Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS), commonly known as Lou Gehrig's disease, is a severe degenerative neurological disorder that is, unfortunately, always fatal. ALS attacks and destroys the nerve cells (neurons) that control voluntary movement. As muscles atrophy, patients lose motor control, progressively losing the ability to walk, to move their extremities, to swallow and to speak. Eventually, patients with ALS become completely paralyzed and require a ventilator to breathe. In the vast majority of cases of ALS, the cause of the disease is unknown. Patients with ALS usually die within 3 to 5 years of diagnosis, although a small percentage survive for a decade or more.
Risk Factors for ALS
At present, no specific cause of ALS has been established. In a small percentage of cases, there is some family history of the disorder, and there is believed to be a genetic predisposition to the disease. Strong evidence exists that environmental factors, such as toxins, pathogens and traumatic injury, also play a role in the genesis of ALS. Studies have shown, for example, that military personnel deployed to the Gulf War have been more prone to develop this disorder than their comrades who served in other parts of the world. Typically, ALS affects individuals between 40 and 60 years of age and the disease is more common in men than in women.
Symptoms of ALS
The symptoms of ALS are progressive and may not always follow the same sequence of development. These symptoms may include:
- Twitching (fasciculations) of shoulders, arms or tongue
- Muscle cramps
- Slurred speech
- Weakness of hands, feet and legs
- Tripping or stumbling
- Difficulty walking
- Difficulty chewing or swallowing
- Stiff muscles (spasticity)
- Inability to breathe without ventilation
Because of difficulty chewing and swallowing, patients with ALS may have trouble maintaining their weight. While cognitive difficulties are not symptoms of this disorder, patients often become anxious and depressed about their progressive loss of function.
Diagnosis of ALS
In order to diagnose ALS, the patient's medical history is reviewed and a physical examination is performed. Since symptoms of this disorder are similar to those of other medical conditions, many diagnostic tests are usually administered. These may include:
- Electromyogram (EMG)
- Muscle biopsy
- Nerve conduction study
- Blood tests
- Urine tests
- MRI scan
Patients diagnosed with ALS have signs and symptoms of both upper and lower motor neuron damage that cannot be attributed to other causes.
Treatment of ALS
Currently, there is no cure available for ALS, so treatment focuses on relieving symptoms. A combination of medications, speech therapy, physical therapy and occupational therapy may be used to manage this condition. Medications may be prescribed to help ease muscle cramping and spasticity and to reduce the production of saliva or phlegm in order to diminish the risk of choking.
In 1995, a medication called riluzole (Rilutek) was approved by the FDA as a treatment for ALS. Although this drug does not undo damage already suffered, it has been shown to extend the period of time before certain symptoms, such as swallowing or breathing difficulties, become disabling, and is a sign of hope about ongoing research. At present, the goal of treatment for patients with ALS is to provide them with as much physical and emotional comfort as possible, to help them adjust as they lose function, and to help them make sensible life choices.