ALS, or amyotrophic lateral sclerosis, is a fatal neurological disease that progresses rapidly throughout the body. It is sometimes referred to as Lou Gehrig’s disease for the baseball player that brought recognition to the disease prior to Stephen Hawking. It is a motor neuron disease that is classified by the death and degeneration of the motor neurons.
Signs of Lou Gehrig’s Disease
The very early stages of ALS are very subtle and can just be cramping, twitching, muscle stiffness or weakness, nasal-like speech, slurred speech, trouble swallowing, or trouble chewing. Later on there is more progressive muscle weakness and atrophy and can include falling and stumbling. As the weakness progresses even further there is trouble moving, speech difficulties, trouble forming words, and choking. The final stages of the disease will show the patient unable to walk and typically on a breathing ventilator.
There is not a simple test for ALS. It is a diagnosis based on symptoms and exclusions of other diseases. It may be wrongly diagnosed as Parkinson’s early on. There will be several neurological tests given to gauge the loss of muscle tissue and function.
One way to help diagnose ALS is through an EMG, or electromyography, test. This is a recording that gives electrical activity in the muscles. There are findings in this test that will show ALS or not.
Another test for ALS is the nerve conduction velocity test. Where there are abnormalities in this test will show any damage to the peripheral nerves, or peripheral neuropathy, or if there is muscle disease, or myopathy, instead of amyotrophic lateral sclerosis.
Treatment of Amyotrophic Lateral Sclerosis
While there is no cure for ALS there is one drug -riluzole- that the FDA has approved for use. It is for reducing the damage to the motor neurons. It will decrease the release of glutamate. Other than that, there are some ways to help prolong life by a few months. There is still more research and clinical trials that will need to be done to help further the treatment options of this fatal disease.
ALS is still a misunderstood disease with few treatment options and dire prognosis. Patients with ALS are in full control of their cognitive functions instead of the general belief that they will lose their memory and thought processes. They are in aware of what is going on around them, perhaps the saddest of all items in regard to ALS suffering.
Source: “ALS (Amyotrophic Lateral Sclerosis) Fact Sheet,” NINDS. Publication date February 2010. NIH Publication No. 10-916