Motor Neurone Disease MND

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What is MND

Motor Neurone Disease (MND) is the name given to the group of diseases in which the motor neurones undergo degeneration and die. Motor neuron diseases cause the nerves in the spine and brain to lose function over time. They are a rare but severe form of neurodegenerative disease.

Amyotrophic Lateral Sclerosis (ALS), Progressive Muscular Atrophy (PMA), Progressive Bulbar Palsy (PBP) and Primary Lateral Sclerosis (PLS) and Spinal muscular atrophy (SMA) are all subtypes of motor neurone disease.

Although MND is the widely used generic term in the United Kingdom, Australia and parts of Europe, ALS is used more generically in the United States, Canada and South America.

MND is also known as Lou Gehrig's disease in the US after a famous baseball player who died of the disease. The renowned English physicist Stephen Hawking lived with ALS for many decades until his death in March 2018. 

The below video is a brief overview of the condition and the NICE guidelines on MND

Risk Factors

MNDs

• occurs in adults or children, depending on the type.
• more likely to affect men than women.
• inherited forms of the condition may be present at birth
• most likely to appear after the age of 40 years, usually between 55–75 years.
• The various types may have different risk factors. SMA is always hereditary, but this is not true for all forms of MND.
• According to NINDS, around 10% of ALS cases in the United States are hereditary.
• veterans appear to have a 1.5–2 times higher chance of developing ALS than non-veterans. This may indicate that exposure to certain toxins increases the risk of having ALS.
• A 2012 studyTrusted Source found that footballers have a higher risk of dying from ALS, Alzheimer's disease, and other neurodegenerative diseases, compared with other people. Experts think that this could indicate a link with recurrent head trauma

Clinical Presentation

Early symptoms may be mild and include:

• Stumbling due to weakness of the leg muscles
• Difficulty holding objects caused by weakness of the hand muscles
• Slurring of speech or swallowing difficulties due to weakness of the tongue and throat muscles
• Cramps and muscle twitching

As the disease progresses symptoms may include:

• Breathing difficulties from decreased lung capacity caused by muscle weakness
• Fatigue caused by muscle exhaustion, decreased lung capacity, metabolic changes, weight loss and reduced food intake
• Insomnia caused by discomfort, pain from stiff joints and muscles, excessive saliva, dry mouth or breathing problems
• Mild changes in cognitive skills and processes and/or behavioural change
• Fronto-temporal cognitive changes (a type of dementia), which is prominent in 5-10% of MND cases
• Excessive laughing or crying due to damage to the upper motor neurones
• Some pain or discomfort
  

Diagnostic Procedures

Motor neurone disease (MND) is difficult to diagnose as the initial symptoms can be similar to many other conditions.

Neurologists use a series of tests to eliminate other conditions before making a definitive diagnosis of MND.

Tests may include:

• Blood tests to look for a rise in a creatine kinase, which is produced when muscle breaks down.
• Nerve conduction studies (NCS), which involve taping electrodes over nerves and recording muscle activity when nerves are stimulated by electrical impulses.
• Electromyography (EMG), which involves inserting a needle electrode into muscles to measure their electrical activity.
• Magnetic Resonance Imagery (MRI) scans, which involve being placed in a cylinder-like machine. The machine takes images of the internal structures of the body and can show up damaged areas. An MRI scan will not diagnose Motor Neurone Disease, as the damage caused by this disease does not show up on this scan. However, it may be used to eliminate other conditions which can mimic symptoms of MND.

Physiotherapy Management

There is no cure or standard treatment for the MNDs.  Symptomatic and supportive treatment can help people be more comfortable while maintaining their quality of life.  Multidisciplinary clinics, with specialists from neurology, physical therapy, respiratory therapy, and social work are particularly important in the care of individuals with MNDs.

Physical therapy rehabilitation may help to improve posture, prevent joint immobility, and slow muscle weakness and atrophy. 

• Stretching and strengthening exercises may help reduce spasticity, increase range of motion, and keep circulation flowing. 
• Applying heat may relieve muscle pain. 
• Assistive devices such as supports or braces, orthotics, speech synthesizers, and wheelchairs may help some people retain independence.