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Amyotrophic lateral sclerosis: diagnostic criteria

  1. The revised “El Escorial” World Federation of Neurology criteria.

    Is the clinical standard for diagnosis of ALS. These criteria allow assignment of diagnostic certainty and were designed for research purposes, to ensure appropriate inclusion of patients into clinical trials.

    By the “El Escorial” criteria, diagnosis of ALS requires the presence of:
    1. Evidence of lower motor neuron (LMN) degeneration by clinical, electrophysiological, or neuropathological examination
    2. Evidence of upper motor neuron (UMN) degeneration by clinical examination
    3. Progressive spread of symptoms or signs within a region or to other regions, as determined by history or examination.

    The “El Escorial” criteria require the absence of electrophysiological and pathological evidence of other disease processes that might explain the signs of LMN and/or UMN degeneration, and the absence of neuroimaging evidence of other disease processes that might explain the observed clinical and electrophysiological signs.


    Reference: Elman L, McCluskey L. Diagnosis of amyotrophic lateral sclerosis. Available at: Up To Date, version 17.1. Current through January 2009.