Multiple system atrophy – what, where and why?

 

Multiple system atrophy

Multiple system atrophy

Multiple system atrophy is a rare nervous system disorder where nerve cells in several parts of the brain deteriorate over time.

From the NHS Choices  website

This causes problems with balance, movement and the autonomic nervous system, which controls a number of the body’s automatic functions, such as breathing and bladder control.

Symptoms of multiple system atrophy

Symptoms of multiple system atrophy usually start when someone is between 50 and 60 years of age, but they can come at any time after 30.

The symptoms are wide-ranging and include muscle control problems, similar to those of Parkinson’s disease.

Many different functions of the body can be affected, including the urinary system, blood pressure control and muscle movement.

Although there are many different possible symptoms of multiple system atrophy, not everyone who’s affected will have all of them.

Bladder problems

Men and women with multiple system atrophy will usually have one or more of the following bladder symptoms:

constantly feeling the need to pee

peeing more frequently

loss of bladder control

being unable to empty the bladder properly

being unable to pee

Erection problems

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 condition develop.

Low blood pressure when standing up

Someone with multiple system atrophy will often feel lightheaded, dizzy and faint after standing up. This is known as postural hypotension and is caused by a drop in blood pressure when they stand upright.

When you stand up after lying down, your blood vessels usually narrow quickly and your heart rate increases slightly to prevent your blood pressure from dropping and decreasing blood flow to your brain.

This function is carried out automatically by the autonomic nervous system; however, this system doesn’t work properly in people with multiple system atrophy, so the control is lost.

Problems with co-ordination, balance and speech

In multiple system atrophy, a part of the brain called the cerebellum is damaged. This can make the person clumsy and unsteady when walking, and can also cause slurred speech.

These problems are collectively known as cerebellar ataxia.

Slowness of movement and feeling stiff

A person with multiple system atrophy has much slower movements than normal (bradykinesia). This can make it difficult to carry out everyday tasks. Movement is hard to initiate, and the person will often have a distinctive slow, shuffling walk with very small steps.

Some people may also have stiff, tense muscles. This can make it even more difficult to move around and cause painful muscle cramps (dystonia).

The above symptoms are typical of Parkinson’s disease but, unfortunately, the medication used to relieve them in people with Parkinson’s disease (levodopa) isn’t very effective for people with multiple system atrophy.

Other signs and symptoms

People with multiple system atrophy may also have:

shoulder pain and neck pain

constipation

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 – insomniasnoringrestless legs or nightmares

noisy breathing and unintentional sighing

a weak, quiet voice

swallowing problems

blurred vision

depression

dementia (although this is uncommon)

Causes of multiple system atrophy

The causes of multiple system atrophy aren’t 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 may contribute, so research is currently looking at whether there’s a genetic tendency (predisposition) to develop it.

The brain cells of a person with multiple system atrophy contain a protein called alpha-synuclein. A build-up of abnormal alpha-synuclein is thought to be responsible for damaging areas of the brain that control balance, movement and the body’s autonomic functions.

Diagnosing multiple system atrophy

There’s 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.

Multiple system atrophy or Parkinson’s disease?

A person is more likely to have multiple system atrophy rather than Parkinson’s disease if:

their symptoms have progressed rapidly – a person with Parkinson’s disease deteriorates more slowly

they’ve experienced falls in the early stages of the condition – this isn’t a typical symptom of Parkinson’s

they don’t respond well to levodopa therapy – levodopa can significantly improve symptoms of Parkinson’s disease

their speech is severely affected – this isn’t a typical symptom of Parkinson’s disease

they gasp and breathe noisily – this isn’t a typical symptom of Parkinson’s disease

Further tests

If multiple system atrophy is suspected, a doctor (usually a neurologist) will test the person’s reflexes and “automatic” bodily functions, such as their bladder function.

A brain scan is often needed – usually an MRI scan or a SPECT scan – to detect any loss of brain cells. Read more about SPECT scans (PDF, 304kb).

More detailed assessments of autonomic function may also be carried out – for example, recording blood pressure changes when lying down and standing.

Treating multiple system atrophy

There’s currently no cure for multiple system atrophy and no way of slowing its progression.

People with the condition typically live for six to nine years after their symptoms start and may deteriorate quickly during this time. Some people may live for more than 10 years after being diagnosed.

Help and support is available, and the symptoms can be managed so that the person is as independent and comfortable as possible.

Read about the:

treatment of low blood pressure

treatment of constipation

treatment of urinary incontinence

treatment of swallowing problems

Physiotherapy and occupational therapy can help people with multiple system atrophy stay mobile and maintain fitness and muscle strength.

Help and support

Help for carers

Practical and financial help is available if you care for someone with multiple system atrophy.

Your local authority can carry out a carers’ assessment to assess your needs and determine the help and support you’re entitled to.

Find out more about carers’ assessments.

Support for people with multiple system atrophy

If you have multiple system atrophy and are finding coping with day-to-day life difficult, your doctor or nurse can refer you to a social worker.

They can carry out an assessment and recommend the help you require. For example, you may need:

care attendants – who can help with everyday tasks such as housework, dressing and washing

meals on wheels – your local council may be able to offer financial help for this; check your eligibility for getting meals at home

benefits – you may be eligible for a number of benefits, such as Attendance Allowance and Personal Independence Payment (PIP)

home adaptations – to make moving around at home easier and ensure your home environment is as comfortable as possible

Find out more about care and support needs assessments.

Further information and support

The Multiple System Atrophy Trust provides help and support to people with multiple system atrophy, as well as their families, carers and the healthcare professionals treating them.

The charity also funds research to discover the cause of multiple system atrophy and to try to find a cure.

The helpline number is 0333 323 4591 (Monday to Friday, 9am to 5pm). You can also email support@msatrust.org.uk.