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Motor Neurone Diease

Overview

Motor neurone disease is a type of condition that gradually damages the nervous system.  The disease is neurodegenerative in nature, thus increases disability in humans. It occurs when nerve cells in the spinal cord and brain called motor neurons stop working in the way they normally should. It is also known as amyotrophic lateral sclerosis (ALS)

Activities in which Motor Neurone Controlmotor neurone

  1. Walking
  2. Speaking
  3. Swallowing
  4. Gripping
  5. Breathing

Causes of Motor Neurone Disease

The cause of motor neurone disease remains unknown. Although according to statistics, about 5% of patients either have it in their family history or have similar related diseases such as frontotemporal dementia.   Cases are usually diagnosed by experienced neurologist through test of the brain and nervous system.  In recent times, neurologist has concluded that faulty genes are a major contributor to the development of the condition.

Progression of Motor Neurone Disease (MND)

MND symptoms start slowly on one side of the body initially and progresses. Below are early signs and symptoms of Motor Neurone Disease;

  1. Inaudible speech which worsens over time (dysarthria)
  2. Weak ankle muscles resulting in foot drop
  3. Weakness in grip causing difficulty in holding and picking up objects
  4. Shoulder weakness that makes arm lifting extremely difficult

motot neurone brainAs damages progresses, symptoms spread to other parts of the body and the conditions becomes devastating. Overtime, the patient of motor neurone disease will experience permanent mobility loss. Easy activities like breathing, swallowing and communication may become difficult.

Reports have also shown that up to 15% of the patients with the disease are associated with frontotemporal dementia. This type of dementia affects our personality and behavior.  Although, the affected person will not realize his/her behavioural change as the disease progresses gradually over months and sometimes years.

Who does Motor Neurone Disease affect?

Studies have shown that the disease affects about 2 in 100,000 people yearly in the UK. It affects all adult age groups, usually people over 40 years. Common symptoms are realized mostly by people in their 60s, affecting more men than women in the UK.  Also, there are some rare cases where teenagers are diagnosed with this condition. Estimated, there are about 5,000 people living with Motor Neurone Disease in the UK at any given time.

Treatment

There is no current cure for motor neurone disease. Treatments are usually palliative, aiming to compensate for the loss of mobility and bodily functions such as breathing and swallowing. Patients are usually given aiding equipment such as breathing masks and a gastrostomy (feeding tube). This helps with their overall nutrition and comfort.

In addition, medications are used to control excessive salivating. Also, medications such as ‘Riluzole’ has been found to slightly improve a patients overall survival rate. However, it doesn’t stop the progression of the condition.

Survival rates

Life expectancy for people with this condition is usually approximately 3 years from the start of the symptoms. It is known to be severely life shortening.  There are some rare cases whereby patients with this disease have lived up to 10 years and longer.motor neurone 2

Living with the Disease

For people living with the disease, life can be challenging with performing normal daily mobility activities both before and after diagnosis.  However, there are lots of specialist and community support to assist people living with the disease to maintain some independence and quality of life during these times.

In most cases, people with motor neurone disease usually die in their sleep due to the last stage of breathing muscles weakness. Hence, their end stage of life is not usually distressing as most people think.

Advice and support

There are many charities in the UK such as Motor Neurone Disease Association (MNDA) that provide support and assistance for people living with this disease. They provide information and practical advice on dealing with the emotional part of being diagnosed. Neurologist can be found in specialist hospitals across the UK.