Multiple system atrophy

Multiple System Atrophy (MSA) is a rare and progressive neurodegenerative disorder.

It impacts on multiple systems that control movement and autonomic function (the nervous system that controls involuntary action such as blood pressure and bladder control).

Although there’s currently no cure for MSA, a range of drugs, treatments and therapies are available to manage the symptoms.

The management of MSA is focused on optimizing quality of life, relieving symptoms and assisting patients with their activities of daily living (ADL).

MSA symptoms usually develop in the person’s 50’s to 60’s.

MSA affects differing parts of the brain, including the basal ganglia and cerebellum.

The changes in the basal ganglia, which is also affected in Parkinson’s disease (PD), cause the Parkinsonian movement changes. This is the most common form of MSA.

The changes in the cerebellum cause the incoordination of walking and limb movements (cerebellar ataxia). This form occurs in about 20% people with MSA.

MSA progresses faster than PD and does not respond as well to medications. The rate of progression varies greatly between people.

Diagnosis of multiple system atrophy

The diagnosis of MSA can be difficult to make in the early stages.  There are currently no diagnostic tests available to confirm MSA.

A neurologist specialising in movement disorders is the recommended medical person to see when seeking a diagnosis.

 The early symptoms of MSA often include problems with the autonomic system i.e. urinary urgency, postural hypotension (drop in blood pressure) and erectile dysfunction. As the disorder progresses Parkinsonian symptoms, such as slow movements and rigidity/stiffness, become more dominant which then makes the diagnosis of MSA more likely.

It may take several assessments and observations by the neurologist before they are confident of the diagnosis.

Tests such as sphincter electromyogram, cardiovascular autonomic function test, clonidine testing, and brain scans using MRI or SPECT, may help confirm the diagnosis of MSA.

Causes of multiple system atrophy

The deterioration in brain cells in several areas of the brain leads to the symptoms found in MSA.

The key change found in the brain affected by MSA is the abnormal accumulation of the protein alpha-synuclein, which also occurs in PD. The alpha-synuclein accumulates in the brain stem and basal ganglia. It is not known what triggers this accumulation.

Changes occur in the brain stem, the basal ganglia and the cerebellum which controls coordination of movements.

 To date genetics, environmental and infection factors have not been found to be linked MSA.

Symptoms of multiple system atrophy

The symptoms can vary between people because differing parts of the brain can be affected.  Not everyone will develop all of the symptoms.

The main symptoms include:

  • low blood pressure
  • incontinence of the bladder
  • early impotence in men
  • ataxia or incoordination of movement
  • Parkinsonian changes to movement

The most common movement symptoms include:

  • slowness
  • stiffness or rigidity
  • difficulty starting movement or akinesia
  • poor balance and unsteadiness when walking
  • tremor of the hand and fingers in some people
  • dystonia of the trunk, neck or face (sustained abnormal muscle activity and postures)
  • extreme postures, such as the head flexed forward with the chin on the chest due to dystonia
  • poor coordination of walking and limb movements called ataxia

Repeated falls are unusual at the beginning of MSA. Falls may happen when blood pressure is very low or due to unsteadiness or ataxia when walking.

Speech and swallowing are also affected. Speech can be soft, slow and slurred.

Eye movements may also be affected.

Regardless of the type of changes to movement, changes to the autonomic functions affects everyone with MSA. These changes include:

  • marked drop in blood pressure when standing, which may cause fainting
  • problems with heart rate
  • erectile dysfunction
  • loss of bladder control

MSA can cause a range of breathing problems, such as sleep apnoea and dyspnoea and inspiratory stridor. Breathing problems can develop in the early stages of MSA.

Sleep disorders, in particular rapid eye movement sleep behaviour disorder (RBD), may be one of the first symptoms presenting in MSA. RBD affects most people with MSA.

Mood changes are common and may include depression, anxiety and mood swings.

Changes to thought processes, known as cognitive changes, also occur with MSA.

Treatment of multiple system atrophy

Currently there is no treatment available to stop or slow the progression of MSA.

There are, however, a variety of medical treatments and therapies available to manage the symptoms.

A multidisciplinary team that includes a neurologist and therapists who specialise in movement disorders is recommended for the management of the complex MSA symptoms. The appropriate multidisciplinary team members should be seen from the time the diagnosis of MSA is received.

Medical treatment

A neurologist specialised in movement disorders can advise on medical treatments to treat the presenting symptoms.

