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Predictors Of Amyotrophic Lateral Sclerosis Progression Still Unclear

Young patients with amyotrophic lateral sclerosis and those with limb-onset disease appear to survive significantly longer than do older patients and those with bulbar onset.

This finding lends weight to earlier suggestions that bulbar onset and older age, as well as early impairment of forced vital capacity, may be linked with shorter survival in these patients.

This retrospective study of 155 amyotrophic lateral sclerosis (ALS) patients in Germany has found no links between rate of progression and age, site of disease-onset or gender, however.

Understanding ALS progression is increasingly important as trials of new medical treatments for this disease are planned. T. Magnus and colleagues from the Clinical Research Group for Motor Neuron Disorders, University of Wuerzburg, Wuerzburg, compared survival factors with disease progression.

Progress of ALS was analysed in the study patients, every four months over a period of three years, using the ALS functional rating scale (ALS-FRS), forced vital capacity (FVC%) and a Medical Research Council (MRC) compound score based on a nine-step modified MRC scale.

The researchers compared age, (younger than 55 years versus 55 years), different sites of disease onset (bulbar versus limb onset) and gender to the rate of disease progression.

Overall, no overall significant differences were detected in disease progression in these subgroups. By contrast, significantly longer survival was observed in the younger age group (56 months versus 38 months) and in patients with limb-onset disease (51 months versus 37 months).

Further analysis (Cox analyses values) indicated declines of ALS-FRS, FVC% and MRC compound score were predictive of survival.

The authors underline the need for more studies to clarify whether nonspecific factors, such as muscle atrophy, dysphagia and coexisting diseases, influence prediction of survival in these patients.
Muscle & Nerve Volume 25, Issue 5, 2002. Pages: 709-714. "Disease progression in amyotrophic lateral sclerosis: Predictors of survival"

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