Amyotrophic neuropathy, which is more commonly known as amyotrophic lateral sclerosis, or ALS, and is also called Lou Gehrig's disease, is a neurological condition that causes progressive nerve dysfunction. This condition typically affects patients later in life and causes muscle weakness, dysfunction and paralysis. The condition can be inherited, but much of the time its cause is unknown.
Symptoms
Amyotrophic lateral sclerosis causes nerves to send signals to muscles to die, causing impaired muscle function. Often muscles involved in breathing or swallowing are among the first involved, Medline Plus explains, making it difficult for patients to breathe or swallow. Patients may also develop progressive muscle weakness and cramping. Weakening of the spinal and neck muscles can cause the head to droop forward. People with ALS are also prone to drooling and experiencing uncontrollable muscle spasms. Over time they can also develop slowed speech, hoarseness and paralysis.
Causes
Approximately one in 10 cases of ALS are genetic, explains the Genetics Home Reference. Some of these genetic mutations cause a part of the nerves, called axons, to be improperly formed. Others can cause abnormally shaped proteins to accumulate, leading to neuronal damage. Researchers do not know what causes the nongenetic forms of amyotrophic lateral sclerosis. Damage caused by dangerously reactive molecules called free radicals may play a role, notes the Mayo Clinic. Patients with ALS often have elevated levels of a chemical called glutamate in their cerebrospinal fluid, which can be toxic to nerves at high concentrations. ALS may also be triggered by the immune system inadvertently attacking healthy nerve cells.
Risk Factors
A number of risk factors appear to be related to the development of amyotrophic lateral sclerosis. The condition is most commonly diagnosed in people between the ages of 40 and 60. People living in parts of Japan, Guam and West New Guinea also have an increased risk of developing the condition, possibly as a result of their diet. People who have served in the military are also more likely to develop ALS. It is also more common in women up until the age of 65; after the age of 70 there is no gender difference in the likelihood of developing ALS.
Diagnosis
Patients with trouble swallowing as well as muscle weakness, twitching or tremors may be suspected of having ALS. Blood tests as well as MRI or CT scans of the head will often be performed to rule out other diseases that could be causing the symptoms. Breathing tests can be done to see if the muscles necessary for respiration are affected. Patients may also have an electromyograph done, according to Medline Plus, to determine which nerves are affected. Genetic testing may also be indicated for patients with a family history of ALS.
Treatment
Although there is no cure for amyotrophic lateral sclerosis, one compound, called riluzole, has been shown to slow the disease's progression. Patients may also need medications to relieve constipation, pain, fatigue, depression, excessive saliva or phlegm production and constipation. Physical therapy can help patients retain their strength and range of motion for as long as possible, and occupational and speech therapy may help patients cope with their new limitations.
