Motor neurone disease | Hemonga iaia

Motor neurones are nerve cells in your brain and spinal cord that carry signals to make your muscles move. In MND, these motor neurones deteriorate and die. This causes your muscles to weaken and your brain to lose control over your voluntary muscle movements.

In about 10% of cases, MND has a known family link where others in the family have had MND and there are inherited gene mutations. This is called familial MND. 

However, most cases of MND occur in people without a family history of the disease. This is known as sporadic MND. Occasionally, people with sporadic MND can have a gene mutation that  can then be inherited. Free genetic testing is available if you would like to know if you have 1 of the more common gene mutations that can be inherited. Ask your neurologist or healthcare provider. 

Although there can be some overlap in symptoms, generally there are 4 main types of motor neurone disease (MND). Each type starts in a different part of your body and the pattern of weakness indicates the involvement of different motor neurones.

Amyotrophic lateral sclerosis (ALS)

• ALS is the most common type of MND. It involves both your upper and lower motor neurones.
• Key symptoms of ALS are muscle weakness and stiffness, overactive reflexes and, for some people, quickly changing emotions.
• The muscles in your limbs get affected first and then later the muscles you use for speech, swallowing and breathing are usually also affected. Often it starts in 1 limb (eg, 1 arm or hand, or 1 leg or foot) such as with tripping or hand or arm weakness. Then it moves on to include other limbs.

Progressive bulbar palsy (PBP)

• PBP affects about a quarter of people diagnosed with MND and involves both your upper and lower motor neurones.
• It affects the muscles you use for speech and swallowing. The nerves that control these functions are in the bulb (the lower part of your brain), hence the term bulbar, and palsy means paralysis.
• Later, your limb muscles may also be affected.

Progressive muscular atrophy (PMA)

• PMA affects only a small proportion of people with MND. It progresses more slowly and has a longer survival period than ALS and PBP.
• PMA mainly causes damage to your lower motor neurones. Weakness or clumsiness in a hand may be an early symptom.
• Other symptoms include absent reflexes, muscle wasting and weakness.

Primary lateral sclerosis (PLS)

• PLS is a rare form of MND involving the upper motor neurones only.
• This mainly causes weakness in your lower limbs, although some people may experience clumsiness in their hands or speech problems.
• With PLS, there is a longer life expectancy, with an average of over 15 years. However, sometimes PLS develops into ALS.

The early symptoms of MND are usually mild and develop over time – it progresses faster for some people than others. The pattern of weakness is different for each person.

There are 2 types of motor neurones:

• Lower motor neurones control movement in your arms, legs, chest, face, throat and tongue. When signals from your lower motor neurons don't get through clearly, your muscles no longer work properly. They gradually weaken, and may begin wasting away and you may develop muscle twitching and cramps.
• Upper motor neurones enable you to produce movements such as walking or chewing. When signals for the upper motor neurones don't get through clearly, your limb muscles develop stiffness and your movements become slow and require a lot of effort. Your tendon reflexes may become overactive, leading to strong knee and ankle jerks when a healthcare provider tests them. Over time, you may lose the ability to control voluntary movements (movements you choose to make such as lifting your arm).

Common early symptoms include:

• a weak grip, which can make it hard for you to pick up or hold objects
• weakness in your shoulder making it hard to lift your arm
• a ‘foot drop’ caused by your muscles getting weaker
• dragging your leg
• slurred speech.

As the nerve damage progresses, symptoms spread to other parts of your body and you increasingly find it hard to move, speak, swallow and even breathe. 

Up to a third of people with MND will have some reduced ability in thinking and behaviour. This is due to changes in the frontal and temporal lobes of the brain. A small number (about 15%) will develop a form of dementia. This is usually called 'fronto-temporal dementia' and it may make concentrating and thinking more difficult.

If you suspect you have symptoms of MND, it’s important to see your healthcare provider about them. Early diagnosis can help with management of your symptoms. 

There’s no one test used to diagnose MND, so other conditions need to be ruled out.

