Withdrawal of Life-Sustaining Mechanical Ventilation for a Patient With Amyotrophic Lateral Sclerosis in Locked-In Syndrome.
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ABSTRACT:
SUBMITTER: Choi SJ
PROVIDER: S-EPMC11372215 | biostudies-literature | 2024 Sep
REPOSITORIES: biostudies-literature
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Withdrawal of Life-Sustaining Mechanical Ventilation for a Patient With Amyotrophic Lateral Sclerosis in Locked-In Syndrome.
Choi Seok-Jin SJ Yoo Shin Hye SH Lee Sun Young SY Sung Jung-Joon JJ
Journal of clinical neurology (Seoul, Korea) 20240901 5
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Project description: Not available
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Project description:There has been an increase in the use of mechanical ventilation (MV) in motor neurone disease (MND) to alleviate symptoms related to hypoventilation. Little is known about its use at the end of life, and the withdrawal of MV is a challenging topic. A scoping review was conducted to map the existing evidence of ventilation use at the end of life in MND. The Joanna Briggs Institute methodological framework was used, and a total of 31 documents were included in the data extraction. Boarder themes around place, planning, cause and comments on death were identified. Our findings show that the focus of the literature has been on the process of the withdrawal of ventilation, creating the subthemes of timing and reason for withdrawal, along with ethical, emotional, and practical issues, medications used and the time until death. There is a foundation of evidence to guide the process and discuss the ethical and emotional issues of withdrawing ventilation. However, there remains limited evidence from patient and family member perspectives. Importantly, there is almost no evidence exploring dying with ventilation in place, the active or passive decisions around this process, how the interface might impact the dying process, or what families think about this.
| S-EPMC9497211 | biostudies-literature
Project description:This SuperSeries is composed of the following subset Series: GSE39642: NanoString nCounter immune-related gene expression in blood sorted CD14+CD16- monocytes from sALS, fALS and HC subjects GSE39643: NanoString miRNA profiling of peripheral blood sorted CD14+CD16- monocytes from amyotrophic lateral sclerosis, multiple sclerosis and healthy control subjects Refer to individual Series
2012-07-25 | E-GEOD-39644 | biostudies-arrayexpress
Project description:This SuperSeries is composed of the SubSeries listed below.
2012-07-26 | GSE39644 | GEO
Project description:Identification of amyotrophic lateral sclerosis (ALS) associated genes. Post mortem spinal cord grey matter from sporadic and familial ALS patients compared with controls. Keywords: other
2003-11-14 | GSE833 | GEO
Project description:ObjectiveDeficiencies and excess of essential elements and toxic metals are implicated in amyotrophic lateral sclerosis (ALS), but the age when metal dysregulation appears remains unknown. This study aims to determine whether metal uptake is dysregulated during childhood in individuals eventually diagnosed with ALS.MethodsLaser ablation-inductively coupled plasma-mass spectrometry was used to obtain time series data of metal uptake using biomarkers in teeth from autopsies or dental extractions of ALS (n = 36) and control (n = 31) participants. Covariate data included sex, smoking, occupational exposures, and ALS family history. Case-control differences were identified in temporal profiles of metal uptake for individual metals using distributed lag models. Weighted quantile sum (WQS) regression was used for metals mixture analyses. Similar analyses were performed on an ALS mouse model to further verify the relevance of dysregulation of metals in ALS.ResultsMetal levels were higher in cases than in controls: 1.49 times for chromium (1.11-1.82; at 15 years), 1.82 times for manganese (1.34-2.46; at birth), 1.65 times for nickel (1.22-2.01; at 8 years), 2.46 times for tin (1.65-3.30; at 2 years), and 2.46 times for zinc (1.49-3.67; at 6 years). Co-exposure to 11 elements indicated that childhood metal dysregulation was associated with ALS. The mixture contribution of metals to disease outcome was likewise apparent in tooth biomarkers of an ALS mouse model, and differences in metal distribution were evident in ALS mouse brains compared to brains from littermate controls.InterpretationOverall, our study reveals direct evidence that altered metal uptake during specific early life time windows is associated with adult-onset ALS.
| S-EPMC7318091 | biostudies-literature
Project description:Rationale: Noninvasive ventilation (NIV) is standard of care in amyotrophic lateral sclerosis (ALS), yet few data exist regarding its benefits.Objectives: We sought to identify whether the use of NIV was associated with survival in ALS.Methods: This was a single-center retrospective cohort study of 452 patients with ALS seen between 2006 and 2015. We matched one or more NIV subjects (prescribed NIV) to non-NIV subjects (never prescribed NIV) without replacement. The outcome was time from NIV prescription date (NIV subjects) or matched date (non-NIV subjects) until death. We performed a multivariable Cox proportional hazards model with NIV hourly usage as a time-varying covariate and stratified by matched groups.Results: After creating 180 matched groups and adjusting for age, body mass index, ALS Functional Rating Scale Revised dyspnea score, and hourly NIV use, NIV was associated with a 26% reduction in the rate of death compared with non-NIV subjects (hazard ratio [HR], 0.74; 95% confidence interval [CI], 0.57-0.98; P = 0.04). Among those with limb-onset ALS, NIV subjects had a 37% lower rate of death compared with non-NIV subjects (HR, 0.63; 95% CI, 0.45-0.87; P = 0.006). Among NIV subjects, we found that NIV use for an average of ≥4 h/d was associated with improved survival.Conclusions: NIV use was associated with significantly better survival in ALS after matching and adjusting for confounders. Increasing duration of daily NIV use was associated with longer survival. Randomized clinical trials should be performed to identify ideal thresholds for improving survival and optimizing adherence in ALS.
