AMYLOTROPHIC LATERAL SCLEROSIS
The earth is home to a billion species; Homo sapiens being one among them. What makes us unique is our ability to regulate movement and speech on will. When these skills are impaired due to neural loss, it leads to what we call ALS or Amyotrophic Lateral Sclerosis.
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ALS is a progressive nervous system disease that affects nerve cells in the brain and spinal cord, leading to the chronic degradation in fore-brain activity. It affects the motor neurons causing them to gradually deteriorate and further leading to cellular apoptosis. Motor neurons extend from the cerebrospinal system to muscles at various anatomical positions within the body.
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Researchers have been able to assert certain risk factors for ALS exhibiting a rather evident paradigm in terms of heredity. Familial ALS has been observed to likely get passed on from one generation to the other generation. An individual’s sex comes to the picture when observations of women below the age of 65 being less affected by ALS when compared to their male counterparts, were made. Studies also indicate that military personnel with battlefield experience are more likely to be affected by ALS when compared to the civilian population.
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Motor neurons extend from the cerebrospinal system to muscles at various anatomical positions within the body. When motor neurons are blemished, they stop transmitting impulses to the muscles ergo voluntary muscle control gets affected. Most theories regarding causality point to a rather complex interaction between genetic and environmental factors. From a genetic perspective, researchers have proposed that mutations in the TDP-43, FUS, and C9orf72 can cause ALS and that differences in the methylation of arginine residues can also induce ALS.
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ALS varies greatly from person to person depending on the neuron that is affected. It often starts at the limbs and then metastasizes throughout the body. Though the signs and symptoms may show variation to a certain degree, ALS is commonly associated with weakness of the appendages, cognitive and behavioural changes, muscle cramps in the arm region along with some trouble in swallowing. Diagnosing ALS is a herculean task. Due to the lack of proteins and markers that can be tested for, ALS requires a comprehensive series of diagnostic tests such as electromyography (EMG), muscle and nerve biopsy, X-rays, MRI, Spinal tap, Myelogram and a thorough neural examination.
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Though there is no perfect cure for this disease, current treatments include the FDA approved drug Riluzole (RiluTek) that extends the patient’s life by 6-12 months and Edravone (Radvica) which drastically reduces the rate of spread and neuro-degradation. Edaravone is a potent free radical scavenger and reacts with peroxynitrite to predominantly produce 4-NO-edaravone. The latter results in observations that indicate evident reduction in levels of 3-nitrotyrosine present in the cerebrospinal fluid. This, in turn, reduces oxidative stress significantly and also limits the extent of neural damage. Unfortunately, in most of the cases, the doctor only tries to address the comorbidities and not the actual underlying cause.
However, on-going research has shown some promising results. A 2016 review of stem-cell therapy trials was successful in extracting evidence indicating the relative safety and effectiveness of intraspinal stem cell implantation. MASTNIB and Beta-adrenergic agonist drugs have been proposed as a potential treatment option owing to their muscle growth and neuroprotection properties. Both these drugs are awaiting human trails to determine their efficacy.
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