The symptoms of slow movement and rigidity that occur in MSA may improve in the early stages with the use of PD medications, especially levodopa preparations such as sinemet or madopar.

Botulinum toxin A may be useful in treating troublesome dystonia.

To date there are no effective medications for problems with walking and limb coordination, called cerebellar ataxia.

Treatment for bladder incontinence include:

  • Medications such as oxybutynin
  • Intermittent self-catheterisation

Specialised health professionals who work in a continence clinic, such as doctors, nurses and physiotherapists, should also be considered in the management of continence problems.

Medications and several practical solutions can be used to improve low blood pressure, which includes:

  • Medications such as fludrocortisone, midodrine and droxidopa
  • Drinking adequate fluids
  • High salt diet
  • Eating small and frequent meals over the day
  • Pressure stockings
  • Raising the head of the bed when sleeping

There are various medications to help with male impotence. The treatment needs to be tailored to the person’s particular problems and needs.

Constipation can be managed with high fluid and fibre intake and over the counter products. A continence nurse and dietitian can advise on ways to improve constipation.

Breathing disorders, such as sleep apnoea, may require a face mask to be worn at night through which a continuous positive air pressure is applied to keep the upper airways open.

If the sleep disorder rapid eye movement sleep behaviour disorder (RBD) is severe enough, medications such as melatonin may relieve the symptoms.

Depression can be managed effectively with a range of antidepressants.

There are currently no medications that can reverse or slow down the cognitive changes that occur with MSA. Assessment and strategies to assist are available from occupational therapists and neuropsychologists.

Education of symptoms and managing medications

A nurse with specialist training in MSA can provide education and support to the person with MSA and their caregiver. This may include:

  • monitoring the effects of changes to medications as recommended by the neurologist and liaising with the neurologist
  • providing education and support around the recommended medications and the assist with the minimisation of side effects
  • providing individualised education for the person with MSA and their caregivers to ensure optimal understanding and management of the various MSA symptoms as the condition progresses
  • arranging referrals to other relevant health professionals within the specialist team

Treatments for walking difficulties, balance, falls and posture problems

A physiotherapist specialised in movement disorders such as MSA can assess walking and balance problems and recommend ways to improve mobility and safety.

An occupational therapist specialised in movement disorders such as MSA can assess the person at home and suggest ways to improve safety, both inside and outside the home.

As MSA progresses the physiotherapist and occupational therapist can advise on appropriate seating to help achieve a good posture that is comfortable and makes eating and drinking easier.

A physiotherapist can advise on ways to help the person walk safely, reduce risk of falls and remain independent as long as possible.  This may include the use of equipment, walking frames, protective garments such as hip protectors to reduce risk of hip fractures and the use of a wheelchair.

A physiotherapist can also develop an exercise program to improve or maintain flexibility, strength and fitness.

An occupational therapist may come to the home to advise on ways to improve mobility and safety in the home and the community. An occupational therapist can advise on safety aids to suit the needs of the person, such as rails next to steps, equipment for the shower and toilet. If home modifications are required, they can help with the planning of them.

If posture is affected and is interfering with communication, eating, drinking and comfort, the physiotherapist can prescribe exercises to stretch the tight muscles and joints and the occupational therapist can advise on equipment, such as seating.

The physiotherapist and occupational therapist can also assess the possible causes of any pain, such as poor posture, pain secondary to falls and pressure areas due to staying in one position too long.

An occupational therapist can assess and advise on pressure relieving equipment, such as specialised seating cushions and mattresses.

There are various funding bodies that can fully or partially cover the cost of some home modifications and adaptive equipment such the State Wide Equipment Program (SWEP). An occupational therapist can advise you on how to apply for funds to assist with the costs if required.

The person’s neurologist may suggest injections of botulinum toxin into the cramping or dystonic muscles that cause pain and poor posture.

Treatments for speech and swallowing difficulties

A speech pathologist specialised in movement disorders such as MSA can assess speech and swallowing difficulties and teach strategies to improve them.

Changes to voice will vary between people and as the MSA progresses.

Swallowing difficulties are common in advanced MSA and may lead to embarrassment when eating with others socially, loss of enjoyment of food, weight loss, poor nutrition and food or drinks entering the lungs (aspiration).

In the later stage of MSA, stridor when breathing in can develop. Stridor also impacts on the safety of swallowing.

