Motor Neuron Diseases – MND

Motor Neuron diseases, or MND, are uncommon conditions that usually only cause about 20,000 cases in the United States per year.  These diseases affect the brain and the nerves. 

The motor neurons in the body are nerve cells that are in charge of sending messages around the body to make movement.  There are two types of these motor neurons, the upper motor neurons and the lower motor neurons.  The upper motor neurons are in charge of sending messages from the brain to the spinal cord.  The lower motor neurons are in charge of taking the messages sent from the brain to the spinal cord and sending them to the muscles.  When these neurons are damaged or die then messages can no longer make it to your muscles.  This will cause your muscles to become weaker.  This is what happens if you suffer from a Motor Neuron disease. 

These diseases have no treatment, and can shorten your life expectancy.  They usually affect adults ages between 40-60, but they can affect anyone.  Who gets these diseases seems to be sporadic, not causing you to be more at risk if you have someone in your family with one. 

Symptoms 

Symptoms progress overtime.  Usually starting off rather gradual causing them sometimes hard to pick up on.  Typical symptoms will start in the arms, legs, mouth, or respiratory system.  Some of the beginning symptoms could be weakness in your ankle or leg causing it to be a little harder to climb stairs.  Slurred speech can be a first symptom.  Muscle cramps, twitches or pains can also be the first sign of these diseases.  Weak grip, weight loss, trouble breathing, shortness of breath, or difficulty swallowing can all be early symptoms as well.  Another symptom of some of the Motor Neuron diseases is the inability to stop crying or laughing at inappropriate times.  

As the diseases progress your symptoms will become more severe.  Usually by the end people with these diseases will need to be cared for completely.  Progressive symptoms can include muscle shrinkage, difficulty moving, joint pain, drooling, uncontrollable yawning, jaw pain, memory or language problems.  

Diagnosis

Motor Neuron diseases are hard to diagnose.  There is no one test that can identify if you have one of them or not.  Your doctor may need to do a few tests to eliminate other things.  Your doctor may want to do blood tests, a brain or spine scan, an electrical activity test in your muscles or spine, lumbar puncture, or a muscle biopsy.  Depending on how advanced the case of MND is will depend on how many tests your doctor may have to do.  They will want to rule out other things that could be causing your symptoms.  

Types of MND

There are different types of MND, some more common than others.  The most commonly known one is ALS, Amyothropic lateral sclerosis.  This disease affects both the upper and lower neurons.  Gradually you can lose control of walking, talking, chewing, swallowing, and breathing.  ALS can cause stiffness and twitches in the muscles.  It does seem to be sporadic in who gets this.  Normally symptoms don’t occur until around age 40 to 60.  Once symptoms have started though usually people only live for 3 to 5 years after.  

PLS, or Primary Lateral Disease, is another MND.  It only affects the upper neurons, and like ALS usually starts around the age of 40.  You won’t die from this disease though.  It can cause weakness or stiffness in arms, legs, cause slowed walk, poor coordination, balance, or speech may become slow or slurred.  

PBP, Progressive Bulbar Palsy is when the neurons in the brain stem are damaged.  This disease usually will lead to ALS.  It causes you to have the inability to control your emotions, causing you to laugh or cry out of nowhere.  This can also cause slurred speech, trouble talking, difficulty chewing or swallowing.  

Pseudobulbar Palsy is similar to PBP but won’t lead to having ALS.  The damaged neurons affect talking, chewing, and swallowing.  As well as laughing or crying without control. 

Progressive Muscular Atrophy can be either inherited or sporadic.  It only affects the lower motor neurons.  This usually turns into ALS as well.  First weakness in the hands starts then weakness will spread throughout the body.  The muscles will weaken and cramp. 

Two other types of Motor Neuron diseases are Spinal Muscular Atrophy, and Kennedy’s disease.  Most of these types are rare.  

Treatment

There is no cure for Motor Neuron disease.  Treatment though can help slow progression of the disease as well as increase quality of life.  Allowing the person with the disease to stay more independent for as long as possible.  Treatment is truly dependent on type, symptoms, severity of symptoms, personal choice, as well as affordability of drugs and treatments. 

Some treatment options are physical therapy, speech therapy, and occupational therapy.  There are certain drugs that can help slow the progression of different types of MND.  Botox injections can be used for stiff muscles.  Pain relief, either over the counter, or narcotic depending on the pain scale.  Antidepressants can also be given if needed. 

Though there is no cure, there are ways to treat your symptoms and try to keep your quality of life up.  Some people can live longer after symptoms have started though most MND will shorten life spans.  Different types of MND affect different nerve cells.  These are all very rare and seem to be sporadic with no way of preventing them or knowing if you have a higher risk of getting them.