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What is sclerosis amyotrophic lateral and causes > Health and Wellness.

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Lateral sclerosis (als) Amyotrophic is a degenerative disease that affects motor neurons, which are responsible for controlling the movement of voluntary muscles.
Of all diseases of this kind, als is the most frequent, their approximate incidence is three of every 100,000 people per year, and affects more males, with a mean age of onset of the disease about 56 years. Only in Spain it is estimated that about 2,500 people are affected.
There are two forms: a family of hereditary (in 10% of cases) origin; and a sporadic, which is the usual form of appearance (in 90% of cases), and occurs randomly, and no cause apparent.
Causes of amyotrophic lateral sclerosis
The exact cause of the disease is unknown, although it has blamed on multiple factors such as aging, viral infection, and poisoning by heavy metals (such as mercury, cadmium, lead and thallium). Only between 5% and 10% of ALS cases seems to be due to hereditary causes.
There are two hypothesis, that still does not have been demonstrated, but it should be noted as potential causes:
  • Some kind of nerve growth factor deficiency.
  • Excess of a neurotransmitter called glutamate, on the outside of the cells of the nervous system.

Symptoms of ALS

· The lateral sclerosis (ALS) Amyotrophic usually begin to manifest itself from the 50-55 years in the sporadic forms, or to the family forms 11. There is a progressive muscle weakness (which usually begin in a hand or an arm), accompanied by loss of coordination, which makes it difficult to do so common activities such as swallow, climbing stairs or lifting. Among other symptoms, ALS patient may also have muscle cramps - especially after having done some exercise - and speech disorders.
· As the disease progresses, be involved more muscle groups; There is an affectation of the musculature distal extremities (hands and feet), with twitches, exaltation of reflexes and spasms. Fasciculations are brief and involuntary contractions of a muscle, which can be seen under the skin and do not produce any movement.
· ALS tends to move towards a complete paralysis. But unlike other neurological diseases, in the case of ALS have not been disorders of sensation, involvement of sphincters, or loss of intellectual ability or sexual function. The disease nor affects the muscles of the eyes, so the capacity is preserved until the end to make eye movements.

Diagnosis of ALS

· To clarify a diagnosis of ALS, the doctor will draw up a complete medical history, so the patient shall respond to an interrogation about his surgical background and family illnesses, and explain how is at the present time, providing information such as: when started the muscle weakness, which now feels worse, if there are some symptoms such as fever, cough, changes in the bowel, etc.
· The doctor will also perform a physical examination of the patient, assessing strength and endurance, and checking your reflexes and the possible existence of tremors and muscle spasms, twitching, or decrease in muscle tissue. Be observed, in addition, if the respiratory muscles are affected. Spirometry can provide information about the patient's respiratory status.
· The diagnostic test that will confirm the presence of amyotrophic lateral sclerosis is the electromyogram, which will determine the neurological involvement of muscles, and will demonstrate the loss of motor neurons. The test involves placing electrodes into a muscle and, using a computer, record the electrical activity of each muscle fiber.
· The realization of a cranial magnetic nuclear (NMR) resonance will show the existence of cerebral atrophy and changes in the central nervous impulse conduction. This test is contraindicated in patients with pacemakers or metal surgical prosthesis.
· Other diseases presenting a similar clinic; should be disposed in this way, prevent unnecessarily scare the patient and their families.

Treatment and prognosis of ALS

Amyotrophic lateral sclerosis is a disease with a poor prognosis, so it must be informed the patient of his situation with tact and detail. Currently it is not possible to cure ALS, and the treatment is aimed primarily at treating the symptoms.
Riluzole It is a drug that acts by blocking to glutamate and is used to delay the progression of the disease and prolong life. Other medications are given to patients to reduce the discomfort of the ELA (cramps, spasms, disorders of sleep) and the physiotherapy and rehabilitation are used to improve muscle function and mobility of patients as far as possible.
To combat respiratory and swallowing difficulty (the muscles involved in these two functions tend to be the first to be affected) guidelines can be followed when the patient having to eat:
  • Remain with the erect trunk and head slightly flexed.
  • Focus on the moment of meals avoiding, to the extent possible, speak.
  • Not take extremely hot or cold foods.
  • Make little abundant meals but more often throughout the day.
  • Chew slowly.
It is also important to educate family members of the patient on the disease being treated. They should learn how to perform the Heimlich maneuver to avoid possible choking.
This maneuver is to sit or stand behind the victim and surround him with both arms. Right hand closed in a fist is situated and placed below the sternum, left gets clamped to the handle. Once prepared, is compressed quickly and forcefully the victim's abdomen, from below upward. This maneuver should be repeated several times, until it comes out with force the object that had obstructed the airway.
It is recommended to intervention from a nutritionist, because patients with ALS usually lose weight due to the difficulties that have to swallow. In some cases, it may be necessary to place a tube in the stomach of the patient (Gastrostomy tube) so you can feed your needs without having to swallow the food.
The respiratory muscles is affected in almost all the patients of ALS, causing difficulty and even respiratory arrest. To control the respiratory function of the patient spirometry should be time. When it is committed will be necessary to resort to physiotherapy, hygiene of the Airways (to avoid possible pneumonia), oxygen therapy and, occasionally, tracheotomy.

Prognosis of ALS

Amyotrophic lateral sclerosis evolves towards a progressive worsening and the average survival from the development of initial symptoms is located between three and five years, although about 10% of patients get live more than ten years. The English scientist constitutes an exceptional case, Stephen Hawking, who was diagnosed when he was 21 years of age (over 50 years ago).
Currently there are several lines of research for the development of drugs that can combat or slow down the course of the disease, such as Neurotrophic factors, intravenous immunoglobulins, cyclophosphamide, and the glutamic acid antagonists.
It is possible that, in future, the ELA happens to be a disease with a more favorable prognosis. Ongoing research aim to achieve treatment curative or preventive for the disease, as well as attempting to clarify the causes that lead a person to suffer from it.

Help ALS patients

Although als is a disease common in Spain diagnosed around 900 new cases each year. Currently, the people diagnosed of ALS live longer after diagnosis than it did years ago, thanks to drugs such as Riluzole, which slows down the progression of the disease, but also by the effects of other multidisciplinary treatments, such as physical rehabilitation to improve muscular capacity, gastrostomy, which promotes better nutrition, and ventilation-invasive, which does not require intubation, endotracheal or tracheotomy, so it does not keep intact airway, avoiding risks such as pneumonia.
As in all diseases, early diagnosis and adequate information, enabling the sick and their families choose more favourable to appropriate treatment and lifestyle alternatives, improve the quality of life and long-term expectations.
Published for educational purposes
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