Unusual brain morphological changes in amyotrophic lateral sclerosis after long‐term tracheostomy invasive ventilation

2021 ◽  
Author(s):  
Arifumi Matsumoto ◽  
Kinya Hisanaga ◽  
Isao Nagano
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Morphological Changes ◽  
Invasive Ventilation ◽  
Lateral Sclerosis

scholarly journals The MITOS system predicts long-term survival in amyotrophic lateral sclerosis

2015 ◽  
Vol 86 (11) ◽  
pp. 1180-1185 ◽  
Author(s):  
Irene Tramacere ◽  
Eleonora Dalla Bella ◽  
Adriano Chiò ◽  
Gabriele Mora ◽  
Graziella Filippini ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Rating Scale ◽  
Invasive Ventilation ◽  
Floor Effect ◽  
Term Survival ◽  
Long Term Survival ◽  
Non Invasive ◽  
Non Invasive Ventilation ◽  
Lateral Sclerosis

ObjectiveThe choice of adequate proxy for long-term survival, the ultimate outcome in randomised clinical trials (RCT) assessing disease-modifying treatments for amyotrophic lateral sclerosis (ALS), is a key issue. The intrinsic limitations of the ALS Functional Rating Scale-Revised (ALSFRS-R), including non-linearity, multidimensionality and floor-effect, have emerged and its usefulness argued. The ALS Milano-Torino staging (ALS-MITOS) system was proposed as a novel tool to measure the progression of ALS and overcome these limitations. This study was performed to validate the ALS-MITOS as a 6-month proxy of survival in 200 ALS patients followed up to 18 months.MethodsAnalyses were performed on data from the recombinant human erythropoietin RCT that failed to demonstrate differences between groups for both primary and secondary outcomes. The ALS-MITOS system is composed of four key domains included in the ALSFRS-R scale (walking/self-care, swallowing, communicating and breathing), each with a threshold reflecting the loss of function in the specific ALSFRS-R subscores. Sensitivity, specificity and the area under the curve of the receiver operating characteristic curves of the ALS-MITOS system stages and ALSFRS-R decline at 6 months were calculated and compared with the primary outcome (survival, tracheotomy or >23-hour non-invasive ventilation) at 12 and 18 months Predicted probabilities of the ALS-MITO system at 6 months for any event at 12 and 18 months were computed through logistic regression models.ResultsDisease progression from baseline to 6 months as defined by the ALS-MITOS system predicted death, tracheotomy or >23-hour non-invasive ventilation at 12 months with 82% sensitivity (95% CI 71% to 93%, n=37/45) and 63% specificity (95% CI 55% to 71%, n=92/146), and at 18 months with 71% sensitivity (95% CI 61% to 82%, n=50/70) and 68% specificity (95% CI 60% to 77%, n=76/111). The analysis of ALS-MITOS and ALSFRS-R progression at 6-month follow-up showed that the best cut-off to predict survival at 12 and 18 months was 1 for the ALS-MITOS (ie, loss of at least one function) and a decline ranging from 6 to 9 points for the ALSFRS-R.ConclusionsThe ALS-MITOS system can reliably predict the course of ALS up to 18 months and can be considered a novel and valid outcome measure in RCTs.

scholarly journals Improved Long-Term Survival with Edaravone Therapy in Patients with Amyotrophic Lateral Sclerosis: A Retrospective Single-Center Study in Japan

2021 ◽  
Vol 14 (8) ◽  
pp. 705
Author(s):  
Hideki Houzen ◽  
Takahiro Kano ◽  
Kazuhiro Horiuchi ◽  
Masahiro Wakita ◽  
Azusa Nagai ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Rank Test ◽  
Positive Pressure ◽  
Control Group ◽  
Single Center ◽  
Term Survival ◽  
Long Term Survival ◽  
Kaplan Meier ◽  
Lateral Sclerosis

Reports on the long-term survival effect of edaravone, which was approved for the treatment of amyotrophic lateral sclerosis (ALS) in 2015 in Japan, are rare. Herein, we report our retrospective analysis of 45 consecutive patients with ALS who initially visited our hospital between 2013 and 2018. Of these, 22 patients were treated with edaravone for an average duration of 26.6 (range, 2–64) months, whereas the remaining patients were not treated with edaravone and comprised the control group. There were no differences in baseline demographics between the two groups. The primary endpoint was tracheostomy positive-pressure ventilation (TPPV) or death, and the follow-up period ended in December 2020. The survival rate was significantly better in the edaravone group than in the control group based on the Kaplan–Meier analysis, which revealed that the median survival durations were 49 (9–88) and 25 (8–41) months in the edaravone and control groups, respectively (p = 0.001, log-rank test). There were no serious edaravone-associated adverse effects during the study period. Overall, the findings of this single-center retrospective study suggest that edaravone might prolong survival in patients with ALS.

Long-term survival in amyotrophic lateral sclerosis after stem cell transplantation into the frontal motor cortex

Cytotherapy ◽  
2016 ◽  
Vol 18 (6) ◽  
pp. 806-808 ◽  
Author(s):  
Héctor R. Martínez ◽  
María Teresa González-Garza ◽  
Jorge Moreno-Cuevas ◽  
César E. Escamilla-Ocañas ◽  
Juan Miguel Tenorio-Pedraza ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Stem Cell ◽  
Motor Cortex ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Term Survival ◽  
Long Term Survival ◽  
Lateral Sclerosis

Randomized cross-over trial of ventilator modes during non-invasive ventilation titration in amyotrophic lateral sclerosis

Respirology ◽  
2017 ◽  
Vol 22 (6) ◽  
pp. 1212-1218 ◽  
Author(s):  
Bart Vrijsen ◽  
Bertien Buyse ◽  
Catharina Belge ◽  
Goele Vanpee ◽  
Philip Van Damme ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Invasive Ventilation ◽  
Non Invasive ◽  
Non Invasive Ventilation ◽  
Lateral Sclerosis
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