Do the benefits of currently available treatments justify early diagnosis and announcement? Arguments for.
Both a biologic imperative and an ethical imperative exist for providing the diagnosis of amyotrophic lateral sclerosis (ALS) as soon as possible and involving patients and their families in therapeutic decisions. The participation of excitotoxic mechanisms in ALS and the availability of riluzole, which may slow the rate of progression of ALS, provide the biologic rationale for early therapy in ALS. A neuroprotective agent, such as riluzole, is more effective at an early stage of disease, when more undamaged neurons remain. The development of effective therapy for ALS also provides the ethical basis for early announcement of diagnosis. When no primary treatment was available, an accepted approach was one of "protecting" the patient from the diagnosis, which was one of exclusion, requiring almost 100% certainty before announcement. The availability of a primary therapy, albeit not a cure, means that a diagnosis of ALS can now be offered as the most likely diagnosis with 90-95% certainty. The logical corollary of this new model is that patients must be involved immediately, to help decide when therapy is appropriate and to balance the relative personal significance of therapeutic gains versus adverse effects.
Cashman
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Centre for Research in Neurodegenerative Diseases, and Sunnybrook and Women's College Health Sciences Centre, University of Toronto, Ontario, Canada. neil.cashman@utoronta.la
D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D018696:Neuroprotective Agents / Q000008:administration & dosage*; D019782:Riluzole / Q000008:administration & dosage*
D018696:Neuroprotective Agents; D019782:Riluzole