Reliability of the El Escorial diagnostic criteria for amyotrophic lateral sclerosis.
Neuroepidemiology 2002 Nov-Dec;21(6):265-70 (ISSN: 0251-5350)
Beghi E; Balzarini C; Bogliun G; Logroscino G; Manfredi L; Mazzini L; Micheli A; Millul A; Poloni M; Riva R; Salmoiraghi F; Tonini C; Vitelli E Clinica Neurologica, Ospedale San Gerardo, Monza, Italia. be...@marionegri.it; Collective Name: Italian ALS Study Group.
BACKGROUND: The El Escorial diagnostic criteria are the most commonly used in clinical studies and therapeutic trials in patients with amyotrophic lateral sclerosis (ALS). The accuracy of the El Escorial criteria was tested in clinical practice, but the reliability is unknown when the diagnosis of ALS must be assessed on the basis of medical records.
OBJECTIVE: To assess the reliability of the El Escorial criteria for the diagnosis of ALS in different settings.
DESIGN AND METHODS: Semistructured forms were used to include the main diagnostic information on 20 patients with definite, probable, possible, and suspected ALS and 19 patients with clinical conditions considered in the differential diagnosis.
Agreement was tested by comparing the diagnosis made by the attending physician (the 'gold standard') with that of 4 raters with different backgrounds: a teaching neurologist with research and practical experience in the field of motor neuron disorders, a neurologist with specific interest in motor neuron disorders and neurophysiological background, a neurophysiologist, and a general neurologist with only occasional ALS patients. Sources of disagreement were discussed and the agreement was tested further on the medical records of 98 additional cases taken from an ongoing ALS registry. Eight additional cases (ALS: 4; other conditions: 4) were examined directly by the 4 raters.
RESULTS: The interrater agreement on the medical records was poor. When the differential diagnosis was made between ALS (all diagnostic levels) and other conditions, interrater agreement was at best modest, with moderate variations when raters were compared in pairs and when each rater was compared with the physician. Agreement was higher after direct examination of the patients and increased significantly on the medical records after training. However, concordance was low, even after training, at the lowest diagnostic level (definite to suspected ALS vs. other conditions).
CONCLUSIONS: The El Escorial criteria are a poor diagnostic indicator when patients' records are examined. Although medical education significantly improves the reliability of the criteria, concordance is still modest when the diagnosis includes suspected ALS.
[Copyright 2002 S. Karger AG, Basel]. Atypical motor neuron disease and related motor syndromes. > Semin Neurol 2001 Jun;21(2):177-87 (ISSN: 0271-8235) > Verma A; Bradley WG > Department of Neurology, University of Miami School of Medicine, FL > 33136, USA. > > There is an imperative need for the early diagnosis of amyotrophic > lateral sclerosis/motor neuron disease (ALS/MND) in the current era > of emerging treatments. When evaluating the patient with ALS/MND, > the neurologist must consider a number of other motor neuron > disorders and related motor syndromes that may have clinical > features resembling ALS/MND. The revised Airlie House-El Escorial > diagnostic criteria have been established through the consensus of > experts meeting at workshops. However, by definition, using these > criteria a patient is likely to have fairly advanced disease at the > time of a definitive ALS/MND diagnosis. The reasons for the > difficulty in making an early ALS/MND diagnosis are several. No > surrogate diagnostic marker currently exists for ALS/MND. ALS/MND at > its onset is heterogeneous in clinical presentation, its clinical > course is variable, and several clinical variants are recognized. In > addition, certain motor syndromes, such as monomelic amyotrophy, > postpolio muscular atrophy, and multifocal motor neuropathy, can > clinically mimic ALS/MND. Therefore, not only may the diagnosis of > ALS/MND be clinically missed in the early stages, but worse, the > patient may be wrongly labeled as having ALS/MND. The diagnosis of > ALS/MND requires a combination of upper motor neuron > (UMN) and lower motor neuron (LMN) involvement. Motor syndromes in > which the deficit is restricted to the UMN or LMN through the entire > course of the disease are described as atypical MND in this review. > Approximately 5% of patients with ALS/MND have overt dementia with a > characteristic frontal affect. ALS/MND with parkinsonism and > dementia is rare outside the western Pacific region. The clinical > course of motor disorder in these overlap syndromes does not differ > from that in typical ALS/MND. > > Indexing Check Tags: > Language: English > MEDLINE Indexing Date: 200112 > Publication Type: Owner: NLM; Status: Completed > Publication Type: Journal Article; Review; Review, Tutorial > PreMedline Identifier: 0011442326 > Unique NLM Identifier: 21335316 > ___________________________________ >
