Median survival in population based studies of ALS is consistently two to four years ( 1), but the distribution is skewed and at least 5% of patients in clinic based observations survive more than a decade ( 2), particularly those with limb-onset. The region of onset is typically within the upper limb, lower limb or bulbar musculature, and the subsequent rate of disease progression is highly variable. 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.Īmyotrophic lateral sclerosis is characterized by progressive degeneration of cortical, brainstem, and spinal motor neurons. 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. In a regression analysis of the deceased, overall survival from symptom onset approximated to two years plus the time interval for initial spread.

Time to progression as a prognostic factor was independent of initial direction of spread. Factors independently affecting survival were time to next limb progression, age at symptom onset, and diagnostic latency. Initial progression was either to the contralateral leg (76%) or ipsilateral arm (24%). Variables studied were time to next limb progression, site of next progression, age at symptom onset, gender, diagnostic latency and use of riluzole.

Survival analysis was by univariate Kaplan-Meier log-rank and multivariate Cox proportional hazards. 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. Our objective was to establish the pattern of spread in lower limb-onset ALS (contra- versus ipsi-lateral) and its contribution to prognosis within a multivariate model.

Female age progression stories