Prognostic factors for the course of functional status of patients with ALS: a systematic review

Abstract

The progressive course of amyotrophic lateral sclerosis (ALS) results in an ever-changing spectrum of the care needs of patients with ALS. Knowledge of prognostic factors for the functional course of ALS may enhance clinical prediction and improve the timing of appropriate interventions. Our objective was to systematically review the evidence regarding prognostic factors for the rate of functional decline of patients with ALS, assessed with versions of the ALS Functional Rating Scale (ALSFRS). Two reviewers independently assessed the methodological quality of the thirteen included studies using the Quality in Prognosis Studies (QUIPS) tool. The overall quality of evidence for each prognostic factor was assessed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, considering risk of bias, imprecision, inconsistency, indirectness, and publication bias. The quality of evidence for the prognostic value of age at onset, site of onset, time from symptom onset to diagnosis, and ALSFRS-Revised baseline score was low, mainly due to the limited data and inconsistency of results in the small number of studies included. The prognostic value of initial rate of disease progression, age at diagnosis, forced vital capacity, frontotemporal dementia, body mass index, and comorbidity remains unclear. We conclude that the current evidence on prognostic factors for functional decline in ALS is insufficient to allow the development of a prediction tool that can support clinical decisions. Given the limited data, future prognostic studies may need to focus on factors that have a predictive value for a decline in ALSFRS(-R) domain scores, preferably based on internationally collected and shared data.

Heterogeneity in ALSFRS-R decline and survival: a population-based study in Italy

“…For example, score 0 or 1 at item 12 (respiratory insufficiency) of the ALSFRS-R has a different meaning in terms of survival than a score 0 or 1 at item 9 (climbing stairs). This may as well explain the results of a recent systematic review in which authors found in general low levels of evidence for several prognostic factors, often used by professionals in individual prognostication, examined on a selection of 13 papers dealing with ALS prognosis [14]. Although the ALSFRS-R score has been shown to be significantly associated with survival [13,15,18,19], few studies considered factors influencing ALS decline and survival and our study shows that they might differ.…”

“…Studies on prognostic factors on functional decline and survival may enhance clinical decision making, patient counselling and treatment selection [14].…”

The frontotemporal syndrome of ALS is associated with poor survival

“…Median survival of ALS patients is 3 years after symptom onset and the main cause of death is respiratory failure. The disease course is negatively influenced by older age at onset, early respiratory dysfunction, bulbar onset, a short time to diagnosis and the presence of a C9orf72 repeat expansion [6, 11, 12, 15, 34]. Previous studies suggested a negative effect of executive dysfunction or neurobehavioral changes on survival of ALS patients, although the use of general measures for behavioral changes precluded firm conclusions in some of them [10, 15, 18, 23, 26].…”

Hypnosis-based psychodynamic treatment in ALS: a longitudinal study on patients and their caregivers

“…This latter aspect has an enormous importance in a disease that substantially lacks medical treatment (Beghi et al, 2011) and reliable physical prognostic factors (Creemers et al, 2014). …”