Prognosis of amyotrophic lateral sclerosis and the effect of referral selection

Prognosis and epidemiology of amyotrophic lateral sclerosis

“…While many studies report that bulbar symptoms predict shorter survival, [1][2][3][4][5][6][7]13,14,18 in this study the univariate finding of shorter survival in patients with bulbar symptoms disappeared when multivariable analyses were applied (table 2). [1][2][3][4][5][6][7]12,13,16 While we observed a 2.2-month decrease in median survival for those with bulbar disease compared to spinal onset patients, the average age of those diagnosed with a bulbar form of ALS was also notably higher in our patient population, easily accounting for the shorter survival in this group.…”

“…9,13 It has been suggested that patients referred from tertiary care centers include younger patients participating in clinical trials and an overrepresentation of familial disease due to interest in research in this type of ALS, and thus prognosis would be biased. 9 Patients with familial disease comprised fewer than 5% of our cohort, which is the expected proportion in an ALS population.…”

“…It is unclear which, if any, of the clinical variables are important for predicting survival. Previous studies of ALS populations identified several negative prognostic factors, [1][2][3][4][5][6][7][8][9][10][11][12][13] including older age at onset, female sex, and bulbar onset. As there is so much inconsistency in the published literature regarding the relative importance of prognostic factors, clinicians are challenged to provide a well-reasoned prognosis to newly diagnosed patients.…”

Accepting or declining non-invasive ventilation or gastrostomy in amyotrophic lateral sclerosis: patients’ perspectives

“…Amyotrophic lateral sclerosis (ALS) is an incurable neurological disease characterised by progressive multisystem degeneration, with an average survival of 2-5 years after diagnosis [1][2][3]. Current interventions are limited to slowing disease progression or palliating symptoms such as dysphagia, weight loss, and respiratory muscle weakness [3,4] and the recommended interventions, such as gastrostomy and noninvasive ventilation (NIV), tend to be required by people with ALS (pwALS) in later stages of the disease [3,5].…”

127th ENMC International Workshop: Implementation of a European Registry of ALS Naarden, The Netherlands, 8–10 October 2004

“…Amyotrophic lateral sclerosis (ALS) is a rare neurological condition (overall annual incidence rate in Europe 0.4-2.5 per 100,000) [1][2][3][4][5][6][7] with severe prognosis and death within 3-5 years from diagnosis [8][9][10]. Several community studies have been performed to study the incidence and characteristics of ALS in Europe.…”