With MND, muscles in the face, mouth and throat can become weak With MND, muscles in the face, mouth and throat can become weak. These are known as bulbar muscles. Bulbar weakness can affect speech – dysarthria…. …and swallowing - dysphagia (more detail on following slides).

80% of people with MND are affected with slurred, quiet or complete loss of speech. These speech difficulties are termed dysarthria. Dysarthria is caused by weakness in the tongue, lips, facial muscles, pharynx and larynx. Early referral to a speech and language therapist (SLT) is recommended for assessment and strategies to aid communication. SLTs can arrange for assessment and provision of augmentative and alternative communication (AAC), which could include high or low tech options. Early referral to an Occupational therapist (OT) is also recommended. OTs can assist with advice on seating and positioning, environmental controls and mobile arm supports to access communication aids.

The family/carer often become expert at communicating with the person with MND. Sometimes they can manage pen and paper, or a boogie board. E-tran frame – used to spell out words by looking at each letter required.

Lightwriter – converts text to speech.

iPads or other tablets – different apps are available to convert text to speech.

EyeGaze – high tech option, more appropriate for longer periods of use EyeGaze – high tech option, more appropriate for longer periods of use. Can be used to generate speech, control the environment (eg central heating, TV, lights), surf the web, answer emails etc.

Swallowing problems (also called dysphagia) affect at least two-thirds of people with MND…. … resulting in dehydration from not drinking enough…. … and weight loss from not eating enough.

Many people with MND experience episodes of coughing and choking Many people with MND experience episodes of coughing and choking. Choking episodes are very distressing for people with MND and their carers but they can be reassured that choking is rarely a direct cause of death. People with MND produce a normal amount of saliva every day, however many people with MND report a feeling of excess saliva due to poor lip seal and/or difficulty in swallowing. Dietitians, physiotherapists, occupational therapists and speech and language therapists all have a role in assessing, managing and treating swallowing problems. Medications and treatments are available for saliva management, eg hyoscine or glycopyrrolate. Nutritional support for people with swallowing problems should be considered.

Monitoring nutritional intake and weight is important Monitoring nutritional intake and weight is important. Unintentional weight loss and risk of malnutrition can result from a range of factors including increased time taken to finish meals, reduced ability to hold utensils, fatigue etc. Early involvement of a speech and language therapist and dietitian is essential to assess, monitor and review nutritional intake and provide advice. Advice may include: Dietary enrichment to optimise nutritional content Nutritional supplements Practical solutions to ease food preparation and eating/drinking problems. Percutaneous endoscopic gastrostomy (PEG) Radiologically inserted gastrostomy (RIG) Percutaneous Inserted Gastrostomy (PIG) Nasogastric tubes (NGT) – these are usually only used short term. Not everyone with MND will choose to have this type of intervention and their decision should be respected. Explanation of the pros and cons will give people the chance to make an informed decision.

This man is being fed via his PEG.

When the messages from the motor neurones reduce, the muscle they connect to is used less and tends to decrease in mass (known as wasting). Muscle wasting is one of the most common symptoms of MND. Muscle wasting in the limbs causes weakness … … and can affect balance and posture, increasing the risk of falls. Loss of dexterity may lead to difficulties with daily activities, such as holding a glass or buttoning a shirt. Fasciculations are twitching and rippling sensations under the skin, which are sometimes visible. They are often the first signs of MND. General muscle tightness (stiffness, rigidity and spasms) can affect mobility and co-ordination. May cause pain or increase the risk of falls.

This slide lists some of the common signs of respiratory insufficiency. Weakness of the diaphragm and accessory muscles can cause respiratory insufficiency (the lungs cannot take in sufficient oxygen or expel sufficient carbon dioxide to meet the needs of cells). A person with MND may easily get short of breath, especially when lying flat.

Ongoing monitoring of symptoms is important so that timely interventions can be made to improve/maintain quality of life for as long as possible. Assisted ventilation - non-invasive ventilation is a method for providing ventilator support to a person with MND using a mask that goes over their nose/mouth. Studies have shown it improves quality of life and sleep quality, and also increases survival time. Invasive ventilation by tracheostomy – this can be controversial and some units may be reluctant to offer an elective tracheostomy for IV. People with MND may not be able to cough effectively. They may be helped by breath stacking, or by using a lung volume recruitment bag or a machine known by the brand name CoughAssist. A respiratory phyiotherapist or respiratory nurse can suggest the most appropriate cough management regime for the person with MND and teach techniques to help them to cough more effectively.

There is now an increased awareness of cognitive and behavioural changes in MND, including frontotemporal dementia. For many people the changes will be subtle and have little or no effect on daily life, but a small number of people will develop frontotemporal dementia and need additional support. Some people experience emotional lability (also known as pseudo-bulbar affect) – presents as disturbed emotional responses including inappropriate and uncontrollable laughing and crying. It is a common problem, and can occur in people with cognitive change, but is not necessarily a sign of wider cognitive changes. Can be very difficult for people with MND and those around them.

Recent research has shown that the number of people with MND who are unaffected by cognitive change reduces dramatically in the final stage of the disease. Crockford C et al. ALS-specific cognitive and behaviour changes association with advancing disease stage in ALS. Neurology. 2018; 91(15):e1370-e138.

Not all of these problems may be evident and they can vary in degree Not all of these problems may be evident and they can vary in degree. Changes may be subtle and may be masked by movement and speech problems.

It is the only licensed drug available in the UK that prolongs life in MND and clinical trials have shown that on average it extends survival by around three to six months if taken for 18 months. How does riluzole work? Muscles are controlled by electrical messages sent from the brain, which are transported through the motor neurone system. These electrical messages are transmitted from one motor neurone to the next via the release of a chemical messenger. For many motor neurones this chemical messenger is a chemical called glutamate, but high concentrations of glutamate are toxic. Riluzole is thought to work by suppressing glutamate activity. However, riluzole has many other actions too. The anti-glutamate effect may be a reason why it provides some benefit for people with MND, but this is not yet proven. It is available in tablets or liquid form for those who have trouble swallowing. Recommended for people with MND by NICE although generally a hospital consultant will assess a person’s suitability. Normal baseline blood tests should be undertaken, as well as liver function tests.