Motor Neuron Disease

What is Motor Neuron Disease?

Motor neuron disease is a progressive condition which occurs when certain nerve cells degenerate and die. There are two types of motor neuron cells. The upper motor neuron begins in the brain and ends in the spinal cord. The lower motor neuron starts in the spinal cord and ends in the muscles. Nerve cell degeneration and death causes muscle weakness, abnormal reflexes and decreased ability of the brain to control muscle movement. The affected nerve cells do not grow back, but healthy nerve cells can attempt to reconnect to the muscles, which slows the progression of the disease. There are several types of motor neuron disease, including amyotrophic lateral sclerosis (ALS or Lou Gehrig's Disease), spinal muscular atrophy, Werdnig-Hoffman disease, and infantile spinal muscular atrophy. It only affects the skeletal muscles and does not affect automatic muscles like the heart.

Who gets Motor Neuron Disease?

Motor neuron disease can affect anyone, but most people are over the age of 40, and men are affected slightly more often than women. Approximately 2 in 100,000 people will get motor neuron disease. It does not seem to be caused by specific foods, lifestyles, or injuries. It is hereditary in some people (5-10%).

How is Motor Neuron Disease diagnosed?

After learning the patient's history and performing a physical examination, a physician may perform an EMG study. They may also use an MRI or blood studies to look for other diseases which can present like a motor neuron disease.

How is Motor Neuron Disease treated?

There is no cure for motor neuron disease. Only one medication is currently available, called Riluzole. It slows the progression of ALS by a small amount. Physical therapy and bracing can also help with symptoms.

More Information

University of Minnesota Department of Neurology
ALS Association
Motor Neuron Disease Association
ALS International Alliance