If your healthcare provider thinks you might have MND, they will refer you to  a brain and nervous system specialist (neurologist). A diagnosis will be made based on your history, a physical examination and some specialised tests.

Tests may include:

• a needle test called electromyography (EMG)
• an electrical impulse test called a nerve conduction test
• an MRI scan 
• blood tests
• occasionally, a lumbar puncture (LP) where a needle is inserted into your lower spine to remove some spinal fluid for testing. 

MND is a complex disease and people experience different symptoms, and progress at different rates.

Currently there's no cure for MND, so treatment aims to:

• relieve your symptoms
• help keep you mobile and safe (eg, with a wheelchair when it's needed)
• help you communicate well
• help you breathe
• help you eat
• make you feel more comfortable.

Medicines can be used to help with symptoms such as muscle cramps, constipation, drooling and muscle twitching. There are also aids to help you with breathing, communication and swallowing difficulties. Your healthcare provider or MND regional support advisor can help you to access these. Read more about equipment you may find useful if you have MND.(external link)

A medicine called riluzole (also called Rilutek) is available in Aotearoa New Zealand for people with ALS. It’s not suitable for everyone. Different studies show this medicine may help increase survival by an average of 6 to 19 months. Riluzole doesn’t stop the disease but slows its progress somewhat.

To support you in managing your condition, a range of healthcare providers are likely to be part of your healthcare team. These may include your GP, specialist doctors, physiotherapists, occupational therapists, speech and language therapists, nurse specialists, district nurses, dietitians and counsellors.

You are likely to feel grief and need emotional support as you come to terms with your diagnosis. It’s important that you seek support from whānau and friends, as well as from experts in MND (see the support section below).

Being well informed about the condition is also an important way to support yourself to live as well as possible with MND.

Advance care planning is the process of thinking about, talking about and planning your ongoing health care, including end-of-life care. It’s important to talk about these things early on as, over time, your ability to communicate clearly is affected. Read more about advance care planning. 

You may want to consider voice banking which is where you record your natural voice (eg, on your phone) because many people lose their speech. This is very easy to do – you record phrases that will later be used to generate a voice that sounds like yours. You can also record special phrases you may want to use in the future. It's good to voice-bank as soon as possible. Read more about voice banking(external link).

Most people with MND live for 2 to 3 years after diagnosis, but there's a wide range, and it's very difficult to predict what will happen for an individual. About 5–10% of people with MND live for 10 years or more, others can live for less than a year.  

There's a lot of variation in how quickly MND progresses. In rare types of MND, progress may be slower and the outlook better than in ALS which is the most common form of MND. 

Because the progression of MND is so different for different people it's important to do what you want to do as soon as you can and not wait too long.

Motor Neurone Disease New Zealand(external link) (MND NZ, 0800 444 474) is an association that supports you to manage your condition. A support worker from MND NZ will help you to access care so you can live your life as fully as possible. 

MND NZ can help people with MND and their families to make contact with others who are in similar circumstances. It also provides advocacy, best practice care and supports research, including a patient registry, so we can understand more about MND in Aotearoa New Zealand. Read more about the patient registry(external link).

Regional MND support advisors from MND NZ provide free personalised support for people living with MND and their whānau. They can help you:

• navigate the health and community services in Aotearoa New Zealand
• connect with appropriate healthcare providers (eg, physiotherapists, occupational therapists and community nurses)
• get support from government agencies – this may include accessing:
  • a supported living payment
  • a carer support subsidy
  • funding for vehicle or housing modifications and specialist equipment services.

The following video shows how home modifications can help people with motor neurone disease live safely at home. Note that it’s an Australian video and the contact number for MND NZ is 0800 444 474.

Video: Maintaining independence at home with MND

Because MND is a life-limiting condition, palliative care will also be available.

Video: Motor neurone disease Julie's story | NHS

(NHS, UK, 2010)

Video: Graeme's MND story

(MND NZ, 2018)

Video: Kirsty's MND story

(Leeds Hospitals Charity NZ, 2018)

Video: My MND Diagnosis – Sam Tooze

(MND Association, 2020)

Video: Neil Ladyman talks about living with MND