The term "motor neuron disease" refers to a group of neurological diseases affecting the anterior horn cells of the bone (especially of the cervical cord) and brainstem (mainly the spinal bulb) and often the motor cortex, where part of the corticospinal path.

If the disease affects both the first and second motor neuron talking about amyotrophic lateral sclerosis (ALS). A variant of ALS, more unfavorable prognosis, progressive bulbar palsy is characterized by rapidly progressive paralysis of the muscles of the jaw, pharynx and tongue resulting in dysphagia, dysarthria, dysphonia and difficulty chewing.


ALS, disturbance to unknown etiology, is characterized by progressive muscle atrophy secondary to degeneration of motor neurons. It affects about one per 100000 individuals, mostly men of average or advanced age and family type in 5% of cases.



The initial Lou Gehrig disease symptoms due to degeneration of peripheral motor neuron, are usually composed of progressive muscular atrophy, initially mainly in the upper limbs starting from the distal tip ("ape hand"), decreased strength, fasciculation’s and disorders of speech, generally, although some patients complain of cramps, there are no disturbances of sensibility, intellectual deterioration or changes in bowel or bladder, bone tendon reflexes are emphasized.


In ALS, in contrast to the progressive bulbar paralysis, signs of impairment of cerebral nerves are less obvious. After about a year by the person may have signs of muscle degeneration of motor neuron central spasticity and hyper-reflexia, particularly in the legs with plantar reflex rebound.

Electromyography is the most useful laboratory test as it shows a normal nerve conduction in the presence of severe muscle atrophy, this finding serves to distinguish this disease from motor neuropathies, in which conduction velocity is reduced.


There is currently no specific treatment for the motor neuron disease and therapeutic measures should be mainly directed to support physiotherapy profits with all maneuvers to allow the maintenance of vital functions.


The disorder develops gradually and the duration of the disease depends on the presence or absence of bulbar signs (This sentence means that if there are Lou Gehrig disease symptoms of voice, swallowing, etc.., Namely that we are in the presence of a motor neuron disease variant pseudobulbar paralysis instead of variant ALS prognosis is worse. The SLA says it all because when in fact you should say motor neuron disease and only later say if variant ALS (better prognosis) or variant pseudobulbar palsy (a worse prognosis)).



The survival of patients depends upon the maintenance of respiratory function (diaphragm and intercostal muscles) and the protection of the airways (cough reflex and swallowing) as the most frequent cause of death is respiratory paralysis with progressive pneumonia aspiration. Historically, the course description is unfavorable let's a little less scientific.


ALS, sometimes called Charcot disease named after the French neurologist who first described it in 1860, is also known as ALS - Amyotrophic Lateral Sclerosis ", or Lou Gehrig's disease" in Anglo-Saxon countries.


It is a fatal disease characterized by progressive degeneration of motor neurons of the spinal column and brain, or that part of the nervous system, defined as "central nervous system, with the effect of inhibiting the transmission of nerve signals to muscles.

Basically, amyotrophic lateral sclerosis is a progressive disease that damages motor neurons - specialized cells in the spinal cord and brain stem that control muscle movement. As the disease progresses, the control of muscle degenerates.

The early symptoms of Lou Gehrig's disease are subtle - far less than they can easily be overlooked. Early symptoms of Lou Gehrig’s disease may include muscle jerks, muscle muscles pose obstacles, stiff, or weakness. As the disease advances, a person's speech may be slurred. Finally, a person with Lou Gehrig’s disease has a problem chewing and swallowing. The muscles become weaker as the disease advances, taking the force and finally the ability to walk or use arms and legs. The arms and legs look thinner as muscle tissue atrophies. In the late stages of the disease, respiratory muscles weaken, making it difficult to breathe. Most people with Lou Gehrig’s disease die from respiratory failure.

Most cases of Lou Gehrig's disease - more than ninety percent of cases - occur in people without family history of this disease. The cause of onset in these cases is unknown. Only a small percentage of cases it develops a genetic mutation known - these cases are known as Lou Gehrig’s disease or inherited familial Lou Gehrig’s.

There are eight different types of Lou Gehrig’s disease, distinguished by cause genetic pattern of inheritance, age at onset and disease progression. For example, Lou Gehrig’s type 1 and 8 are the adult-onset Lou Gehrig’s. Type 1 of Lou Gehrig’s progress very rapidly, most people with type 1 Lou Gehrig’s die of respiratory failure five years after onset. Lou Gehrig’s type 2 and 4 are the juvenile or early onset Lou Gehrig’s disease. Types 2, 4, and 8 are relatively rare. Approximately twenty percent of familial cases of Lou Gehrig’s disease and three percent of sporadic cases of Lou Gehrig’s disease are type 1.

Around the world, Lou Gehrig's disease occurs between four and eight people per 100 000. In the United States, approximately five thousand people are diagnosed with Lou Gehrig’s disease each year.

Lou Gehrig's is a disease of motor neurons also called amyotrophic lateral sclerosis or Charcot Mal. This is a progressive disease, failure, caused by degeneration of motor neurons and nerves in the central nervous system that voluntary control of the muscle.

The disorder causes muscle weakness and atrophy in the body as the upper and lower motor neurons degenerate, ceasing to send messages to neurons. By losing muscle function muscles will gradually degrade and eventually lead to atrophy. The patient will ultimately lose the ability to initiate and control voluntary movements, the role of bladder and sphincters as controlling the eye muscles Current usage is preserved, but not always.

Cognitive function is not usually affected except in certain situations where the disease occurs in association with frontotemporal dementia. However, there are reports that changes in frontotemporal type in these patients are very subtle in most patients, when tests are performed more detailed type. The autonomic nervous system will usually remain functional.

