Introduction to MND
Motor Neurone Disease is a rare and devastating condition that affects the neurons in the brain and the spinal cord. As the name Motor Neurone suggests, the degeneration of the neurons over time will have an adverse effect on the muscles that govern the motor skills (this includes both gross and fine motor skills).
In order for muscles to work they need electrical signals from the brain. Signals from the brain are carried by neurons that pass messages through the nervous system via the spinal cord. These messages are usually a response to stimuli. As neurons degenerate, the muscles receive fewer signals, which in turn weakens the muscles and they deteriorate (muscular dystrophy).
The effect of muscle weakness can affect the way a person walks, moves, grips objects, talks, eats, drinks and breathes. It can also affect cognitive ability, thought processes and emotional wellbeing. However, not everyone suffers the same symptoms, and some symptoms develop at a later stage than others. Additionally, the symptoms do not appear in any specific order.
The disease usually occurs within the 40-70-year-old age group. The initial sign of symptoms may be as simple as feeling overwhelmingly fatigued to the point where it can adversely affect daily life. People may also experience muscle weakness in the face and neck which can cause speech to become slurred and difficulty in swallowing which can have an impact on health.
Motor Neurone Disease Statistics
Roughly two people in every 100,000 will develop MND each year. MND affects 5,000 people in the UK at any one time.
After being diagnosed with MND, life expectancy is greatly reduced. Approximately one in three will die within one year and a further half of those will die within two years.
In the UK, statistics show that six people are diagnosed with MND every day, which is an annual rate of 2,200 people
Just over one-third of the people with MND will also experience cognitive changes that will cause difficulty with executive functions. This may affect their planning abilities; decision making and difficulties with language.
Additionally, 5-10% of people diagnosed with MND will also display signs and symptoms of frontotemporal dementia, which will cause behaviour changes that are more pronounced and debilitating.
Types of Motor Neurone Disease?
There are four main forms of MND and several even rarer forms. Each of the forms has a slightly different way in which it affects the human body. Additionally, there is a possibility of crossovers between two or more of the differing MND forms. So in practical terms, although it is useful to categorise it is not always possible to do so.
There no particular set test to diagnose the disease nor is there a particular identified cause of MND. However, in 5% of cases, there is a family history of either MIND or a condition known as frontotemporal dementia. This type of dementia is known to contribute to the development of MND.
Frontotemporal dementia is caused by shrinkage and damage to the frontal and the temporal lobes of the brain. Any damage to this area of the brain can result in deterioration of the neurons which in turn will result in few or no messages being sent to the nervous system and muscles.
An experienced Neurologist can usually detect and accurately diagnose the presence of MND. However, tests are still needed to rule out the presence of other conditions that may have similar signs and symptoms and subsequently may require a different treatment plan. Rarer forms of MND affect only 5% of the people who have already been diagnosed with the disease.
Amyotrophic Lateral Sclerosis
Amyotrophic Lateral Sclerosis (ALS) is the commonest form of MND. ALS is also known by another name ‘Lou Gehrig’s disease (or classical motor neurone disease). Like all forms of MND, it is progressive and ultimately fatal. Over time it eventually stops all of the neuron signals to all of the body muscles.
The term ALS seems to be used interchangeably with MND, even by doctors. In the USA, the term ALS is used instead of term Motor Neurone Disease. ALS is the form of MND that affects both the neurons in the brain and neurons in the spine.
This form of MND involves what is termed as the ‘upper neurons’ and the ‘lower neurons,’ which in effect causes loss of strength and muscle wastage in both the legs and the arms. Initially, it can cause frequent unexplained trips and falls or a reduced ability to grip objects. Life expectancy is usually 2-5 years after the symptoms have started.
ALS gained publicity during 2014, with the ALS ice bucket challenge.
