Abstract
Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressive and fatal neurodegenerative disease that leads to a loss of muscle control due to nerve cells being affected in the brain and spinal cord. Some of the common clinical presentations of ALS include weakness of muscles, changes in behavior, dysfunction in speech, and cognitive difficulties. The cause of ALS is uncertain, but through several studies, it is known that mutations in SOD1 or C9orf72 genes could play a role as a factor of ALS. In addition, studies indicate that an excessive amount of free radicals, the reactive oxygen species (ROS), leads to neuronal damage by the peroxidation of unsaturated fatty acids in the neuronal cells. Edaravone, the newly approved antioxidant drug for ALS, halts the progression of ALS in the early stages through its cytoprotective effect and protects the nerves by reducing ROS. In this review, different aspects of ALS will be discussed, including its pathology, genetic aspect, and diagnosis. This review also focuses on edaravone as a treatment option for ALS, its mechanism of action, and its pharmacological properties. Clinical trials and adverse effects of edaravone and care for ALS patient are also discussed.
Highlights
Amyotrophic Lateral Sclerosis was first diagnosed in 1824 in a patient named CharlesBell, but it became more widely known after the death of a baseball player, Lou Gehrig, the name “Lou Gehrig’s disease” [1,2]
In Amyotrophic Lateral Sclerosis (ALS) patients, as the brain and spinal cord lose the ability of the muscle control due to both the upper and lower motor degeneration, they result in some common clinical symptoms of weakness of muscles that usually begins in the extremities such as limb muscles, more commonly in distal muscles [2]
ALS involves its neuropathological hallmark of dysfunction of the lower motor neurons in the brain and spinal cord, leading to a loss of motor functions due to oxidative stress
Summary
Amyotrophic Lateral Sclerosis was first diagnosed in 1824 in a patient named Charles. Amyotrophic Lateral Sclerosis (ALS) was known purely as a motor neuron degeneration that affects the brain and spinal cord and eventually leads to paralysis and possibly death from respiratory failure and complications from immobility. It is defined as a multisystem neurodegenerative disorder with the disease characterized at different levels: clinical, neuropathological, and genetics [3]. As the oxidative stress and increase in ROS are involved in various pathways of human diseases, new perception and comprehension of these linked processes will pave the way in developing new therapies and treatments for neurodegenerative diseases [6]. Known as a free radical scavenger, reduces oxidative stress, playing a role in the disease progression and having a cytoprotective effect on the nerve cells to protect the degeneration of neuronal cells [8]
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