e) Degenerative diseases

Degenerative neurological diseases

Degenerative neurological diseases cause abnormal changes to brain cells and usually have a clinical pattern that can be diagnosed. These diseases cause increasing disruption to neurological function as the disease progresses. Common examples of degenerative neurological diseases are Multiple Sclerosis, Huntington's disease, Parkinson's disease, Motor Neurone disease and Alzheimer's disease.

These brain injuries are different from those cased by trauma, stroke, hypoxia etc in that the brain injury will get progressively worse as the disease progresses.

People with these diseases require treatment for the disease rather than rehabilitation post injury as in TBI and stroke.
Treatment for these diseases depends on the specific condition. While some such as multiple sclerosis and Parkinson's disease often respond well to various medications, others such as senile dementia are generally irreversible.

 

Alzheimer's disease

Alzheimer's disease is a progressive condition that affects the brain, resulting in impaired memory, thinking and behaviour. It is the most common form of dementia, which is the term used to describe a large group of illnesses that cause gradual decline in a person's functioning.

As brain cells shrink or disappear, abnormal material builds up as "tangles" in the centre of the brain cells. Dense spots or "plaques" also build up outside the brain cells. These changes affect the vital connections between cells, disrupting messages within the brain. As areas of the brain become affected in this way, the functions or abilities controlled by that area, such as information recall, become limited or are lost.

Incidence

Alzheimer's disease is most common in people over the age of 65, although it can affect people of any age. It is the most common form of dementia and accounts for between 50% and 70% of all cases.

Treatment

Currently, there is no cure for Alzheimer's disease. However some medications and alternative treatments have been found to relieve some of the symptoms, for some people, for a period of time. Up to date information is available from Alzheimer's Australia Vic.

What is the outlook?

The rate at which Alzheimer's disease progresses is very different for each person but the condition gradually becomes worse over time. A person may live from three to 20 years, but the disease does lead eventually to complete dependence and finally to death, usually from complications associated with the condition.

More information

Brain Link

BrainLink is a community based care and education organisation focusing on the impact of acquired brain disorders.

Brain Link Fact Sheet: Understanding Alzheimer's diseasePDF The above information is a short summary of parts of this fact sheet.

Alzheimer's Australia NSW

It is a peak body for people with dementia and their families and carers.

 

Multiple sclerosis (MS)

Multiple sclerosis (MS) is a condition that affects the central nervous system (the brain and the spinal cord) in a variety of ways. MS may affect a person's mobility, their ability to coordinate muscles or their eyesight. It may impair functions such as bladder control, speech, concentration or memory. MS affects each person differently.

Scientists don't know what causes MS but they do know that it provokes an "auto-immune reaction". This means that the body fails to recognise its own tissue (in this case, myelin tissue) as part of itself and the immune system swings into action to destroy it, as it would any "invader" such as a virus or bacteria.

Most healthy nerve fibres are wrapped in myelin tissue, a fatty substance that insulates the nerves and assists the communication flow between the brain and the body. In MS, the myelin sheath becomes inflamed. Sometimes the inflammation dies down, but if it continues, the myelin is damaged and a scar forms. Scientists have called these scars "plaques" or "sclerosis" (from the Greek word for scar). This process is called demyelination.

The scars can distort or completely block the nerve impulses (the messages to and from the brain and body). The inflammation occurs randomly over time and throughout the central nervous system, causing scarring in many places.

Who Develops MS?

More than 16,000 Australians have MS. Symptoms usually appear for the first time between the ages of 20 and 50. Women are affected more often than men at a ratio of about 3:1.

MS is also more common in temperate zones. For example in Queensland, MS affects roughly 12 people per 100,000 compared to 76 people per 100,000 in Tasmania.

Treatment

There is currently no cure for MS. Treatment involves a combination of medication and therapies.

What is the Outlook?

MS is rarely fatal. Most people live a normal life-span. While some people become entirely dependent, many remain relatively independent and mobile over many years. There is a terrific amount happening in the world of international research, in which Australia is heavily involved.

More information

Brain Link

BrainLink is a community based care and education organisation focusing on the impact of acquired brain disorders.

Brain Link Fact Sheet: Understanding Multiple Sclerosis PDF The above information is a short summary of parts of this fact sheet.

MS Australia

Multiple Sclerosis Australia is committed to:

  • Enhance the quality of life of people with MS and reduce the impact of MS on the families and carers of those with Multiple Sclerosis.
  • Maintain and increase its role as a leading not-for-profit organisation and a preferred charity in Australia for community and corporate support.

 

Huntington Disease (HD)

Huntington Disease (HD) is a genetic neurodegenerative condition, which means it affects the brain and is inherited through a family line. HD destroys the brain cells that affect our emotions, intellect and movement. People with HD experience uncontrollable jerking movements (chorea) of the limbs, torso and face, loss of mental abilities and may experience behavioural and personality changes.

HD is caused by a faulty gene that is passed on by one parent. Researchers do not yet understand how, but the faulty gene leads to damage of nerve cells (neurones) in the brain. Two areas of the brain in particular are affected: the basal ganglia, which controls and coordinates body movements and the cerebral cortex, which is responsible for processes such as thought, emotions, perception and planning.

