Unexpected decline in survival from amyotrophic lateral sclerosis/motor neurone disease

Abstract

Objectives: To describe survival of 1226 Scottish adults with amyotrophic lateral sclerosis/motor neurone disease (ALS/ MND). Methods: Ten year, prospective, population based disease register. Cox time dependent proportional hazards modelling for multivariate survival analyses. Results: Median survival from onset was 25 months (interquartile range 16-34 months). In multivariate models we found an increased hazard with more recently diagnosed cases-that is, there was an unexpected decline in survival over the 10 year period (hazard ratio (HR) 1.06 (95% CI 1.04 to 1.09). Positive effects on survival were demonstrated for longer time from onset to diagnosis (HR 0.38 (95% CI 0.33 to 0.42), assessment by a neurological specialist (HR 0.56 (95% CI 0.40 to 0.77), and treatment with riluzole (HR 0.24 (95% CI 0.14 to 0.42). Poor prognosis was associated with bulbar onset (HR 1.25 (95% CI 1.09 to 1.46) and a mixed lower and upper motor neurone syndrome (HR 1.23 (95% CI 1.01-1.49) and increasing age. Conclusions: We found an unexpected decline in survival over the 10 year period, despite controlling for potential confounding variables. We would be cautious about overinterpreting these observations and suggest that further research is required to confirm or refute these findings.

Multidisciplinary Care Improves Survival of Patients with Amyotrophic Lateral Sclerosis in the Unique Health System (SUS) in Brazil

“…Generally, the risk of death of bulbar onset ALS is 4.5-fold higher (RR: 4.56, p=0.0007) than that of spinal onset ALS [20,22]. One reason for this finding is malnutrition [12,23], and as shown in this study, the BMI was significantly lower in bulbar onset patients (Table 1).…”

Trigeminal neuralgia due to pontine infarction

“…Cases with FTLD-MND also had a very short survival, with disease duration on average 2 to 3 years, similar to that of amyotrophic lateral sclerosis (ALS). 36 This is to be expected, since the motor neuron degeneration and other pathologic findings in FTLD-MND are similar to those in ALS where survival is about 3 years. 37 After pathologic reexamination, we did not have any cases of DLDH, unlike a recent clinicopathologic series.…”

Epidemiological and clinical factors impact on the benefit of riluzole in the survival rates of patients with ALS

“…A retrospective study with 1,226 patients with ALS showed a median survival time of 25 months (range: 16-34 months) and described an age difference, benefiting younger patients on clinical presentation, survival rate and functional independence 13 . Mandrioli et al 7 , Zoccolella et al 15 and Lee et al 6 also associated a greater age with a lower survival rate.…”

“…The patient may experience fatigue, gait instability, changes in speed or rhythm during speech, and a weak hand grip. Survival time after diagnosis is variable in the literature ( from eight to 66 months) 5,6,7,9,10,11,12,13,14,15,16,17,18,19,20,21,22 . In general, shorter intervals from symptom onset to diagnosis predict lower survival rates.…”