Table 1: Awaji-shima Consensus Recommendations for the Application of Electrophysiological Tests to Diagnose ALS, as Applied to the Revised El Escorial Criteria (Airlie House 1998) [1]

  1. Principles (from the Airlie House criteria)The diagnosis of ALS requires
    1. 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; and
      3. progressive spread of symptoms or signs within a region or to other regions, as determined by history, physical examination, or electro-physiological tests
    2. the absence of
      1. electrophysiological or pathological evidence of other disease processes 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
  2. Diagnostic categoriesDefinite ALSdefined by clinical or electrophysiological evidence by the presence of LMN as well as UMN signs in the bulbar region and at least two spinal regions or the presence of LMN and UMNsigns in three spinal regionsProbable ALSdefined on clinical or electrophysiological evidence by LMN and UMN signs in at least two regions with some UMN signs necessarily rostral to (above) the LMN signsPossible ALSdefined when clinical or electrophysiological signs of UMN and LMN dysfunction are found in only one region; or UMN signs are found alone in two or more regions; or LMN signs are found rostral to UMN signs.Neuroimaging and clinical laboratory studies will have been performed and other diagnoses must have been excluded