Progressive Bulbar Palsy
Progressive Bulbar Palsy (PBP) affects both the upper and lower neurons. Generally, 20% of people who have been diagnosed with MND, will be further diagnosed with this type of the disease. The average life expectancy is between six months to three years after the symptoms manifest themselves.
The muscles that are first affected are usually the facial and neck muscles, the ones that are used for talking, chewing and swallowing. Symptoms include slurred speech, difficulty in swallowing and sometimes choking, either for no reason or on small pieces of food. Over time without adequate support and care, life can be very challenging.
Progressive Muscular Atrophy
Progressive Muscular Atrophy (PMA) is an uncommon but not rare type of MND. This type of MND only usually affects a few MND sufferers. Most people who have been diagnosed with this type of MND can usually live for up to five years after the symptoms first appear. It seems to affect mainly the lower motor neurones.
The first part of the body that is usually affected is the small muscles that are found in the hands and feet. One of the main symptoms is experiencing a loss of strength or manual dexterity with their hands. The muscles do not seem to be tensed or rigid (spasticity), but will have difficulty in gripping objects; opening jars or writing.
Primary Lateral Sclerosis
Primary Lateral Sclerosis (PLS) is also an uncommon form of MND. This type of MND affects the neurons that send messages to the lower limbs. As this type of MND affects mainly the legs, the muscles that help to breathe are not affected so life expectancy can be within normal limits, but if the disease progresses to ALS, then life expectancy is shortened.
Although PLS causes weakness mainly in the leg muscles, some people may also develop problems with the small muscles in their hands causing problems with manual dexterity. Additionally, their facial muscles may be affected which will cause them to slur their speech which will cause communication problems and frustration.
It is important to get a diagnosis as soon as possible to help relieve the symptoms, but also to plan ahead for specific support and care that will be needed. Especially if other diseases such as dementia or COPD are present.
Signs & Symptoms
Motor Neurone Disease can affect different people in different ways. Motor Neurones control muscle activity that we take for granted. Simple activities that may previously have required little or no effort will become difficult to the point where they are impossible to maintain without help and support.
The first symptoms are that are experienced may be in the hands and arms or the feet and legs. However, in other cases, it may be the muscles around the face and throat (bulbar muscles) are initially affected.
Hand and Arm symptoms. This can affect the ability to grip objects as reduced muscle control will affect the ability to hold onto things, open jars, turn keys and sew. This may appear to be similar to dyspraxia, but these difficulties would be new symptoms.
Feet and Leg symptoms. One of the first symptoms may be experiencing a problem with tripping, which may be caused unintentionally by dragging a leg. Problems with the muscles in the lower leg can cause problems getting out of low chairs and climbing the stairs.
Bulbar muscle symptoms. As these muscles affect the face and throat changes may include losing the ability to shout or sing. Changes may occur in voice quality and speech may become slurred. Difficulties with swallowing will make eating difficult.
Other symptoms may include: experiencing muscle cramps; feeling overly tired or finding simple activities may be exhausting; muscles that have already become weakened may twitch due to the interrupted neuron messages received; jerking of an arm or leg while at rest.
Progression of the disease
Initial symptoms may be experienced in just one leg or one arm, but as the disease progresses, it spreads to the other limbs and other muscle groups. Some muscle groups can be more affected than others. Observable signs may include:
- Walking becomes increasingly difficult to the point where it can be dangerous due to unexplained frequent trips or falls.
- Tasks that involve the use of hands and arms become more and more difficult. This can be life changing for someone who has worked with their hands all of their life.
- As the muscles around the mouth, tongue, and throat become weaker, it can cause difficulty in swallowing, which will affect the ability to communicate and to eat.
- Sneezing and coughing become weaker. This can cause problems as it can be difficult to clear the lungs due to mucus or irritation.
- As the chest muscles are also affected, any exertion may result in breathlessness and a feeling of total exhaustion. Breathing may become more and more difficult.
However, there are things that MND does not affect
Brain – Very rarely does MND impact on intellect. However, there are always exceptions to the rule, and in the very rare cases where there is a history of frontotemporal dementia or early dementia the brain may be affected.
