Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive muscular paralysis reflecting degeneration of motor neurons in the primary motor cortex, corticospinal tracts, brainstem and spinal cord. Incidences and prevalence are relatively uniform in western countries, although foci of higher frequency occur in western pacific. The mean age of onset of sporadic ALS is about 60 years. Overally, there is slight male prevalence. HIRE WRITERS ONLINE.
Approximately two thirds of patients with typical ALS have a spinal form of the disease and presents with symptoms related to focal muscle weakness and wasting, where the symptoms may start either distally or proximally in upper and lower limbs. Gradually, spasticity may develop in the weakened atrophic limbs, affecting manual dexterity and gait. Patients with bulbar onset ALS usually presents with dysarthria and dysphagia for solid or liquids and limbs symptoms may develop almost simultaneously with bulbar symptoms, and in that vast majority of cases will occur within 1-2 years.
Paralysis is progressive and leads to death due to respiratory failure within 2-3 year for bulbar onset cases and 3-5 years for limb onset AlS cases. The diagnosis is based on clinical history, examination, electromyography, and exclusion is ALS-mimics’ by appropriate investigation. Noninvasive ventilations prolongs survival and improves quality of live. Riluzole is the only drug that has been shown to extend survival.
Symptoms of ALS
Free radicals are molecules that carry electrical charges that make them unstable and liable to damage cellular structures. Free radicals may build to toxic levels and damage cell, though an attack process called “oxidative stress”.
Defects in mitochondria
Processes inside the mitochondria produce potentially dangerous chemicals, mitochondrial DNA is always in danger of being damaged. Some amount of damages occurs naturally as part of the aging process, but in ALS there may be more damage to mitochondria than in average aging cell sustains.
The heavy metals lead, mercury and arsenic, although they can be toxic to the nervous system. Haven’t been shown to causative agents in ALS. Lead can damage upper and lower motor neurons, but, exposure to lead has been monitored and limited for most people for several decades.
Viruses and other infectious agents
Scientist suggest that viruses may play a role in ALS and other disorders that involve degeneration of nerve cells so far, however there is no proof of an ALs related viral trigger.
Immune system abnormalities
Many disorders that affect the nervous system are “auto immune” in nature meaning they occur when the body’s immune system mistakenly attacks its own tissues. Microglia, immune system cells found in the nervous system, appear to play a role in ALS.
Most cells have built in “suicide” program known as “programmed cell death” or apoptosis.” Under some circumstances, programmed cell death is normal. But in ALS and other degenerative diseases, it’s possible that the cell death program is activated inappropriately
Prevention and maintenance of ALS condition
Preserving hand function
Special grip for writing implements and eating utensils, devices that fit over keys to make the easier to turn, zipper pulls and button hooks can help make weakening hand more functional.
Today’s technology allows for mobility for almost everyone no matter how muscles remain functional. Physical and occupational therapist can help you identify the equipment that’s best for each stage of the disorder.
Getting enough to eat and drink
As the muscles involved in chewing, moving food toward the back of the mouth and swallowing weaken in ALS. Eating of sweet fruits may help prevent may help prevent ALS
Relief of symptom
While researchers continue efforts to identify compounds that slow or stop motor neuron degeneration in ALS, physicians can prescribe medications to treat troublesome symptoms during the course of the disease. These includes drugs to ease cramps and muscle twitches and helps in handling saliva etc.
Maintaining respiratory function
Most serious medical complications in ALS is the gradual deterioration of the muscles involved in breathing when the respiratory organs relax and weaken the act of breathing becomes energy consuming this leads to invasive ventilation.
There are several clinical trials and studies currently being conducted and here are some examples:
Arimolclomol, an experimental compound that may activate molecular “chaperones” which help proteins fold into correct shape, pyrimethamine, a drug use to threat malaria and toxoplasmosis that appears to target the SOD1 protein. ISIS-SOD1-Rx an experimental compound directed to SOD1.
A combination of five existing immunosuppressant drugs. Fingolimod a drug approved for use in multiple sclerosis. Acthar a drug to treat multiple sclerosis and other disorder in which autoimmunity plays a role.NPOO1, an experimental compound in development that may restore normal function of immune system.
GM604 an experimental compound that may protect nerve cells via control and regulation of numerous genes and pathways. Neurastem NSI-566, stem cells derived from early stage spinal cord cells.
Rasagiline, a drug use in parkinsons disease.
Tirasemativ, an experimental compound that may make muscles more sensitive to weak signals from the nervous system. Nuedexta, a drug used to treat uncontrolled emotional expression in people with ALS and multiple sclerosis that also may help with speech and swallowing.
The national ALS registry, a registry being overseen by ATSDR that is collecting data about who develops ALS send possible factors for ALS and develop care for the patient.
Near diaphragm pacing system, a surgically implanted device that simulates the respiratory diaphragm and may help ALS patients live longer and sleep better.
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