Revised Criteria for the Diagnosis of Amyatrophic Lateral Sclerosis
In order to standardize the
diagnosis of ALS the El Escorial
Criteria (EEC) have been developed in 1994 following the older
Airlie-House-Criteria.
The EEC have been revised in 1998
(Brooks et al. 2000). The EEC are mainly clinical and form the gold standard of
ALS diagnosis. They are, however, relatively rigid and are not suitable for
early diagnosis.
They are important for scientific
classification and so far for recruitment into clinical trials, but not for clinical praxis.
According to the EEC, the diagnosis of ALS requires the presence of A-criteria and
the absence of B-criteria:
A-criteria:
A1: Degeneration of the lower motor
neuron approved by clinical,
electrophysiological
or neuropathological examination.
A2: Degeneration of the upper motor
neuron approved by clinical examination.
A3: Progressive dissemination beyond
typical nerve supply areas.
B-criteria:
B1: Electrophysiological or
neuropathological findings typical for other diseases
which
could explain the degeneration of
the upper and lower motor neuron.
B2: Findings in imaging studies
which can explain the clinical symptoms.
The diagnosis ALS requires an
extensive history and a thorough clinical and electrophysiological examination.
The electrophysiological tests can demonstrate the involvement of the lower
motor neuron in affected and frequently also in clinically
not affected body regions. The EEC
define four body regions to be evaluated:
• brain stem (bulbar),
• cervical (neck and upper
extremities),
• thoracal (trunk, abdominal wall)
• lumbosacral (lumbar spine and
lower extremities).
The classification according to the
revised EEC distinguishes between:
Clinically definite ALS
Proof of both, lower and upper motor
neuron signs in at least 3 body regions.
Clinically probable ALS
Proof of both, lower and upper motor
neuron signs in at least 2 body regions. The upper motor neuron signs have to
be demonstrated rostrally to the region of the lower motor neuron signs.
Clinically probable – laboratory supported
ALS
Clinical signs of the lower and the
upper motor neuron in 1 body region only. Additionally there are
electromyographic signs of active and chronic degeneration in at least 2
extremities.
Possible ALS
Signs of lower and upper motor
neuron in 1 body region only or upper motor neuron signs in 2 or more body
regions.
The old version of the EEC
additionally contained the category “clinically suspected ALS”
which has been deleted in the revised version.
