Criteria
Revised El Escorial criteria for ALS[52][53]
These criteria require the presence of upper motor neuron (UMN) signs (by clinical evaluation) and lower motor neuron (LMN) signs (by clinical, electrodiagnostic, or neuropathological evaluation) involving the cranial, cervical, thoracic, and lumbosacral segments of neuroaxis, with progressive quality over time. These criteria are mainly used to ensure a homogeneous population in clinical trials and are not particularly helpful in routine clinical practice.
Clinically definite ALS:
Clinical evidence for UMN and LMN signs in 3 segments of neuroaxis.
Clinically probable ALS:
Clinical evidence of UMN and LMN signs in at least 2 segments of neuroaxis
Some of the UMN signs located rostral to the LMN signs.
Clinically probable-laboratory supported ALS:
Clinical signs of UMN and LMN are present in 1 segment
Or UMN signs are present in 1 region and LMN signs defined by electromyography criteria (acute denervation with re-innervation) are present in at least 2 regions, and other diagnoses were excluded.
Clinically possible ALS - criteria needed for clinically probable-laboratory supported ALS could not be met, and:
Clinical signs of UMN and LMN are present in 1 segment
Or only UMN signs are found in 2 or more regions
Or LMN signs are found rostral to UMN signs, and other diagnoses were excluded.
Clinically suspected ALS:
Only clinical signs of LMN or of UMN in 1 or more regions.
Awaji criteria[54]
The revised El Escorial criteria were felt to be too restrictive and limited early diagnosis of ALS, thus precluding many patients from involvement in clinical trials. Therefore, the World Federation of Neurology ALS group proposed a revision to the revised El Escorial criteria, called the Awaji criteria. The main change was that the Awaji criteria accept electromyography (EMG) fasciculations as evidence of acute denervation on EMG, whereas previously EMG had to demonstrate fibrillation potentials and/or positive sharp waves. Also, the Awaji criteria state that EMG evidence of denervation is equivalent to clinical bedside evidence, making the 'clinical probable-laboratory supported ALS' category from the revised El Escorial criteria obsolete.
Gold Coast criteria[55]
The El Escorial criteria were felt to be overly complicated and prone to error. In addition, ALS clinicians were concerned that patients would perceive doubt in their diagnosis if they were told that they had 'probable' ALS rather than definite ALS, when there was really no doubt. Therefore, in 2020 the World Federation of Neurology ALS group proposed new criteria for diagnosis of ALS, called the Gold Coast criteria:
Progressive motor impairment documented by history or repeated clinical assessment, preceded by normal motor function, and
Presence of upper and lower motor neuron dysfunction in at least 1 body region (with upper and lower motor neuron dysfunction noted in the same body region if only 1 body region is involved) or lower motor neuron dysfunction in at least 2 body regions, and
Investigations excluding other disease processes.
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