Pattern of spread and prognosis in lower limb-onset ALS

Abstract

Our objective was to establish the pattern of spread in lower limb-onset ALS (contra-versus ipsilateral) and its contribution to prognosis within a multivariate model. Pattern of spread was established in 109 sporadic ALS patients with lower limb-onset, prospectively recorded in Oxford and Sheffield tertiary clinics from 2001 to 2008. Survival analysis was by univariate KaplanMeier log-rank and multivariate Cox proportional hazards. Variables studied were time to next limb progression, site of next progression, age at symptom onset, gender, diagnostic latency and use of riluzole. Initial progression was either to the contralateral leg (76%) or ipsilateral arm (24%). Factors independently affecting survival were time to next limb progression, age at symptom onset, and diagnostic latency. Time to progression as a prognostic factor was independent of initial direction of spread. In a regression analysis of the deceased, overall survival from symptom onset approximated to two years plus the time interval for initial spread. In conclusion, rate of progression in lower limb-onset ALS is not influenced by whether initial spread is to the contralateral limb or ipsilateral arm. The time interval to this initial spread is a powerful factor in predicting overall survival, and could be used to facilitate decision-making and effective care planning.

Crowdsourced analysis of clinical trial data to predict amyotrophic lateral sclerosis progression

“…Overall, 15 different features were identified by more than one solver. Several were features reported in the literature 17,18,[24][25][26][27][28][29][30][31][32][33] , including age, site of disease onset, gender, the slope of disease progression so far, past ALSFRS slope, and past FVC slope, thus serving as validation of both the features and the algorithms. Unfortunately for many of the patients in PRO-ACT, FVC information was not available or other key features required to calculate FVC were missing.…”

Being PRO-ACTive: What can a Clinical Trial Database Reveal About ALS?

“…Beyond the speed of disease progression and survival outcome, it is important to understand the symptom profile [52,53]-why do some symptoms occur together more than others-and the patterns in which new symptoms manifest over time. For a patient, knowing which function is likely to deteriorate next will be of substantial impact on his/her daily life.…”

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

“…Lower limb onset shows a longer progression and higher survival rates because the motor neurons that are close to the ones degenerating are affected. Therefore, the propagation of clinical manifestations occurs contiguously, and it takes longer to impair spinal and bulbar structures related to vital functions (breathing, swallowing) 17,20 . The bulbar phenotype seems to interact with age.…”

“…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.…”