Limb-onset amyotrophic lateral sclerosis patients visiting orthopedist show a longer time-to-diagnosis since symptom onset

Kano, Osamu; Iwamoto, Konosuke; Ito, H.; Kawase, Yuji; Cridebring, Derek; Ikeda, Ken; Iwasaki, Yasuo

doi:10.1186/1471-2377-13-19

Cited by 26 publications

(24 citation statements)

References 17 publications

(23 reference statements)

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“…Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disorder characterized by rapidly progressive paralysis and death due to respiratory failure, typically within 2–3 years of disease onset 1 , 2 . Approximately 10% of ALS are familial, whereas the remaining are sporadic.…”

Section: Introductionmentioning

confidence: 99%

Neuronal apoptosis inhibitory protein is implicated in amyotrophic lateral sclerosis symptoms

Kano

Tanaka²,

Kanno

et al. 2018

Sci Rep

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The delineation of the molecular pathology underlying amyotrophic lateral sclerosis (ALS) is being hampered by the lack of suitable biomarkers. We have previously reported that bromocriptine upregulates the endogenous antioxidative factor, neuronal apoptosis inhibitory protein (NAIP), sustains motor function and slows disease progression in ALS patients, implying the NAIP’s implication in ALS. Here, we aimed to verify a correlation of NAIP level with disease progression in ALS patients. The amount of NAIP in mononuclear cells (MNC) from peripheral blood from ALS patients (n = 18) and the age matched healthy controls (n = 12) was validated by NAIP-Dot blotting. Notably, the MNC-NAIP level in ALS patients (0.62 ± 0.29 ng) was nearly half of that in the healthy controls (1.34 ± 0.61 ng, P = 0.0019). Furthermore, the MNC-NAIP level in ALS patients and their ALS Functional Rating Scale-Revised (ALSFRS-R) score were evaluated through 1 year. Regression analysis of the MNC-NAIP vs ALSFRS-R indicated that a higher amount of MNC-NAIP was associated with a smaller change in ALSFRS-R at 12 months (R2 = 0.799; P = 0.016), suggesting that a progressive increment of the MNC-NAIP led to slower ALS progression. Our present report implies that NAIP will have broad implications for ALS symptoms as a risk factor and a promising prognostic biomarker.

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Section: Introductionmentioning

confidence: 99%

Neuronal apoptosis inhibitory protein is implicated in amyotrophic lateral sclerosis symptoms

Kano

Tanaka²,

Kanno

et al. 2018

Sci Rep

Self Cite

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“…The average age of the onset for ALS was around 60 years old. It was expected that caregivers would often be partners, and there was no marked age difference among the respondents ( 3 ). In contrast, most MEs were in their 20-40s, showing a significant difference.…”

Section: Discussionmentioning

confidence: 99%

“…Hospitals have reported a shortage of key equipment, including ventilators and personal protective equipment for medical staff needed to care for critically ill patients with the disease. Amyotrophic lateral sclerosis (ALS) is a progressive, incurable, and fatal neurodegenerative disease with an average life expectancy of 3 to 5 years after its onset (2,3). While there is no treatment to stop its progression, a tracheostomy with invasive ventilation (TIV) can prolong the survival (4).…”

Section: Introductionmentioning

confidence: 99%

Contradictory Responses to the COVID-19 Pandemic in Amyotrophic Lateral Sclerosis Patients and Their Families and Caregivers in Japan

et al. 2021

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Objective The coronavirus disease 2019 (COVID-19) pandemic has resulted in a shortage of medical resources, including ventilators, personal protective equipment, medical staff, and hospital beds. We investigated the impact of COVID-19 in amyotrophic lateral sclerosis (ALS) patients, their families, caregivers, and medical experts. Methods We conducted a nationwide ALS webinar about COVID-19 in May 2020 and sent a questionnaire to those enrolled. Results A total of 135 participants (31 ALS patients; 23 families and caregivers of ALS patients; 81 medical experts) responded to this cross-sectional self-report questionnaire. The results showed that tracheostomy and invasive ventilation (TIV) was used in 22.6% of ALS patients, whereas 77.4% of ALS patients were not under TIV. Among non-TIV patients (n=24), 79.2% did not want TIV in the future. However, 47.4% of non-TIV patients not wanting a tracheostomy in advanced stages replied that they would want an emergency tracheostomy if they developed COVID-19-related pneumonia. These results suggest that ALS patients may be receptive to emergency treatments for reasons other than ALS. In addition, approximately half of the ALS patients agreed with the policy of not ventilating the elderly or ALS patients in case of a ventilator shortage. Furthermore, compared with medical experts, few ALS patients reported that the chance for ALS patients to obtain work was higher due to the increasing availability of remote work. Conclusions This survey indicates that the COVID-19 pandemic might be associated with increased distress about access to care and work, inducing contradictory responses and potential hopelessness among ALS patients.

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“…Nevertheless, bias from this source is likely modest because death certificates have been estimated to accurately identify 70%-90% of cases of ALS or motor neuron diseases (22,35,36), and the median survival of ALS patients after diagnosis is relatively short (1.5-3 years) (1), making death a reasonable substitute for incidence. Because ALS patients usually develop symptoms 1 year before ALS diagnosis (37,38), diet before diagnosis may have been affected by early symptoms of ALS in these cohorts. However, this is unlikely to be a source of large bias because results were similar for the lagged and the main analyses.…”

Section: Discussionmentioning

confidence: 99%

Dietary Fiber and Amyotrophic Lateral Sclerosis: Results From 5 Large Cohort Studies

Fondell

O’Reilly

Fitzgerald

et al. 2014

American Journal of Epidemiology

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Amyotrophic lateral sclerosis (ALS) is a fast-progressing neurodegenerative disease with a median survival time from diagnosis of 1.5-3 years. The cause of ALS is unknown, but inflammation may play a role. Fiber has been shown to lower inflammatory markers, and a high fiber intake was associated with a lower risk of ALS in a case-control study; however, prospective studies are lacking. We explored the relation between dietary intake of fiber and the risk of ALS in 5 large prospective cohort studies comprising over 1,050,000 US citizens who contributed 1,133 ALS cases during a mean of 15 years of follow-up (1980-2008). Cox proportional hazards models were used within each cohort, and cohort-specific estimates were subsequently pooled using a random-effects model. We found that intakes of total fiber, cereal fiber, vegetable fiber, and fruit fiber were not associated with ALS risk when comparing the highest quintile of intake with the lowest (for total fiber, pooled multivariable relative risk (RR) = 0.99, 95% confidence interval (CI): 0.80, 1.24; for cereal fiber, RR = 1.13, 95% CI: 0.94, 1.37; for vegetable fiber, RR = 0.97, 95% CI: 0.77, 1.23; and for fruit fiber, RR = 1.05, 95% CI: 0.86, 1.29). These findings do not support the hypothesis that fiber intake is a major determinant of ALS risk.

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Limb-onset amyotrophic lateral sclerosis patients visiting orthopedist show a longer time-to-diagnosis since symptom onset

Cited by 26 publications

References 17 publications

Neuronal apoptosis inhibitory protein is implicated in amyotrophic lateral sclerosis symptoms

Neuronal apoptosis inhibitory protein is implicated in amyotrophic lateral sclerosis symptoms

Contradictory Responses to the COVID-19 Pandemic in Amyotrophic Lateral Sclerosis Patients and Their Families and Caregivers in Japan

Dietary Fiber and Amyotrophic Lateral Sclerosis: Results From 5 Large Cohort Studies

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