El Escorial revisited: Revised criteria for the diagnosis of amyotrophic lateral sclerosis

  title={El Escorial revisited: Revised criteria for the diagnosis of amyotrophic lateral sclerosis},
  author={Benjamin Rix Brooks and Robert G. Miller and Michael Swash and Theodore L. Munsat},
  journal={Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders},
  pages={293 - 299}
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder involving primarily motor neurons in the cerebral cortex, brainstem and spinal cord. The variability in clinical Žndings early in the course of ALS and the lack of any biological diagnostic marker make absolute diagnosis difŽcult and compromise the certainty of diagnosis in clinical practice, therapeutic trials and other research purposes. The El Escorial criteria for the diagnosis of ALS have been widely accepted… 
Amyotrophic lateral sclerosis: considerations on diagnostic criteria.
Of the 540 patients with initial ALS diagnosis, either probable or definite, seen at UNIFESP-EPM, 190 underwent thorough investigation, following regular clinical and therapeutic treatment for over two years, and thirty patients (15.78%) had their diagnosis completely changed.
Cortical hyperexcitability in Amyotrophic Lateral Sclerosis: Diagnostic and pathophysiological biomarker
Adding cortical hyperexcitability as a biomarker of UMN dysfunction, resulted in a greater proportion of ALS patients reaching a definitive diagnosis, and the first multicenter prospective study looking at both these diagnostic criteria was designed.
Electrodiagnosis of Amyotrophic Lateral Sclerosis: A Review of Existing Guidelines.
  • M. de Carvalho
  • Medicine
    Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society
  • 2020
Neurophysiological investigation of upper motor neuron dysfunction is difficult in ALS, detecting decreased cortical inhibition by threshold tracking cortical magnetic stimulation is a promising method, which needs to be validated in different centers.
Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria
These revised consensus criteria expand upon those of 2009 and embrace the concept of the frontotemporal spectrum disorder of ALS (ALS-FTSD), which is a re-conceptualisation that neuropsychological deficits in ALS fall along a spectrum.
Early diagnosis of amyotrophic lateral sclerosis – a way forward?
  • M. Swash
  • Medicine
    European journal of neurology
  • 2014
An analysis of ongoing studies of a group of 82 patients with ALS followed from the early stages of the disease for up to 3 years shows that transcortical motor stimulation with threshold tracking to assess UMN function allow detection of UMN disorder as shown by increased cortical motor excitability at an early stage of the Disease, at a time when conventional examination revealed no evidence of U MN deficit.
El Escorial or Awaji Criteria in ALS diagnosis, what should we take?
  • R. Dengler
  • Medicine, Psychology
    Clinical Neurophysiology
  • 2012
The gold standard of the diagnosis of amyotrophic lateral sclerosis (ALS) are still the revised El Escorial Criteria (EEC) of the World Federation of Neurology but several studies have been reported agreeing in their conclusion that the AC increase the sensitivity in ALS diagnosis without impairing the specificity.
Reliability of the El Escorial Diagnostic Criteria for Amyotrophic Lateral Sclerosis
The El Escorial criteria is a poor diagnostic indicator when patients’ records are examined and although medical education significantly improves the reliability of the criteria, concordance is still modest when the diagnosis includes suspected ALS.
Diagnostic criteria for amyotrophic lateral sclerosis (ALS)
A group of clinical neurophysiologists have taken up the above-mentioned points at an International Federation of Clinical Neurophysiology (IFCN) sponsored consensus conference on Awaji Island 2006 and suggested some helpful modifications to the revised El Escorial Criteria.
Amyotrophic lateral sclerosis: moving towards a new classification system
A new strategy is needed to combine the benefits of a systematic approach to classification with the rich and varied phenotypic descriptions used in clinical practice to avoid confusion between diagnosis and phenotype.
Comparison of the diagnostic criteria in patients with amyotrophic lateral sclerosis - the contribution of EMG findings
Application of the AS criteria improve the achievment of category of probable or definite diagnosis of ALS by 2.7 months earlier compared to the rEE, which is particularly affected by a higher frequency of positive EMG findings, when the As criteria were employed.


Inclusion body myositis. Observations in 40 patients.
The findings support the notion that IBM is a distinct entity in which a set of pathological features is associated with a constellation of clinical findings, and Prednisone treatment at dose levels frequently effective in polymyositis failed to prevent disease progression in those patients observed for 2 or more years.
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Full interference pattern in a clinically weak muscle
Signi cant abnormalities in autonomic function or electronystagmography