WHAT IS MOTOR NEURON DISEASE
Motor neurone disease (MND) is a progressive neurological condition. It is a group of diseases that affects the nerves (motor neurons) in the brain and spinal cord that tell muscles what to do. In an individual with MND, the messages from these nerves gradually become more impaired and eventually stop reaching the muscles, causing them to weaken, stiffen and waste away. MND can affect how you walk, talk, eat, drink and breathe. Some people also experience changes to their thinking and behaviour. However, MND affects everyone differently. Not all symptoms will affect everyone, or in the same order. Symptoms also progress at varying speeds, which makes the course of the disease difficult to predict.
The disease will progress, which means symptoms will get worse over time. For some people this can be rapid, for others it is slower. There is currently no cure for MND, but your doctors and other health and social care professionals can help you to manage symptoms and remain independent for as long as possible.
TYPES OF MOTOR NEURON DISEASE
There are four main types of MND, each affecting people in different ways. However, it is often difficult to make an exact diagnosis, as they share some of the symptoms.
Amyotrophic lateral sclerosis (ALS): is the most common form, with weakness and wasting in the limbs, muscle stiffness and cramps. In the early stages a person may trip or drop things.
Progressive bulbar palsy (PBP): affects fewer people than ALS, and in the early stages tends to appear in the muscles of the face, throat, and tongue. The individual may notice slurred speech or difficulty swallowing.
Progressive muscular atrophy (PMA): affects fewer people than ALS and is usually slower to progress or worsen. The individual may notice weakness, diminished reflexes, or clumsiness in their hands.
Primary lateral sclerosis (PLS): affects fewer people than ALS and is usually slower to get worse. This causes weakness in the lower limbs, although the person may also experience clumsiness in the hands or speech problems.
HOW MOTOR NEURON DISEASE IS DIAGNOSED
If the doctor suspects a neurological problem (something that involves the brain and nervous system), a referral to a neurologist will be made, who will conduct a number of tests. These tests can range from blood samples to various nerve tests to check the symptoms are not being caused by anything else. Following the test results, a diagnosis of MND is made, but it can take time before doctors feel sure about the cause of the symptoms.
MND is difficult to diagnose as:
- It is a rare disease and early symptoms such as clumsiness, weakness or slightly slurred speech could have other causes.
- It can be some time before the person goes see a doctor as initial signs may not be severe.
- Not all symptoms happen to everyone or in the same order.
- Testing can only prove that you do not have other conditions, as there is no specific test for MND
SPEECH AND COMMUNICATION IN MND
Speech problems occur in more than 80% of people living with MND as the disease progresses. Approximately one third of people with MND experience bulbar motor neuron disease, a particularly aggressive form affecting speech and swallowing muscles early in the disease process.
Speech can deteriorate over a period of a few months and is cited by people living with MND as one of the most problematic symptoms. Fatigue is a significant symptom of MND and this can cause an individual’s speech to deteriorate as the day progresses.
This can result in slurred speech or indistinct speech that is one level of loudness and/or pitch. Harsh or strangled voice quality, nasal regurgitation – food or liquid coming through the nose when swallowing. Food or liquid falling from the mouth or drooling due to weak lip closure. Difficulty chewing food or forming it into an easy to swallow bolus due to weakness. Poor ability to create pressure in the oral cavity which requires many swallows to completely clear.
fatigue, an individual’s swallow may deteriorate as the day progresses. A management plan that includes diet modification and positioning may be recommended at varying stages of the disease.
Speech therapists help to minimise the physical, emotional, and psychological effects of swallowing difficulties. They promote quality of life and safety in eating, drinking, swallowing and saliva management.
Furthermore, speech therapists collaborate with other members of the multidisciplinary team such as dietitians, doctors, and nurses to help advise patients when feeding tube placement would be appropriate and that this occurs in a timely manner.
SUMMARY
- MND often has an impact on speech and swallowing functions.
- The multidisciplinary team caring for people living with MND should assess, manage and review a range of areas including swallowing, speech and communication frequently as the disease progresses.
- The multidisciplinary team should include speech therapists and assessment and review of swallowing should occur without delay to prevent frequent hospitalisations for pneumonia, dehydration, and/or dehydration.
- Counselling and decision-making regarding tube-feeding should be provided by the speech therapist in conjunction with other team members.
- In order to preserve active participation in communicative interactions AAC equipment that meets the needs of the person should be provided without delay to maximise participation in activities of daily living and maintain quality of life.
- All discussions should be tailored to the person’s needs, taking into account their communication ability, cognitive status, and physical abilities.