| 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 |