Riluzole, a tedrotoxin-sensitive sodium channel blocker, is the only existing treatment and can delay disease progression with few months [8,9]. Non-invasive ventilation (NIV) can relieve dyspnoea, increase quality of life, and improve survival outcomes; particularly among ALS

patients without severe bulbar symptoms [10-13]. Several guidelines currently recommend the use of NIV as palliative treatment for ALS [4-7]. The effect of age has not been addressed in previous studies assessing the impact of NIV on survival outcomes [10,12,13]. Therefore, the effect of NIV on survival was compared Inhibitors,research,lifescience,medical in patients aged 65 years and older of age at the time of diagnosis, designated as Group 1 and Group 2 respectively. Based on previous studies

[10,12,13] it was hypothesised that NIV users would experience improved survival outcomes in both age groups when compared with patients who declined NIV were unable to tolerate treatment. Methods Design A registry-based retrospective cohort study was undertaken, Inhibitors,research,lifescience,medical covering the period January 2001 to June 2012, on 91 patients fulfilling the El Escorial World Federation Inhibitors,research,lifescience,medical criteria for probable or definitive ALS [14]. Once a positive diagnosis was made by a neurologist, patients were referred to a specialist to evaluate their suitability for NIV. All patients were systematically followed up at intervals of 3 to 6 months, until the date of death or June 1 2012, when the follow-up period ended. NIV and other palliative

treatments were offered to all the patients. Six patients showed survival Inhibitors,research,lifescience,medical over ten years and this finding was quite consistent with earlier studies which have been shown that 5 to 10% of ALS patients will survive over ten years [15]. Two of these six patients (age 54 and 79 years) used NIV while the rest of the patients (age range 49 – 63 years) refused NIV. Because of possible bias resulting from small number of patients with slow disease progression, these cases were excluded. In addition, one patient Inhibitors,research,lifescience,medical was excluded because of commencement of Oxalosuccinic acid NIV before the time of diagnosis for treatment of respiratory insufficiency due to pulmonary embolism. Because of current clinical trials legislation in Finland, patient consent is not required for register studies. Patient consent was therefore not obtained in this instance. The study protocol was approved by the ROCK inhibitor Ethics Committee of the Hospital District of South-West Finland. Altogether, 84 patients were included and retrospectively divided into two groups based on their age at the time of the diagnosis: Group 1 (age≤65 years) and Group 2 (age>65 years). Both groups were then subdivided further based on patients´ ability to tolerate the NIV. These were designated as the NIV Group and the Conventional Group, as presented in Figure 1. Figure 1 Flowchart of the study.