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Criteria for the diagnosis of Amyotrophic Lateral Sclerosis

The diagnoses of ALS requires the presence of:

1. Signs of lower motor neuron (LMN) degeneration by clinical, electrophysiological or neuropathologic examination,
2. Signs of upper motor neuron (UMN) degeneration by clinical examination, and
3. Progressive spread of signs within a region or to other regions, together with the absence of:
   • Electrophysiological evidence of other disease processes that might explain the signs of LMN and/or UMN degenerations; and
   • Neuroimaging evidence of other disease processes that might explain the observed clinical and electrophysiological signs.

Steps in the diagnosis of Amyotrophic Lateral Sclerosis

The diagnoses of ALS is made possible by:

1. History, physical and appropriate neurological examinations to ascertain clinical finding which may suggest suspected, possible, probable or definite ALS,
2. Electrophysiological examinations to ascertain findings which confirm LMN degeneration in clinically involved regions, identify LMN degeneration in clinically uninvolved regions and exclude other disorders,
3. Neuroimaging examinations to ascertain findings which may exclude other disease processes,
4. Clinical laboratory examinations, determined by clinical judgment, to ascertain possible ALS-related syndromes,
5. Neuropathologic examinations, where appropriate, to ascertain findings which may confirm or exclude sporadic ALS, coexistent sporadic ALS, ALS-related syndromes or ALS variants,
6. Repetition of clinical and electrophysiological examinations at least six months apart to ascertain evidence of progression.