Is there treatment for Motor Neuron Disease

The treatment of motor neuron disease can be divided in to treatment of the disease itself and treatment of the symptoms that occur as a result of the disease- addressing both at the same time is very important.

 

Treatment of the disease:

1. A medication called riluzole has been shown to slow the progression of motor neuron disease. The effect is modest and may prolong life by a few months. It is a medication that is thought to reduce the damage to the motor neurons that is caused by certain neurotransmitters such as glutamate. The dose is 50mg twice daily. It is generally well tolerated and the main limiting side effects are gastrointestinal such as nausea and vomiting.

 

2. A medication called edavarone is also used to treat motor neuron disease. This works differently to riluzole. It acts to reduce “free radicals” within the nervous system that are thought to mediate some of the damage of nerve cells that occurs in motor neuron disease. Again, the benefit of this medication is modest at best. This drug is given by intravenous infusion every day for 14 days followed by a 14 day break. It has only recently come on the market. There are limitations with it in terms of how it is given. Also in the clinical trials only certain types of patients with motor neuron disease were included and so applying this to the general population of patients with motor neuron disease is more challenging.

 

 

Treatment of the symptoms that arise from the disease:

1. Breathing problems:

Motor neuron disease can affect the muscles involved in breathing. It tends to do this much later in the disease. However, because it happens commonly in late stages of the disease it is something that should be addressed early on. Most patients after receiving the diagnosis will go on to have “baseline” tests of breathing function. This test will then be repeated every 3- 6 months depending on the patient and clinician. When it comes to the point in the illness that breathing is affected there are several options depending on the patient’s wishes. The first stage would be the non-invasive forms of ventilation. This means the breathing can be supported by the use of a mask that is applied to the face and is therefore “non-invasive”. This can be worn exclusively at night or if necessary also throughout the day. The masks can be quite uncomfortable, however, over time more masks have become available so it is worth trying a few before deciding that you cannot tolerate it. Also, there can be some anxiety surrounding the need for the mask so often treatment with a low dose of medication to counteract this anxiety can make the mask more tolerable. Eventually the mask will no longer be sufficient to support breathing. At this point the patient, family and physicians will have a discussion regarding ventilation with a tracheostomy (tube in the neck to which a breathing machine or ventilator is attached). The decision to proceed with this will be entirely up to the patient and family and will likely take in to account the advice from the physician in terms of what to expect. As stated above by the time this stage in the condition is reached these decisions will already have been made. However, the patient should know that they can always change their mind to either have ventilation or not at any point.

 

2. Difficulty Swallowing:

Motor neuron disease can affect the muscles involved in swallowing. This can lead to a number of problems. Firstly at a practical level if a patient is unable to swallow adequately the intake of nutrition will be poor. Therefore there may be weight loss which is not desirable in this condition. It can also be distressing for the patient in terms of episodes of choking or feeling as if they are going to choke. Therefore getting swallow function assessed early in the course of the disease is advisable. Adjustments can then be made to the consistency of food that is eaten. If adequate nutrition is not being met then dietary supplements may be used. If this is still inadequate the patient and family may consider a tube that goes directly in to the stomach. This is a useful way of maintaining adequate weight and nutrition. The decision to go for this or not should be made only after exploring all the potential risks and benefits with your neurologist.

 

3. Difficulty Speaking:

Motor neuron disease can also affect the muscles involved in speaking. This can lead to speech becoming difficult to understand. There are electronic assistive communication devices that can be used with either hand or eye controls that may improve quality of life for both the patient and carers.

 

4. Fatigue:

Fatigue is a commonly encountered symptom in motor neuron disease. It can be difficult to treat. Assessing for contributors such as poor sleep, pain and low mood is useful. There are some amphetamine based medications that may provide some symptomatic relief of this symptom.

 

5. Muscle Spasms:

Muscles spasms can be a source of pain and discomfort in motor neuron disease. A medication called quinine can be useful for treating this particular symptom. The spasms are often worse at night so this medication can be taken just at night if the patient wishes.

 

6. Muscle Weakness and Functional Decline:

These symptoms are hallmarks of motor neuron disease. Assistive devices such as walking aids, crutches and ankle braces may be helpful. Other practical things such as elevating the toilet seat and bathtub lifts to try and maintain some independence may be worth considering. Specialised eating utensils with better grips and holders for drinks can also be of practical value. Pressure relieving mattresses may help prevent pressure sores which can be a source of pain.

 

7. Drooling:

This can also occur in motor neuron disease. There are certain medications that can help dry up secretions and thereby reduce drooling.  If these do not work botox can be injected in to the salivary gland to reduce the drooling.

 

8. Pain:

A combination of reduced mobility, muscle spasms and spasticity as well as cramping are all potential sources of pain in motor neuron disease. The important thing is to identify the nature of the pain and where it is coming from. If the pain is primarily muscular a combination of physiotherapy and pain killers will give the best pain relief. If the patient is unable to mobilise this can cause pain from muscle stiffness and frequent changes in position will help minimise this form of pain. Other practical measures such as special mattresses and pillows can also be of use.

 

9. Mood issues:

Having a chronic illness such as motor neuron disease can have a dramatic impact upon mood and patients will often suffer from depression and anxiety. Addressing these issues early is important. Identifying the nature of the stressor is important. For example, is the patient concerned re finances for the family or anxiety regarding how family members are coping with the diagnosis or are they struggling to cope with the diagnosis themselves? There may be many other issues contributing to the low mood. Identifying these will help with management. Medication to treat depression can also be very helpful here.

 

10. Sleep:

Sleep difficulty is common in motor neuron disease. It may relate to anxiety, pain, inability to change posture or weakness and muscle cramps. Exploring the most likely contributing factor will help with management of the symptom.