Article

Amyotrophic lateral sclerosis is the most common motor neuron disorder in adults.
Although the diagnosis appears obvious in theory, clinical practice shows the
contrary as diagnosis is delayed in many patients; the average time between
symptom onset and diagnosis can reach 12months.
The delay can be explained by the
variability of the clinical presentation and by the absence of diagnostic
markers. In order to standardize diagnosis for enrolment in clinical research,
diagnostic criteria for ALS were created and revisited during the last 20years.
In 2006, the Awaji criteria for the diagnosis of ALS were proposed, adding two
major points to the diagnostic criteria: electromyography is considered
equivalent to clinical examination for the identification of LMN signs and
fasciculation potentials resume their prominent place in the diagnosis.
Comparisons of the accuracy of the revisited El Escorial and Awaji criteria
support improved diagnostic sensitivity without any effect on specificity with
the new classification. The only weakness of the new classification involves
patients with UMN signs in one region and LMN in two regions; these patients were
previously classified as laboratory-supported probable ALS and currently as
possible ALS, a lower level of diagnostic certainty. In all other instances the
accuracy appears to be improved by the Awaji criteria. Nevertheless, there is a
body of evidence suggesting the need for a revision of these new criteria, giving
more weight to clinical and complementary findings of UMN involvement. The need
to diagnose and treat ALS quickly could be facilitated by the inclusion of
complementary investigations that detect UMN signs.
CI - Copyright (c) 2012 Elsevier Masson SAS. All rights reserved.

Langue : FRANCAIS