The Mr Joe B. Carvalho Pdf Free Download
Objectives:Summarize the etiology of ALS.Outline the typical findings on the examination of a patient with ALS.Describe the interventions available for patients with ALS.Review the importance of collaboration and communication among the interprofessional team to enhance the coordination of care for patients affected by ALS.Access free multiple choice questions on this topic.
The Mr Joe B. Carvalho Pdf Free Download
Although many potential mechanisms have been proposed, a precise, single etiology of sporadic amyotrophic lateral sclerosis is yet unproven. These mechanisms include altered RNA processing leading to prion-like self-aggregation, superoxide dismutase type 1 SOD1 mutations leading to free radical toxicity, cascading inflammatory responses, and excessive concentrations of glutamate, among others.[1][2] The rarer entity of familial ALS has numerous genetic mechanisms, most frequently repeat expansion of the C9ORF72 gene and various mutations of the SOD1 gene.[3] Mutated SOD1 protein misfolds and forms aggregates, leading to cellular injury and eventually apoptosis. Both genetic aberrations are inherited in a mainly autosomal dominant pattern.[4] Ultimately, rather than a single unifying cause, ALS is an etiologically diverse clinical entity, which is the result of a multitude of separate potential preceding aberrations.[5]
Edaravone is a free radical scavenger that is thought to reduce oxidative stress and has proven to be beneficial on a subset of patients with early-stage (less than two years of symptoms, independently living, VC >80% and scores of 2 or more in all items of ALSFRS-R) probable or definite ALS. One study showed approximately 33% slower functional decline in patients at 24 weeks follow up.[43] It has the limitation of being very expensive at around $146,000 per year and is administered in 60 mg daily infusions for 14 days followed by 14 days off for the first cycle, and then 60 mg/day for 10 days and 14 days off for the subsequent cycles. Edaravone should be used with caution in patients with asthma as it can cause serious asthmatic reactions in up to 5%.