Sensory System – Although the sensory system is fed by a system of nerves and nerve endings, it is not affected by MND. The senses of smell, sight, taste, and hearing are unaffected and likewise, the sense of movement and body awareness should remain unchanged.
Continence – It is very rare for incontinence to be experienced as part of MND, but due to progressive mobility difficulties, this could be a problem during the later stages of the disease.
Emotion – Although feelings and desires (including sexual desire) may not be directly affected by MND, depression and anxiety are widespread. It has been known that people with MND may laugh or cry involuntarily at inappropriate times, this can be embarrassing for the patient.
The test for MND – what to expect
Unlike many other diseases, no single test will indicate the presence of MND. Many of the early stage symptoms can be explained away by ageing, or they may be initially attributed to other diseases. The usual route for diagnosis is by referral to a Neurologist for assessment. Sometimes a diagnosis can only be made when the symptoms become more apparent.
However, by gathering a medical history and using some or most of the following tests, a diagnosis can be made or at least can help to rule out other causes of the symptoms.
Magnetic Resonance Imaging (MRI) scans – As these are very expensive and time consuming these are usually performed if there is a concern that the experienced symptoms may be due to another cause or to rule out any other disease.
Electromyography (EMG) – This test involves the use of small fine needles that are placed under the skin. These small needles help to record the nerve impulses that are taking place in the muscles particularly the ones in the arms, legs and throat.
Nerve Conduction Studies In this test small pads are applied strategically on the body. A small electrical impulse is applied. Measurements are taken to determine the speed at which the electrical signal affects particular muscles (involuntary movements).
Transcranial Magnetic Stimulation (TMS) – This test involves the measurement of the activity of the nerves that send messages from the brain and the spinal cord. This test can be beneficial in the diagnosis of the particular type of MND and its prognosis.
Palliative care focuses not just on the improvement of the physical quality of life but also on the enhancement of mind and spirit. It supports not just the person receiving the care but also helps enable loved ones to cope with the situation.
Who delivers Motor Neurone Disease Care?
A good care agency whose care philosophy is based on a person-centred approach can understand that every client is unique. A good care agency also promotes client independence, encourages a positive attitude by supporting them to achieve a high quality of life, by making choices rather than have choices made for them.
Not every care agency can provide such a range of support to meet the needs of clients who have severe conditions such as MND.
Live-in care has become increasingly popular as an alternative to moving into a care home, especially if being at home means that there are better resources that can accommodate changing health needs.
Assessment for MND Care
As the effects of progress, you may find that you will need help and support for everyday living. The assessment is informal and it involves a senior care manager meeting you in your own home. During this meeting, the care manager will get to know you as a person, how your illness affects you and the level of care that you will need initially.
Questions about your care
- What level of care do you think that I need at the moment?
- If my disease gets worse how quickly can you put in additional support?
- How often do you review my package of care?
- When my MND gets worse, will I be able to continue to live in my own home?
- Can you help me to look at the advantages and disadvantages of having a live-in carer?
- What expectations should I have about my quality of life if I have a live-in carer?
- How do I organise the funding for my care package?
- Would my carer be able to organise my medications and communicate with my GP for me?
- Can my carer help me attend hospital appointments?
- Will I get the chance to meet my carer before we make a decision?
What sort of care to expect?
The type of care that you can expect will depend on your need. Your care package may include:
- Help with personal care.
- Support with taking medication.
- Assistance with household task including gardening and looking after pets.
- Organising meals and doing shopping.
- Accompanying you when you attend medical appointments.
- Arranging for you to take part in social activities.
- Being an advocate. Being a companion.
- Look after you at night.
- If you live alone, by having a live-in carer will make sure that you have support at night when MND symptoms can be more troublesome.
You also need to know what plans are in place should your carer become unwell. You need reassurance about continuity of care.