Multiple system atrophy
Multiple system atrophy is a disease of the nervous system that leads to premature death. It results in parts of the brain and spinal cord gradually becoming more damaged over time.
It also causes a gradual loss of brain cells from the autonomic nervous system – the nervous system in charge of automatic functions we don't have to think about, like breathing and bladder control.
This results in a wide range of symptoms, including the muscle control problems seen in Parkinson's disease.
What are the symptoms?
Symptoms usually start between 50 and 60 years of age, but can start any time after 30.
Many different functions of the body can be affected, including the urinary system, blood pressure control and muscle movement.
The range of symptoms is described below, although it is important to note that a person with multiple system atrophy won't necessarily develop all of these.
Men and women with multiple system atrophy will usually have one or more of the following bladder symptoms:
constantly feeling the need to urinate
urinating more frequently
passing urine unintentionally –
not being able to empty the bladder properly
not being able to urinate
Men with multiple system atrophy will usually experience erectile dysfunction (the inability to get and maintain an erection), although this is a common problem that many men without the disease develop.
Low blood pressure when standing up
It's common for someone with multiple system atrophy to feel lightheaded, dizzy and faint when they suddenly sit or stand up. This is because their blood pressure drops when they become upright, which is known as postural hypotension.
When you stand up after lying down, your blood vessels usually narrow quickly and your heart rate increases slightly, to prevent blood pressure dropping and maintain blood flow to the brain. This function is carried out automatically by the autonomic nervous system.
However, because this doesn't work properly in people with multiple system atrophy, this control is lost.
Problems with coordination, balance and speech
A part of the brain called the cerebellum becomes damaged in multiple system atrophy, which can make the person clumsy and unsteady when walking, and can also cause slurred speech.
These problems are collectively known as cerebellar ataxia.
Feeling stiff, and slowness of movement
A person with multiple system atrophy typically has much slower movements than normal. This is referred to as "bradykinesia" and can make everyday tasks difficult. Movement is hard to initiate, and they often have a distinctive slow, shuffling walk with very small steps.
Some people may also have stiffness and tension in the muscles, which can make it even more difficult to move around and can result in painful muscle cramps (dystonia).
The above symptoms are typical of Parkinson's disease, but unfortunately the medication used to relieve these symptoms in people with Parkinson's disease (levodopa therapy) is not very effective for people with multiple system atrophy.
Other signs and symptoms
People with multiple system atrophy may also have:
shoulder and neck pain
cold hands and feet
problems controlling sweating
muscle weakness in the body and limbs – it may be more pronounced in one arm or leg
uncontrollable laughing or crying
sleep problems – insomnia, snoring, restless legs, nightmares
noisy breathing and unintentional sighing
a weak, quiet voice
dementia (although this is uncommon)
What's the cause?
The cause of multiple system atrophy is not well understood.
It doesn’t appear to be inherited – there's no evidence that an affected person's children will develop it.
However, it's possible that both genetic and environmental factors contribute to the disease, so scientists are currently researching whether there is a genetic tendency (predisposition) to develop it.
The brain cells of a person with multiple system atrophy contain misfolded alpha-synuclein protein (of which there is lots of in the brain). It's thought that a build-up of abnormal alpha-synuclein is responsible for the loss of brain cells.
How common is multiple system atrophy?
Multiple system atrophy is estimated to affect around 5 in every 100,000 people worldwide.
There are almost 3,000 people in the UK living with the disease.
How is it diagnosed?
There is no specific test to diagnose multiple system atrophy.
A diagnosis can usually be made based on the symptoms, although it can potentially be confused with Parkinson's disease.
The box on this page explains how multiple system atrophy is different to Parkinson's disease.
If a doctor (usually a neurologist) suspects multiple system atrophy from a patient's symptoms, they will test the patient's reflexes and "automatic" body functions, such as bladder function. These body functions and responses may be altered in someone with multiple system atrophy.
A brain scan is often needed – usually an MRI scan or a SPECT scan to detect any loss of brain cells.
More detailed assessments of autonomic function may also be performed – for example, recording blood pressure changes when lying and standing.
How is it treated?
Unfortunately, there is no cure for multiple system atrophy and no way of slowing the disease's progression.
People with multiple system atrophy typically live for six to nine years after symptoms start, and may deteriorate quickly during this time. However, some people may live for more than 10 years after a diagnosis.
Help and support is available, and symptoms can be managed so the patient is as independent and comfortable as possible.
treatment of low blood pressure
treatment of constipation
treatment of urinary incontinence
treatment of swallowing problems
Support from physiotherapists and occupational therapists can help people with multiple system atrophy to safely keep active, in order to maintain fitness and muscle strength.
Support for carers
If you're a carer of someone with multiple system atrophy, it's important to remember that you're not alone and that there is support available. By law, you're entitled to a free health and social care assessment, which you can access through your local authority. The assessment will look at the possibility of you getting practical and financial help.
Being a carer means that you may be entitled to certain financial benefits, especially if you have to give up work.
Carers can also get help with breaks from caring from local authorities or organisations such as Carers Trust.
Support for patients
If you have been diagnosed with multiple system atrophy, your treatment and medical care will probably be the first thing on your mind. However, there are other aspects of your life to consider, and it's important to know exactly what kind of assistance is available and where you can get it.
If you are finding it hard to cope with day-to-day life, talk about your needs with your doctor or nurse, who will refer you to a social worker. Your social worker will assess exactly the kind of help you need:
Care attendants can help with housework, dressing and washing, or even just keep you company and give your carer a break. Look into this as soon as you can, as many care attendants have waiting lists.
Carers Trust is an organisation that helps carers in the UK by providing access to breaks, information and advice, education, training and employment opportunities. Visit the Carers Trust website at http://www.carers.org/ (links to external site), or phone 0844 800 4361.
Meals on wheels. Contact your local council about its meals on wheels service. It may be able to offer financial assistance to help pay for this. Go to GOV.UK for details of your eligibility.
Benefits – You may be eligible for Income Support, Disability Living Allowance (DLA) or Attendance Allowance. Get in touch with the Benefit Enquiry Line for more details on 0800 882200 (textphone 0800 243355) or online at www.dwp.gov.uk (links to external site).
Home adaptations – Occupational therapists provide a detailed assessment of your needs at home, making life easier by arranging equipment and making adaptations to your home. The aim is to create a comfortable and practical place to live during your treatment. This could mean anything from adding handrails around the house to installing a downstairs shower.
More information and support
Multiple System Atrophy Trust – The Multiple System Atrophy Trust is the UK's main charity supporting people with the disease, as well as their families, carers and the health professionals treating them. It provides specialist nurses, an email and telephone support service, and runs a UK-wide support group network. It also funds vital research that aims to find the cause and a cure for multiple system atrophy.
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