Disease duration of progression is helpful in identifying isolated bulbar palsy of amyotrophic lateral sclerosis

Zhang, Huagang; Chen, Lu; Tian, Jinzhou; Fan, Dongsheng

doi:10.1186/s12883-021-02438-8

Cited by 7 publications

(5 citation statements)

References 13 publications

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“…However, limb-onset ALS accounts for two-thirds of all ALS cases, 1 and the median time from onset to significant limb weakness in bulbar-onset ALS is approximately nine months. 31 Furthermore, the MRC scale has been shown to be as good of a predictor of disease progression as forced vital capacity and ALSFRS-R, and the mBI is also strongly correlated with the ALSFRS-R. 32,33 In conclusion, we found that ALS patients face increased risk of ischemic stroke compared with controls, although the risk did not differ by the presence of disability in ALS patients. Further investigations are needed to elucidate the pathomechanism and potential risk factors of the increased risk of ischemic stroke in ALS.…”

Section: Discussionmentioning

confidence: 60%

Increased Risk of Ischemic Stroke in Amyotrophic Lateral Sclerosis: A Nationwide Cohort Study in South Korea

Kwon

Kim

Han

et al. 2023

Preprint

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Background: We investigated the risk of ischemic stroke in ALS and analyzed the effect of ALS-related physical disability using the Korean National Health Insurance Service database. Methods: A total of 2,252 ALS patients diagnosed between January 1, 2012, and December 31, 2015, and 1:10 age- and sex-matched control populations were included. After selection of cases that participated in the national health check-up programs, 659 ALS patients and 10,927 non-ALS participants were remained. Newly developing ischemic stroke as primary outcome was also defined by the ICD code and the incidence probability was assessed using by the Kaplan–Meier method during the follow-up duration. A Cox hazard regression model was used to examine the hazard ratios (HRs) for ischemic stroke in ALS after adjusting for potential confounders. Results: ALS patients were younger than the control group (60.3 ± 10.2 years vs. 61.4 ± 10.5 years, p = 0.008), and the proportion of male patients was similar between the two groups (61.0% vs. 62.5%, p = 0.447). ALS patients were more likely to have a lower body mass index (23.1 ± 2.92 vs. 24.0 ± 3.00, p < 0.001) and be non-drinkers (64.2% vs. 57.4%, p = 0.002) than the controls. In ALS patients, the incidence of ischemic stroke was 7.8 per 1,000 person-years, and the adjusted HR of ischemic stroke was 3.67 (95% confidence interval 2.02–6.67) compared with the control group. The risk of ischemic stroke did not differ by the presence of disability in ALS patients. Conclusions: Our findings suggest that ALS patients face increased risk of ischemic stroke compared with controls, but the risk did not differ by the presence of disability in ALS.

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Section: Discussionmentioning

confidence: 60%

Increased Risk of Ischemic Stroke in Amyotrophic Lateral Sclerosis: A Nationwide Cohort Study in South Korea

Kwon

Kim

Han

et al. 2023

Preprint

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“…When we examined differences in utilization for each intervention according to provider type, we found that women underwent feeding tube placement far more often than men, when a neurologist provided care, possibly due to greater prevalence of bulbar onset ALS in women than men [ 37 , 38 ]. Black beneficiaries received riluzole prescriptions and NIV less frequently than non-Hispanic White patients, despite studies suggesting that Black patients have lower baseline vital capacity and lower baseline functional scores than non-Hispanic White patients at first clinic visit for ALS [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

Adherence to practice parameters in Medicare beneficiaries with amyotrophic lateral sclerosis

Laurido-Soto,

Faust,

Nielsen

et al. 2024

PLoS ONE

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Objective Physician adherence to evidence-based clinical practice parameters impacts outcomes of amyotrophic lateral sclerosis (ALS) patients. We sought to investigate compliance with the 2009 practice parameters for treatment of ALS patients in the United States, and sociodemographic and provider characteristics associated with adherence. Methods In this population-based, retrospective cohort study of incident ALS patients in 2009–2014, we included all Medicare beneficiaries age ≥20 with ≥1 International Classification of Diseases, Ninth Revision, Clinical Modification ALS code (335.20) in 2009 and no prior years (N = 8,575). Variables of interest included race/ethnicity, sex, age, urban residence, Area Deprivation Index (ADI), and provider specialty (neurologist vs. non-neurologist). Outcomes were use of practice parameters, which included feeding tubes, non-invasive ventilation (NIV), riluzole, and receiving care from a neurologist. Results Overall, 42.9% of patients with ALS received neurologist care. Black beneficiaries (odds ratio [OR] 0.56, 95% confidence interval [CI] 0.47–0.67), older beneficiaries (OR 0.964, 95% CI 0.961–0.968 per year), and those living in disadvantaged areas (OR 0.70, 95% CI 0.61–0.80) received less care from neurologists. Overall, only 26.7% of beneficiaries received a feeding tube, 19.2% NIV, and 15.3% riluzole. Neurologist-treated patients were more likely to receive interventions than other ALS patients: feeding tube (OR 2.80, 95% CI 2.52–3.11); NIV (OR 10.8, 95% CI 9.28–12.6); and riluzole (OR 7.67, 95% CI 6.13–9.58), after adjusting for sociodemographics. These associations remained marked and significant when we excluded ALS patients who subsequently received a code for other diseases that mimic ALS. Conclusions ALS patients treated by neurologists received care consistent with practice parameters more often than those not treated by a neurologist. Black, older, and disadvantaged beneficiaries received less care consistent with the practice parameters.