The condition is called Lou Gehrig's after the baseball player for the New York Yankess who was diagnosed with the disease in 1939 and died from it in 1941 at the age of 37.

What is ALS? - This is the short term for Amyotrophic lateral sclerosis , rarely called also the  Lou Gehrig's disease, is a progressive neurological disease, invariably fatal, which attacks nerve cells (neurons) responsible for controlling voluntary muscles. This disease is included  into a group of diseases called motor neuron diseases, which are characterized by the gradual degeneration and death of motor neurons.



Motor neurons are nerve cells located in the brain, brainstem, and spinal cord that serve as controlling units and vital communication links between the nervous system and voluntary muscles of the body. Messages from motor neurons brain (called upper motor neurons) are transmitted to motor neurons in the spinal cord (called lower motor neurons) and from there to each particular muscle. In ALS, motor neurons both upper and lower degenerate or die and stop sending messages to muscles. Unable to function, the muscles gradually weaken and become waste (atrophy) and contract. Eventually, the brain loses the ability to initiate and control voluntary movement.

ALS is the cause of weakness having a wide array of disabilities. Are affected eventually all muscles under voluntary control and patients lose their strength and ability to move his arms, legs and body. When muscles of the diaphragm and chest wall fail, patients can't breathe any more without a ventilator or breathing machine. Most people with ALS die from respiratory failure, usually between 3 to 5 years from the onset of ALS disease symptoms. However, about 10 percent of ALS patients survive 10 years or more.

Because ALS affects only motor neurons, the disease does not impair the mind, personality, intelligence or memory of the person. Nor does it affect the senses of sight, smell, taste, hearing or touch. Patients usually maintain control of eye muscles and functions of the bladder and intestines.

What are the ALS disease symptoms? - The onset of ALS may be so subtle that often are overlooked symptoms. The first symptoms may include twitching, cramps or muscle stiffness, muscle weakness affecting an arm or leg, speech impaired or nose, or difficulty chewing or swallowing. These widespread complaints then become more obvious weakness or atrophy, which can lead to the doctor suspecting ALS.

The parts of the body affected by early symptoms of ALS depend on which muscles in the body are damaged first. In some cases, symptoms initially affect one of the legs and patients have difficulty walking or running or realize that they face or stumble more often. At first, some patients see the effects of the disease in a hand or arm when they are hard to do simple tasks requiring manual dexterity such as buttoning a shirt, write or turn the key into a lock. Other patients notice trouble in speaking.

For patients to be diagnosed with ALS, they must have signs and symptoms of damage to the upper and lower motor neurons that can not be attributed to other causes. Although the sequence of emerging symptoms and the rate of disease progression vary from person to person, eventually patients will not stand or walk, lie down or get out of bed alone, or use his hands and arms.

Difficulty swallowing and chewing hurts the ability of patients to eat normally and increase the risk of choking. Maintaining weight becomes a problem. Because the disease usually does not affect cognitive abilities, patients realize their gradual loss of function, and can be anxiety or depression. Health professionals should explain the course of the disease and describe available treatments so that patients can make informed decisions in advance.

Amyotrophic lateral sclerosis, also known as Lou Gehrig’s disease, is characterized through a progressive reduction of the number of nervous cells in the brain and spine, called motor neurons. The motor neurons regulate the contraction and relaxation of the voluntary muscles, which make the motion of the body possible.



ALS is a progressive disease, which is invalidating and fatal. Walking, speaking, eating, swallowing and other fundamental functions of the body become more difficult in time. These afflictions can cause various injuries and other complications.



One, maybe two, people in 100000 develop ALS every year. The men are affected slightly more often than women. Even though the condition can start at any age, it is more frequent in middle aged people and the elderly. The causes for ALS are unknown. Some 5-10% of people with ALS present an inherited form of the disease. ALS is not infectious.

Symptoms

Lou Gehrig’s disease early symptoms and later symptoms include: - weakness of lack of precision and dexterity in the hands and feet; - gradual loss of strength in the arms and legs; - inability of voluntary control of the arms and legs; - muscle spasms ; - rigid and unsteady walking; - difficulty with swallowing, swallowing and speaking; - fatigue; - muscle cramps, more frequent during the night, which can start later in the evolution of the disease; - pain in the final stages of the disease.

It is important to remember that Lou Gehrig’s disease early symptoms are not sure indicators of ALS, since these effects could be noted in many other circumstances as well.

The first sign of ALS is usually represented by a slight feeling of weakness in one leg, arm, the face or the tongue. Other problems are represented by greater imprecision and difficulty in the performance of actions that require precise movements of the fingers and hands. There can also be muscle spasms. The weakness extends to the arms and legs for months, or even years. While the neurons continue to decrease in numbers, the muscle cells that would have normally be stimulated by these nerves begin to decrease in number and the muscles atrophy.

Respiratory afflictions are perhaps some of the most frequent severe complications of ALS. While the muscles of the throat and chest atrophy, swallowing and coughing become more difficult, which sometimes triggers the aspiration of food or saliva into the trachea. This determines the occurrence of pneumonia (inflammation of the pulmonary tissue). Respiratory afflictions worsen as the disease progresses, increasing the risk of infections and respiratory insufficiency.



Pneumonia, pulmonary embolism, respiratory insufficiency and cardiac insufficiency (probably due to the inexistence of adequate respiration following the atrophy of the muscles) are the most common causes of death among ALS patients. In most cases, death occurs at the latest after three to six years from the initial symptoms, otherwise there are individual who survive with the disease even for decades. Even if there is no remedy for ALS, treatment may help maintain stability and independence, control of the symptoms and can avoid complications for as long as possible. The treatment is aimed at promoting emotional support as the invalidity increases.