ABSTRACT
The clinical diagnosis of probable or definite amyotrophic lateral sclerosis (ALS) based on El Escorial criteria (1) is probably correct in well over 95% of patients as determined by autopsy (2). The autopsy report helps us to reassure surviving family members that the diagnosis of the patient was correct during life in most instances and that clinical management was appropriate. As there is no available treatment to halt progression of ALS and prevent a fatal outcome, it is essential that new therapies be tested in clinical trials to eventually achieve success (3). Autopsy plays an important role in clinical trials by verifying the diagnosis of ALS in participating subjects and by detecting potentially adverse effects of treatment on tissue. Finally, autopsy shows an occasional unsuspected major disorder, such as an occult malignancy, that coexists with ALS.