| S-EPMC7919153 | biostudies-literature
Project description:Study objectiveTo evaluate the effects of noninvasive ventilation (NIV) on sleep in patients with amyotrophic lateral sclerosis (ALS) after meticulous titration with polysomnography (PSG).MethodsIn this prospective observational study, 24 ALS patients were admitted to the sleep laboratory during 4 nights for in-hospital NIV titration with PSG and nocturnal capnography. Questionnaires were used to assess subjective sleep quality and quality of life (QoL). Patients were readmitted after one month.ResultsIn the total group, slow wave sleep and REM sleep increased and the arousal-awakening index improved. The group without bulbar involvement (non-bulbar) showed the same improvements, together with an increase in sleep efficiency. Nocturnal oxygen and carbon dioxide levels improved in the total and non-bulbar group. Except for oxygen saturation during REM sleep, no improvement in respiratory function or sleep structure was found in bulbar patients. However, these patients showed less room for improvement. Patient-reported outcomes showed improvement in sleep quality and QoL for the total and non-bulbar group, while bulbar patients only reported improvements in very few subscores.ConclusionsThis study shows an improvement of sleep architecture, carbon dioxide, and nocturnal oxygen saturation at the end of NIV titration and after one month of NIV in ALS patients. More studies are needed to identify the appropriate time to start NIV in bulbar patients. Our results suggest that accurate titration of NIV by PSG improves sleep quality.CommentaryA commentary on this article appears in this issue on page 511.
| S-EPMC4410930 | biostudies-literature
Project description:Amyotrophic lateral sclerosis is a fatal CNS neurodegenerative disease. Despite intensive research, current management of amyotrophic lateral sclerosis remains suboptimal from diagnosis to prognosis. Recognition of the phenotypic heterogeneity of amyotrophic lateral sclerosis, global CNS dysfunction, genetic architecture, and development of novel diagnostic criteria is clarifying the spectrum of clinical presentation and facilitating diagnosis. Insights into the pathophysiology of amyotrophic lateral sclerosis, identification of disease biomarkers and modifiable risks, along with new predictive models, scales, and scoring systems, and a clinical trial pipeline of mechanism-based therapies, are changing the prognostic landscape. Although most recent advances have yet to translate into patient benefit, the idea of amyotrophic lateral sclerosis as a complex syndrome is already having tangible effects in the clinic. This Seminar will outline these insights and discuss the status of the management of amyotrophic lateral sclerosis for the general neurologist, along with future prospects that could improve care and outcomes for patients with amyotrophic lateral sclerosis.
| S-EPMC10089700 | biostudies-literature
Project description:Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease. Although affected patients may develop cancers, major surgical intervention has been hampered by its questionable overall benefit due to limited prognosis and risk of postoperative respiratory collapse. A recent study, however, showed that tracheostomy invasive ventilation (TIV) prolonged median survival to 11.3 years; thus, patients with ALS receiving TIV might benefit from major surgery. A 66-year-old man with ALS, who had received TIV and enteral tube feeding for 8 years, presented with bloody stool. The patient also had type 2 diabetes mellitus, stage 4 chronic kidney disease, abdominal aortic aneurysm, and anti-phospholipid syndrome, as well as multiple episodes of pneumonia and catheter-related urinary tract infection treated by antibiotics. Medical examination and esophagogastroduodenoscopy revealed a type 3 tumor in the middle part of the stomach. The patient's preoperative diagnosis was gastric cancer (GC), MU, type3, Less-Post, T3(SS), N1, H0, P0, M0, cStage III. The estimated mortality rate was 30.5%, according to the Japanese National Clinical Database. The patient and his family were fully informed of the risk of surgery; the patient clearly requested curative surgery by eye movement. Thus, robot-assisted total gastrectomy (RATG) was performed. The tissues were extremely fragile and hemorrhagic. The surgical time was 7 h 0 min; intraoperative blood loss was 324 ml. Pathological examination revealed GC, MU, type3, T4a(SE), N2, H0, CY0, P0, M0 fStage IIIB. The postoperative course was uneventful. He has remained in stable condition for 3 months. Our findings suggest that patients with ALS who achieve longer survival with TIV can undergo major cancer surgery, including robot-assisted surgery, which may facilitate a better mid-long-term prognosis.Supplementary informationThe online version contains supplementary material available at 10.1007/s13691-021-00499-7.
| S-EPMC8421503 | biostudies-literature