Skip to main content

Table 1 Summary of Revised El Escorial Research Diagnostic Criteria for ALS (Brooks et al., 2000)

From: Amyotrophic lateral sclerosis

The diagnosis of ALS requires:  
1 Evidence of LMN degeneration by clinical, electrophysiological or neuropathological examination;  
2 Evidence of UMN degeneration by clinical examination, and  
3 Progressive spread of symptoms or signs within a region or to other regions, as determined by history or examination,  
Together with the absence of:  
[1] Electrophysiological and pathological evidence of other disease that might explain the signs of LMN and/or UMN degeneration, and  
[2] Neuroimaging evidence of other disease processes that might explain the observed clinical and electrophysiological signs  
Categories of clinical diagnostic certainty on clinical criteria alone  
Definite ALS  
• UMN signs and LMN signs in 3 regions
Probable ALS
• UMN signs and LMN signs in 2 regions with at least some UMN signs rostral to LMN signs  
Probable ALS – Laboratory supported  
• UMN signs in 1 or more regions and LMN signs defined by EMG in at least 2 regions  
Possible ALS  
• UMN signs and LMN signs in 1 region (together), or  
• UMN signs in 2 or more regions  
• UMN and LMN signs in 2 regions with no UMN signs rostral to LMN signs  
  1. UMN signs: clonus, Babinski sign, absent abdominal skin reflexes, hypertonia, loss of dexterity.
  2. LMN signs: atrophy, weakness. If only fasciculation: search with EMG for active denervation.
  3. Regions reflect neuronal pools: bulbar, cervical, thoracic and lumbosacral.