Author, Year (reference no)
|
Type of Study
|
N
|
Aims of study
|
Summary of Results
|
Aboussouan et al. 1997 [24] |
Prospective Observational study |
39 |
To assess if there is a survival benefit in those who tolerate BiPAP versus those who do not tolerate BiPAP |
18 patients tolerated NIV. The rate of death was 3.1-fold greater in intolerant versus those who tolerated BiPAP |
Aboussouan et al. 2001 [29] |
Prospective Observational study |
60 |
Effect of NIV on survival and quality of life |
Improved survival and better compliance in those that tolerated NIV |
Bach et al. 2002 [28] |
Retrospective Observational study |
101 |
Assess if survival benefit to the use of NIV in those with ALS |
A 14-17-month improvement in survival in those with ALS treated with NIV |
Berlowitz et al. [24] |
Retrospective cohort analysis |
929 |
Assess the effect of NIV on survival, whether MND phenotype influences survival and if NIV affects the rate of pulmonary function decline |
Median tracheostomy free survival 28 months in those who received NIV compared with 15 months in those without NIV and a slower rate of PFT decline with NIV |
Bourke et al. 2003 [10] |
Prospective Study |
22 |
Assess the impact of NIV on quality of life |
A survival and quality of life benefit is strongly related to NIV compliance |
Bourke et al. 2006 [25] |
Randomised control trial |
41 |
Assess quality of life and survival in patients treated with NIV compared with standard care |
Improved quality of life and survival in the group treated with NIV |
Czaplinski et al. 2006 [32] |
Retrospective cohort analysis |
1034 |
Assessment of FVC at baseline to time to progression of 20 points in Appel ALS score or tracheostomy free survival |
Higher FVC at diagnosis is associated with longer survival and slower progression of FVC decline |
Kleopa et al. 1999 [21] |
Retrospective Observational study |
122 |
The effects of BiPAP on survival and the progression of pulmonary function tests with ALS |
BiPAP improved survival and slowed the progression of pulmonary function decline |
Lechtzin et al. 2007 [31] |
Retrospective study |
92 |
Assessment of tracheostomy free survival from diagnosis |
Improved survival with early initiation of NIV |
Miller et al. 2009 [6] |
Systemic literature review |
142 articles |
Update on the management of ALS |
Provision of several recommendations to improve the management of ALS |
Morgan et al. 2005 [36] |
Prospective observational study |
98 |
Prognostic value of FVC, MIF and SNIP over 3 years |
SNIP correlated well with transdiaphragmatic pressures, SNIP was more likely to be assessed pre death and was more sensitive and specific at predicting6 month mortality |
Piepers et al. 2006 [27] |
Systemic review |
12 studies |
Assessment of survival, quality of life and rate of respiratory function decline in those treated with NIV compared with those that didn’t tolerate NIV |
Seven studies showed a survival benefit in those treated with NIV versus those who didn’t tolerate it. There was also an improvement in the quality of life and a slower rate of decline of respiratory function compared with those who didn’t tolerate NIV |
Pinto et al. 1995 [23] |
Prospective controlled trial |
20 |
To assess the utility of BiPAP in improving survival in patients with ALS |
Overall, significant improvement in survival with patients treated with BiPAP (p<0.004) |
Pinto et al. 2003 [30] |
Prospective comparative study |
42 |
Assess survival in MND patients treated with NIV |
Total survival was longer in those treated with NIV |
Sherman et al. 1994 [22] |
Observational Study |
170 |
Assessment of Respiratory care in ALS |
There was a significant survival benefit in those treated with BiPAP |
Vitacca et al. 2018 [16] |
Retrospective Observational study |
194 |
Aim to assess would very early initiation of NIV have an impact on survival |
Very early commencement of NIV had a significant improvement in survival |