A speech pathologist can assess the person’s swallowing and suggest strategies to improve it.

It is important to see a speech pathologist if any of the following swallowing problems develop as they increase the risk of aspirating fluids or solids into the lungs which may then lead to pneumonia:

  • if you need to clear your throat when eating solid foods or feeling food gets stuck
  • difficulty swallowing tablets which may feel ‘stuck’ in the back of the throat
  • coughing especially after drinking thin fluids such a tea and coffee
  • choking on solid foods such as meat, toast
  • a wet or gurgly voice after having a drink

If there is a need to go on to a specialised thickened diet to avoid aspiration, the speech pathologist and dietitian will work together to advise on the food groups to select and the best way to prepare them.

MSA may lead to changes in the person’s saliva

  • the automatic swallowing of saliva can be disrupted in MSA leading to drooling
  • saliva or mucous may be very thick which can be very difficult to swallow
  • the mouth may be dry due to medications

These changes in saliva may cause social embarrassment as well as oral hygiene problems.

The speech pathologist can recommend ways to improve these problems with saliva and advise on ways to maintain good oral hygiene.

Management of nutrition

Adequate intake of nutritional food is essential with MSA.

Due to swallowing difficulties, the person with MSA may not be eating well and therefore not getting enough nutrients.

A dietitian specialised in movement disorders such as MSA can assess the person’s diet and provide them with suggested menus to improve any dietary problems they may have.

A dietitian specialised in movement disorders such as MSA can assess the person’s diet and provide them with suggested menus to improve any dietary problems they may have. The dietitian can ensure the person with MSA is fully informed on what foods they need to eat in order to maintain a nutritional diet, how diet can improve constipation and how to avoid weight loss.

They will also advise on how to plan small and frequent meals over the day so as to avoid worsening the person’s low blood pressure and to help with fatigue.

The dietitian can advise on the amount of daily fluids needed to help control low blood pressure.

The dietitian will work closely with the speech pathologist to ensure the person with MSA and their caregiver and family know how to lessen the risk of aspiration when eating and drinking.

Managing the social and emotional impact

The effects of MSA are broad and impact on the social and economic life of the person and their family from the time of diagnosis in a variety of ways.

Coping and adjusting to the diagnosis, facing increasing challenges at work and planning for the future can be a daunting undertaking.

Navigating this newfound uncertainty requires careful consideration and appropriate specialised support.

As the disorder progresses, caregiver burden also increases and quality of life can decrease.

A social worker specialised in movement disorders such as MSA can assist the person with MSA, their caregiver and family negotiate the challenges faced across the life time of the person’s condition.

Social workers are highly skilled professionals who can assist you and your loved ones by:

  • providing a range of different therapy’s like counselling
  • assistance with linking to other support services
  • directing you to financial support services
  • information provision of entitlements for care packages
  • help to facilitate suitable respite arrangements for the caregiver
  • assistance and support with navigating the pathway to permanent residential care if the need arises.

After receiving a diagnosis of MSA it is normal to experience a change in mood. A social worker has the appropriate specialised skills to help you deal with the reaction to your diagnosis.

Depression and anxiety are common clinical symptoms of MSA. Depression and anxiety can benefit from a variety of interventions and approaches. It is important to talk openly with your doctor or neurologist if experiencing any of these to determine the best treatment plan for you.

Social workers are specifically trained to support you in understanding how best to manage such symptoms.

Cognition and behaviour

MSA causes changes to the person’s cognition. Cognition refers to mental processes and includes the ability to learn, to reason and remember.

Changes in cognition may make the person feel less confident and anxious.

It is important that the person with MSA, their caregiver and family speak to the neurologist about any changes in cognition.

A neuropsychologist specialised in movement disorders such as MSA can assess a person’s cognition and provide strategies to optimise independence.

The common cognitive changes include

  • difficulty with decision making and planning
  • disturbance to visuospatial functions

MSA may also cause the loss of control of emotional outbursts, such as crying inappropriately without sadness or laughing inappropriately without mirth.

There are specialists, such as a neuropsychologist, who can assess the severity of these changes. The information gained from the assessment is important in ensuring the person receives the best care and advice to help them manage these changes.

Though there is no evidence to support the benefits of any medication to improve cognition or prevent its deterioration, a neuropsychologist and occupational therapist can advise on ways to manage the challenges caused by changes to the person’s cognition and behaviour.

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