ACCELERATING THE DIAGNOSIS OF AMYOTROPHIC LATERAL SCLEROSIS

BACKGROUND- Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease in adults and is a relatively frequent cause of progressive weakness. Early recognition of ALS may be difficult because of its insidious onset and variable distribution of clinical involvement at presentation. In the past, the inexorable progression of ALS and lack of effective drugs did not favor a prompt diagnosis. The recent approval of the first effective drug for ALS, riluzole, and the wider availability of ALS clinical centers with experience in the care of patients with ALS has made an accelerated diagnosis beneficial to the patient. REVIEW SUMMARY- In this article, I review the process of diagnosing ALS in an efficient manner, focusing on the use of a set of clinical and electrodiagnostic criteria and including an algorithm as an aid to making a timely diagnosis. The accuracy of the diagnostic criteria, as well as false-positive and false-negative diagnoses, is reviewed. To aid in recognizing ALS early in its course, clinical presentations of ALS that may be mistaken for other disorders are discussed. The article concludes with a diagnostic algorithm and a discussion of laboratory tests. CONCLUSIONS- ALS is a sufficiently common disease that should be considered in the differential diagnosis of focal, atrophic weakness without sensory disturbance in an adult of any age.