Predictors of delay in the diagnosis and clinical trial entry of amyotrophic lateral sclerosis patients: A population-based study

University of Milan, Milano, Lombardy, Italy
Journal of the Neurological Sciences (Impact Factor: 2.26). 01/2007; 250(1-2):45-9. DOI: 10.1016/j.jns.2006.06.027
Source: PubMed

ABSTRACT The El Escorial and the revised Airlie House diagnostic criteria for amyotrophic lateral sclerosis (ALS) were introduced to select patients for clinical trials. Heterogeneity of clinical presentation at onset and delay in diagnosis may decrease the likelihood for trial entry.
Identify risk factors for delay in the diagnosis and trial exclusion.
ALS incident cases were identified with El Escorial (EEC) and Airlie House criteria (AHC) through a population-based registry established in Puglia, Southern Italy, in the years 1998-99.
130 ALS incident cases were diagnosed with a median interval between onset of symptoms and diagnosis of 9.3 months and not different across both EEC and AHC categories. Twenty percent of cases were not eligible for clinical trials according to the AHC. About 5% of subjects in this series died with only lower motor neuron signs. Predictors for delay in the diagnosis were age between 65 and 75 years and spinal onset while fasciculations and cramps as first symptoms were predictors of exclusion from trials.
In this population-based series, diagnostic delay was longer in subjects with spinal onset and age between 65 and 75 and fasciculation as first symptoms. About 80% of incident cases were trial eligible with AHC criteria. However, a significant number of subjects with ALS, characterized by a limited spread of signs, were not trial eligible while alive.

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Available from: Giancarlo Logroscino, Nov 26, 2014
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    • "Bulbar presentation was associated with a shorter time to diagnosis [4] [8] [9]. We suggest that this implies recognition that bulbar symptoms are not derived from spinal cord or root lesions; indeed, neurologists associate progressive bulbar weakness as typically suggestive of ALS. "
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