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A Population-Based Study

Bryan J. Traynor, MD, MRCPI; Mary B. Codd, MD, PhD; Bernadette Corr, RSN; Colm Forde, MB; Eithne Frost, BA; Orla M. Hardiman, MD, MRCPI

Arch Neurol. 2000;57:1171-1176.

Background  The El Escorial and the revised Airlie House diagnostic criteria for amyotrophic lateral sclerosis (ALS) classify patients into categories reflecting different levels of diagnostic certainty. We conducted a prospective, population-based study of the natural course of ALS in the Republic of Ireland during a 6-year period to examine the utility of these ALS diagnostic criteria.

Methods  Using data from the Irish ALS Register, we studied the clinical features of all patients diagnosed as having ALS in Ireland throughout their illness.

Results  Between 1993 and 1998, 388 patients were diagnosed as having ALS. Forty percent of patients reported bulbar-onset symptoms. Disease progression occurred over time: at last follow-up, 75% of all patients had bulbar signs, compared with 59% at diagnosis. When the El Escorial criteria were applied, more than half of patients (218 [56%]) had definite or probable ALS at diagnosis. Of the 165 possible and suspected ALS cases at diagnosis (trial ineligible), 110 (67%) were trial eligible at last follow-up. Of the 254 patients who had died, 229 (90%) had definite or probable ALS, whereas 25 patients (10%) remained trial ineligible at death. El Escorial category at diagnosis was not a significant prognostic indicator. Use of the Airlie House criteria had no effect on the median time from symptom onset to trial eligibility (12.9 vs 12.8 months).

Conclusions  The El Escorial and Airlie House diagnostic criteria are excessively restrictive. Furthermore, levels of diagnostic certainty cannot be used as prognostic indicators.

From the Department of Neurology, Beaumont Hospital (Drs Traynor, Forde, and Hardiman and Ms Corr), Department of Epidemiology and Biostatistics, Mater Misericordiae Hospital (Dr Codd), and Irish Motor Neuron Disease Association (Ms Frost), Dublin, Ireland.

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