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Toward earlier diagnosis of amyotrophic lateral sclerosis

Revised criteria

Address correspondence and reprint requests to Dr. Mark A. Ross, Department of Neurology, University of Iowa Hospitals, 200 Hawkins Drive, Iowa City, IA 52242.

We modified the World Federation of Neurology (WFN) diagnostic criteria for ALS to facilitate early diagnosis and used these criteria for enrollment of ALS patients in a clinical trial. The criteria developed required lower motor neuron (LMN) involvement in at least two limbs and upper motor neuron involvement in at least one region (bulbar, cervical, or lumbosacral). The EMG finding of fibrillation potentials was required for evidence of LMN involvement. Electrodiagnostic studies, neuroimaging, and laboratory studies were also used to exclude disorders that might mimic ALS. Using these criteria, the diagnosis of ALS was made at a mean time of 9.7 months from onset of symptoms, which compares favorably with the 12-month period cited in the literature. Using clinical assessment at completion of the trial, the diagnosis of ALS was believed to be accurate in those patients entered in the trial. However, pathologic confirmation of the diagnosis of ALS was not obtained. Based on our preliminary experience, we propose that these ALS diagnostic criteria will facilitate early diagnosis of ALS. Future studies should prospectively compare these criteria with the WFN criteria currently in use.

*See the Appendix on page 771 for rhCNTF ALS Study Group participants and their affiliations.

Presented in part at the 47th annual meeting of the American Academy of Neurology, Seattle, WA, May 1995.

Received October 7, 1997. Accepted in final form October 30, 1997.

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