Motor neuron disease (MND) is a serious form of progressive neurodegeneration – over time the nerves in the spine and brain progressively lose its function. In the case of motor neuron disease, motor neurons – types of nerve cells – are affected. The renowned guitar virtuoso Jason Becker are living with motor neuron disease. There are different forms of motor neuron disease. ALS (amyotrophic lateral sclerosis) is the most common form and accounts for approximately 60% to 70% of all cases. PBP (progressive bulbar palsy) accounts for about 20% of all cases. PMA (progressive muscular atrophy) accounts for the remaining 10% of cases. In all three MND forms symptoms are very similar. However, they progress at different speeds.
PLS (primary lateral sclerosis) is a very rare form of MND. PLS, unlike the other forms, is not fatal. In some very rare cases, patients with PLS eventually have ALS. MND is a very rare condition that affects the nervous system (neurological condition). In India approximately 1 person in every 50,000 is diagnosed with MND each year—this statistic is more or less the same across geographies.
MND can affect patients of any age, but most of them develop the disease and are affected by it after the age of 40 (specifically between the ages of 50 and 70 years). Out of every 10 people with AMD, 6 are men and 4 are women. What are the risk factors for motor neuron disease? A risk factor is something that increases a person’s chances of developing a disease. For example, smoking increases the risk of developing some types of cancer; therefore, smoking is a risk factor for cancer.
Heredity – approximately 1 in every 10 people with ALS are known to have inherited it from their parents. A child who has a parent with MND has a 50% chance of developing the disease.
Age – after the age of 40 the risk of developing MND rises significantly (but is still very small).
Sex – men are much more likely to develop the disease before the age of 65 than women. After 70 years of age the risk is the same for both sexes.
Since ALS mimics the symptoms of several other similar neurological conditions and is, therefore, difficult to diagnose, doctors at BRAINS may want suspected patients to go through several tests essentially to rule out other possibilities. These may include electromyogram, nerve conduction study, MRI, blood and urine tests and muscle biopsy (see Technology & Procedures). Results from these diagnostic studies in combination with an expert analysis of a patient’s symptoms and medical history are often enough for the doctors to make a firm diagnosis.
Since there’s no reversing the course of amyotrophic lateral sclerosis, treatments mainly involve efforts to slow the progression of symptoms and make you more comfortable and independent. The drug riluzole (Rilutek), the first and only medication approved by the Food and Drug Administration for slowing ALS, appears to slow the progress of the disease in some people, perhaps by reducing the levels of glutamate — a chemical messenger in the brain that’s often elevated in people with ALS. Your doctor may also prescribe medications to provide relief from many of the symptoms of ALS such as muscle cramps, constipation, fatigue, excessive salivation, excessive phlegm, pain and depression.
In addition to the drugs, a physical therapist at BRAINS may recommend low-impact exercises to maintain your cardiovascular fitness, muscle strength and range of motion as long as possible, helping you preserve a sense of independence. Regular exercise can also help improve your sense of well-being. Similarly, an occupational therapist can help you become accustomed to a brace, walker or wheelchair and may be able to suggest devices such as ramps that make it easier for you to get around. Since ALS affects the muscles you use to speak, communication becomes an issue as the disease progresses. A speech therapist can help teach adaptive techniques to make your speech more clearly understood or help you explore alternative methods of communication, such as an alphabet board or simple pen and paper.
Electromyogram: This test measures involves the insertion of fine electrode into muscles to help doctors study the electrical activity inside them. This activity is recorded by an instrument as you rest and contract the muscles. Generally, this test is mildly uncomfortable.
Nerve conduction study: For this test, electrodes are attached to your skin above the nerve or muscle to be studied. A small shock, which may feel like a twinge or spasm, is passed through the nerve to measure the strength and speed of nerve signals.
MRI: Using radio waves and a powerful magnetic field, an MRI can produce detailed images of your brain and spinal cord. It involves lying on a movable bed that slides into a tube-shaped machine that makes loud thumping and banging noises during operation.
Blood and urine tests: Analyzing samples of your blood and urine in the laboratory may help your doctor eliminate other possible causes of your signs and symptoms.
Muscle biopsy: If your doctor believes you may have a muscle disease rather than ALS, you may undergo a muscle biopsy. In this procedure, a small portion of muscle is removed while you’re under local anesthesia and is sent to a lab for analysis.