INTRODUCTION

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder involving primarily motor neurons in the cerebral cortex, brainstem and spinal cord. The variability in clinical findings early in the course of ALS and the lack of any biological diagnostic marker make absolute diagnosis difficult and compromise the certainty of diagnosis in clinical practice, therapeutic trials and other research purposes.

The El Escorial criteria (1994) for the diagnosis of ALS have been widely accepted, but it was felt that they should be revised in order to increase their sensititvity. The criteria described below represent the result of a three-day workshop, convened at Airlie Conference Center, Warrenton, Virginia on April 2-4, 1998 by the World Federation of Neurology Research Committee on Motor Neuron Diseases.

This consensus document, reviewed, amended and ultimately accepted by all workshop participants, has been placed on the WFN ALS website for additional clinicians, researchers involved in ALS research, as well as appropriate scientific review bodies and concerned voluntary organizations, to review the criteria for determining the diagnostic certainty of ALS, prior to formal publication.

Correspondence:
Benjamin Rix Brooks M.D.
ALS Clinical Research Center
Neurology Department
University of Wisconsin Hospital & Clinics
Clinical Science Center H6-563
600 Highland Avenue
Madison, Wisconsin 53792-5132 USA
Telephone: 608-263-9057
Fax: 608-263-0412
Email: brooks@neurology.wisc.edu

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