Amyotrophic lateral sclerosis. Recent advances in pathogenesis and therapeutic trials
H. Mitsumoto, M. R. Hanson and D. A. Chad
Department of Neurology, Cleveland Clinic Foundation, OH 44106.
We reviewed the current status of pathogenesis and therapeutic trials in
amyotrophic lateral sclerosis (ALS). Clinical studies have identified
several rare but definable causes for apparent ALS. Certain clinical
features previously considered unlikely to occur in ALS are found on
careful examination. Epidemiologic surveillance and recent studies of
neurotoxic plant seeds used in Guam have shed light on the pathogenesis of
endemic ALS. Extensive analyses of biochemical, metabolic, immunologic,
viral, and toxic factors have provided provocative results requiring
further studies. Reflecting on some of these hypotheses, therapeutic trials
have been performed more vigorously than ever. Amyotrophic lateral
sclerosis is now investigated at the molecular genetic level. Human autopsy
and experimental animal studies have expanded our understanding of basic
mechanisms involving motoneuronal degeneration. In the future, we must
continue a relentless search for the pathogenesis of ALS, prospective
clinical studies to define the limits of ALS, and well-designed, controlled
therapeutic trials.