Incidence

About 5 – 10 people per 100,000 will develop Huntington Disease. A person who has the faulty HD gene has a 50% chance of passing it on to each of their children.

If the child inherits the gene, HD symptoms will develop.

Treatment

There is currently no cure for HD but there are many ways to improve a person's quality of life and cope with the symptoms.

What is the Outlook?

HD progresses slowly and a person may live for 15 to 25 years after developing their first symptoms. Generally, the older the age of onset, the more slowly the condition progresses.


More information

Brain Link

BrainLink is a community based care and education organisation focusing on the impact of acquired brain disorders.

Brain Link Fact Sheet: Understanding Huntington Disease PDF The above information is a short summary of parts of this fact sheet.

Huntington's New South Wales

Huntington's New South Wales works towards satisfying the needs of people with or at risk for Huntington's Disease and their families in NSW and the ACT by providing and/or facilitating delivery of a range of quality services.

 

Parkinson's disease

Parkinson's is a neurological condition that affects the control of body movements. People with Parkinson's experience trembling, rigidity, slowness of movement and changes in posture. The condition is neither fatal nor contagious, but it is degenerative, which means the symptoms become worse over time.

At present, we do not know the cause of Parkinson's. We do know that there is a loss of nerve cells (neurones) in the substantia nigra (a pigmented area in the base of the brain), which is part of the basal ganglia (the area that controls and coordinates body movements).

Neurones produce a chemical called dopamine that enables nerve impulses (messengers to and from the brain) to move between one nerve cell and the next. With fewer neurones in the substantia nigra, there is less dopamine, which interferes with the transmission of messages within the basal ganglia. As a result, some parts of the brain which control body movement become overactive, while other parts become underactive. The first signs of Parkinson's are usually noticed when approximately 60% to 70% of these dopamine-producing neurones are lost. Why these cells die remains a mystery to researchers.

Incidence

Parkinson's is the second most common degenerative neurological condition after dementia. More than 80,000 people in Australia are living with it and about 4000 new cases are diagnosed each year. Parkinson's affects men and women and is more prevalent in people aged between 50 and 75 years. Approximately 10% of those diagnosed are 40 years and younger.

Treatments

The main treatments are individual management through medication or surgery.

Managing Parkinson's

While there is no cure for Parkinson's, there are plenty of ways to reduce some symptoms and the condition can be managed well with a combination of therapies, regular activity and a healthy diet.


More information

Brain Link

BrainLink is a community based care and education organisation focusing on the impact of acquired brain disorders.

Brain Link Fact Sheet: Understanding Parkinson's PDF The above information is a short summary of parts of this fact sheet.

Parkinson's NSW

Parkinson's NSW Incorporated is a non-profit, community-based organisation established in 1979 to provide information, counselling and support to people living with Parkinson's disease.

 

Motor neurone disease (MND)

Motor neurone disease (MND) is the name given to a group of diseases in which the muscles that enable us to move, speak, breathe and swallow fail to work normally.

The cause of MND is unknown but researchers' theories about its origins include discussion of viruses, environmental toxins and chemicals as well as immune factors. They also discuss transmitter chemicals that control communication between nerve cells (neurones), and the way in which the neurones that control movement grow, repair and age.

Motor system: Muscles, and the nerves that supply them, are called the motor system. It looks after action and movement. The system fails when the neurones that control movement (motor neurones) become damaged and stop working. Without nerve impulses (messengers travelling to and from the brain and the body) to activate them, the muscles gradually waste away.
There are two different types of motor neurones. MND can affect both.

Upper motor neurones: Upper motor neurones are in the brain. Damage to these causes "spastic" or stiff paralysis of the muscles that they serve.

Lower motor neurones: Lower motor neurones are in the spinal cord. Damage to them causes floppy paralysis.

Incidence

Each year, about one in 50,000 Australians develops MND. MND can affect anyone but is slightly more common in men than in women and among people in their 50s and 60s. MND is not contagious or infectious.

Treatment

At present there is no cure for MND, although research continues throughout the world and there has been encouraging progress. In Australia a medication has been approved for the treatment of amyotrophic lateral sclerosis (ALS) – the most common form of MND. This drug is Rilutek (riluzole), which is available at a subsidised price on the Pharmaceutical Benefits Scheme. There are some strict criteria governing which people can receive subsidised Rilutek, and it is best to discuss these with a neurologist.

Researchers are developing and trialling drugs to slow down the progression of the condition. Your neurologist or the Motor Neurone Disease Association can give you the latest information.

How MND Progresses

In most cases, MND does not affect intellect, memory or the senses (sight, hearing, taste, smell, touch), although it occasionally causes dementia. Bowel and bladder are not usually affected, but constipation can be a problem when people become less mobile or have to change their diet because of swallowing difficulties.

Most people will eventually have difficulty moving about and many will have difficulty with speech and swallowing.


More information

Brain Link

BrainLink is a community based care and education organisation focusing on the impact of acquired brain disorders.

Brain Link Fact Sheet: Understanding Motor Neurone Disease PDF The above information is a short summary of parts of this fact sheet.

MND NSW

MND NSW is a registered charitable not-for-profit organisation providing support and information for people with all types of motor neurone disease, their families and carers in NSW, ACT and NT.