Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease and Charcot disease, is a specific disorder that involves the death of neurons. The disease belongs to a group of disorders known as motor neuron diseases, which are characterized by the gradual degeneration and death of motor neurons. ALS is characterized by stiff muscles, muscle twitching, and gradually worsening weakness due to muscle wasting. This results in difficulty speaking, swallowing, and eventually breathing.
"The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others."
Alternate Names: Aran-Duchenne, Gehrig's Disease, Lou Gehrig's Disease, Motor Neuron Disease.
Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig's disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles.
In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away, and twitch. Eventually the ability of the brain to start and control voluntary movement is lost.
Individuals with ALS lose their strength and the ability to move their arms, legs, and body.
When muscles in the diaphragm and chest wall fail, individuals lose the ability to breathe without ventilatory support.
The disease does not affect a person's ability to see, smell, taste, hear, or recognize touch, and it does not usually impair a person's thinking or other cognitive abilities. However, several recent studies suggest that a small percentage of patients may experience problems with memory or decision-making, and there is growing evidence that some may even develop a form of dementia.
The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others.
There is no one test or procedure to establish the diagnosis of ALS.
The diagnosis of ALS is based on history, neurological findings consistent with the diagnosis of ALS and eletro-physiological and neuroimaging testing to rule out other impairments that may cause similar signs and symptoms. The diagnosis may be supported by electrophysiological studies (Electromyogram [EMG] and nerve conduction study [NCS] but these tests may be negative or only suggestive of the diagnosis.
No cure has yet been found for ALS. However, the FDA has approved the first drug treatment for the disease-riluzole (Rilutek).
Clinical trials with ALS patients showed that riluzole prolongs survival by several months. Riluzole does not reverse the damage already done to motor neurons, and patients taking the drug must be monitored for liver damage and other possible side effects. However, this first disease-specific therapy offers hope that the progression of ALS may one day be slowed by new medications or combinations of drugs.
Other treatments for ALS are designed to relieve symptoms and improve the quality of life for patients.
Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses.
Most individuals with ALS die from respiratory failure, usually within 3 to 5 years from the onset of symptoms.
The white navy blue striped ribbon is the symbol of awareness for Amyotrophic lateral sclerosis (ALS) or Lou Gehrig Disease, Aran-Duchenne, Gehrig's Disease, Lou Gehrig's Disease, Motor Neuron Disease)
The month of May is National ALS Awareness Month and each year the International Alliance of ALS/MND Associations celebrates 21 June as the global awareness day of recognition of ALS/MND.
In Canada, June has been declared as ALS Awareness Month by the Canadian Minister of Health.
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