of age, site and respiratory symptoms are strongly associated with respiratory decline in sporadic amyotrophic lateral sclerosis: a long-term longitudinal study
Objective The objective of this study is to identify factors influencing progression of respiratory decline from the onset of neurological symptoms to respiratory failure in patients with amyotrophic lateral sclerosis (ALS).Methods In 100 patients with sporadic ALS, %vital capacity (%VC) was continu...
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| Format: | Article |
| Language: | English |
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BMJ Publishing Group
2024-12-01
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| Series: | BMJ Neurology Open |
| Online Access: | https://neurologyopen.bmj.com/content/6/2/e000829.full |
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| author | Gen Sobue Shin-ichi Terao Yasunobu Nosaki Atsunori Murao Ryota Torii Nanayo Ogawa Naofumi Miura Yousuke Sasaki |
| author_facet | Gen Sobue Shin-ichi Terao Yasunobu Nosaki Atsunori Murao Ryota Torii Nanayo Ogawa Naofumi Miura Yousuke Sasaki |
| author_sort | Gen Sobue |
| collection | DOAJ |
| description | Objective The objective of this study is to identify factors influencing progression of respiratory decline from the onset of neurological symptoms to respiratory failure in patients with amyotrophic lateral sclerosis (ALS).Methods In 100 patients with sporadic ALS, %vital capacity (%VC) was continuously measured from the first visit to the respiratory endpoint (REP). Cox proportional hazards model identified factors influencing the duration from onset of ALS to REP (Onset-REP). We performed Kaplan-Meier survival curve analysis for onset-REP according to identified factors.Results Onset sites were the upper limb (U-ALS), lower limb (L-ALS), bulbar paralysis (B-ALS) and respiratory paralysis (R-ALS) in 37, 19, 32 and 12 patients, respectively. Duration from the onset of ALS to the onset of respiratory symptoms (Onset-Rp) and REP (Onset-REP) was 16.1 (SD 12.1) and 24.9 months (SD 14.6), respectively. Multivariate analysis revealed that age at onset, site of onset, Onset-Rp and %VC decline rate significantly influenced Onset-REP duration. Elderly patients had a significantly shorter Onset-REP duration. Onset-REP duration did not significantly differ between patients with U-ALS and L-ALS, but was longer in these patients than in those with B-ALS and R-ALS. Onset-REP duration was positively associated with Onset-Rp duration. The average monthly %VC decline rate was −5.6% (SD 3.3). Age at onset, onset site and Onset-Rp duration significantly influenced the %VC decline rate.Conclusions Our findings revealed strong and independent patient-specific factors that influence the Onset-REP duration and the %VC decline rate in patients with ALS. These could inform future clinical trials and interventions considering the respiratory function and natural history of patients with ALS. |
| format | Article |
| id | doaj-art-a692c9c7903a47ed85427c9cdd8c3004 |
| institution | Kabale University |
| issn | 2632-6140 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | BMJ Publishing Group |
| record_format | Article |
| series | BMJ Neurology Open |
| spelling | doaj-art-a692c9c7903a47ed85427c9cdd8c30042025-01-03T10:35:14ZengBMJ Publishing GroupBMJ Neurology Open2632-61402024-12-016210.1136/bmjno-2024-000829Onset of age, site and respiratory symptoms are strongly associated with respiratory decline in sporadic amyotrophic lateral sclerosis: a long-term longitudinal studyGen Sobue0Shin-ichi Terao1Yasunobu Nosaki2Atsunori Murao3Ryota Torii4Nanayo Ogawa5Naofumi Miura6Yousuke Sasaki7Aichi Medical University, Nagakute, Aichi, JapanNeurology, Mitaki General Hospital, Yokkaichi, Mie, JapanNeurology, Toyohashi Municipal Hospital, Toyohashi, Aichi, JapanNeurology, Higashinagoya National Hospital, Nagoya, Aichi, JapanNeurology, Kasugai Municipal Hospital, Kasugai, Aichi, JapanRehabilitation, Mitaki General Hospital, Yokkaichi, Mie, JapanAoki Internal Medicine, Kuwana, Mie, JapanSatista Co, Kyoto, JapanObjective The objective of this study is to identify factors influencing progression of respiratory decline from the onset of neurological symptoms to respiratory failure in patients with amyotrophic lateral sclerosis (ALS).Methods In 100 patients with sporadic ALS, %vital capacity (%VC) was continuously measured from the first visit to the respiratory endpoint (REP). Cox proportional hazards model identified factors influencing the duration from onset of ALS to REP (Onset-REP). We performed Kaplan-Meier survival curve analysis for onset-REP according to identified factors.Results Onset sites were the upper limb (U-ALS), lower limb (L-ALS), bulbar paralysis (B-ALS) and respiratory paralysis (R-ALS) in 37, 19, 32 and 12 patients, respectively. Duration from the onset of ALS to the onset of respiratory symptoms (Onset-Rp) and REP (Onset-REP) was 16.1 (SD 12.1) and 24.9 months (SD 14.6), respectively. Multivariate analysis revealed that age at onset, site of onset, Onset-Rp and %VC decline rate significantly influenced Onset-REP duration. Elderly patients had a significantly shorter Onset-REP duration. Onset-REP duration did not significantly differ between patients with U-ALS and L-ALS, but was longer in these patients than in those with B-ALS and R-ALS. Onset-REP duration was positively associated with Onset-Rp duration. The average monthly %VC decline rate was −5.6% (SD 3.3). Age at onset, onset site and Onset-Rp duration significantly influenced the %VC decline rate.Conclusions Our findings revealed strong and independent patient-specific factors that influence the Onset-REP duration and the %VC decline rate in patients with ALS. These could inform future clinical trials and interventions considering the respiratory function and natural history of patients with ALS.https://neurologyopen.bmj.com/content/6/2/e000829.full |
| spellingShingle | Gen Sobue Shin-ichi Terao Yasunobu Nosaki Atsunori Murao Ryota Torii Nanayo Ogawa Naofumi Miura Yousuke Sasaki Onset of age, site and respiratory symptoms are strongly associated with respiratory decline in sporadic amyotrophic lateral sclerosis: a long-term longitudinal study BMJ Neurology Open |
| title | Onset of age, site and respiratory symptoms are strongly associated with respiratory decline in sporadic amyotrophic lateral sclerosis: a long-term longitudinal study |
| title_full | Onset of age, site and respiratory symptoms are strongly associated with respiratory decline in sporadic amyotrophic lateral sclerosis: a long-term longitudinal study |
| title_fullStr | Onset of age, site and respiratory symptoms are strongly associated with respiratory decline in sporadic amyotrophic lateral sclerosis: a long-term longitudinal study |
| title_full_unstemmed | Onset of age, site and respiratory symptoms are strongly associated with respiratory decline in sporadic amyotrophic lateral sclerosis: a long-term longitudinal study |
| title_short | Onset of age, site and respiratory symptoms are strongly associated with respiratory decline in sporadic amyotrophic lateral sclerosis: a long-term longitudinal study |
| title_sort | onset of age site and respiratory symptoms are strongly associated with respiratory decline in sporadic amyotrophic lateral sclerosis a long term longitudinal study |
| url | https://neurologyopen.bmj.com/content/6/2/e000829.full |
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