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“…The differences in demographic and clinical features were compared between the patients with FLS and the patients with typical lower limb onset ALS by SPSS 18.0 software for Windows (SPSS Inc., Chicago, IL, USA). The quantitative data that were normally distributed were expressed as mean ± standard deviations (SDs), and quantitative data that were non-normally distributed were expressed as the medians and ranges (minimums, maximums) ( 12 ). The differences in categorical variables were analyzed by χ 2 test or Fisher's exact test, and continuous variables were evaluated by an independent-sample t -test or the Mann–Whitney U -test depending on the parametric or non-parametric nature of the data ( 12 ).…”

Section: Methodsmentioning

confidence: 99%

“…The quantitative data that were normally distributed were expressed as mean ± standard deviations (SDs), and quantitative data that were non-normally distributed were expressed as the medians and ranges (minimums, maximums) ( 12 ). The differences in categorical variables were analyzed by χ 2 test or Fisher's exact test, and continuous variables were evaluated by an independent-sample t -test or the Mann–Whitney U -test depending on the parametric or non-parametric nature of the data ( 12 ). The differences in survival between the two groups were compared using Kaplan–Meier analysis and the log-rank test.…”

Section: Methodsmentioning

confidence: 99%

Differentiating Slowly Progressive Subtype of Lower Limb Onset ALS From Typical ALS Depends on the Time of Disease Progression and Phenotype

2022

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BackgroundFlail leg syndrome (FLS) is a regional variant of amyotrophic lateral sclerosis (ALS) with the characteristics of slow progression and the symptoms confined to the lumbosacral region for extended periods. However, FLS may not be easily differentiated from typical ALS.ObjectiveThe objective of the study was to determine a cutoff time of disease progression that could differentiate FLS from the typical lower limb onset ALS.MethodsA cutoff point analysis was performed with maximally selected log–rank statistics in patients with lower limb onset ALS registered from 2009 to 2013. Based on the cutoff duration from the lower limb onset to second region significantly involved (SRSI), all patients were divided into the slowly progressive subtype of lower limb onset ALS group and the typical lower limb-onset ALS group. Patients with the slowly progressive subtype of the lower limb onset ALS, who had the flail leg phenotype, were classified as patients with FLS. Differences between groups were analyzed.ResultsAmong the 196 patients recruited, 157 patients with a duration <14 months from lower limb onset to SRSI were classified as having typical lower limb onset ALS. Twenty-nine patients with a duration more than or equal to 14 months and the flail leg phenotype were classified as having FLS. Patients with FLS exhibited a median diagnostic delay of 25 months, a median duration of 24 months from lower limb onset to SRSI, a forced vital capacity abnormity rate of 12.5% at the first visit to our department, and a median survival time of 80 months, which were significantly different from those of patients with typical lower limb onset ALS (p < 0.001, p < 0.001, p = 0.024, p < 0.001). The 5-year survival rate of the FLS group (79.3%) was much higher than that of the other group (1.9%).ConclusionsA crucial feature in differentiating FLS from typical lower limb onset ALS in Chinese patients may be symptoms confined to the lumbosacral region for at least 14 months, which may be better than 12 or 24 months used in the previous studies. The FLS was characterized by slower progression, less and later respiratory dysfunction, and a more benign prognosis than the typical lower limb onset ALS.

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Disease duration of progression is helpful in identifying isolated bulbar palsy of amyotrophic lateral sclerosis

Cited by 7 publications

References 13 publications

Increased Risk of Ischemic Stroke in Amyotrophic Lateral Sclerosis: A Nationwide Cohort Study in South Korea

Increased Risk of Ischemic Stroke in Amyotrophic Lateral Sclerosis: A Nationwide Cohort Study in South Korea

Adherence to practice parameters in Medicare beneficiaries with amyotrophic lateral sclerosis

Differentiating Slowly Progressive Subtype of Lower Limb Onset ALS From Typical ALS Depends on the Time of Disease Progression and Phenotype

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