Multiple-System Atrophy (MSA)

Multiple-system atrophy (MSA) is a progressive neurological condition in which specific areas of the brain undergo neural degeneration. The three brain areas primarily affected by MSA include the brain stem, basal ganglia, and cerebellum, all of which are portions of the brain that regulate bodily functions and motor control.

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Previously referred to as Shy-Drager syndrome, MSA presents with many symptoms that are similar to those seen in Parkinson’s disease such as poor balance and movement control, as well as loss of automatic functions such as blood pressure, bladder, and bowel control.

Although MSA often presents similarly to Parkinson’s disease, it does not respond to any of the treatments for Parkinson’s disease. MSA also tends to progress more quickly than Parkinson’s disease, with patients not usually surviving for more than 7 or 8 years after diagnosis.

Causes

It is not yet clear what causes nerve cells to become damaged in MSA. Nerve cells in the affected brain areas deteriorate and shrink (atrophy).

Microscopy studies have shown that structures referred to as glial inclusion bodies are seen in cells affected by this atrophy and that these bodies overexpress a protein called alpha-synuclein. Mutation of the synuclein gene, SNCA, which codes for alpha-synuclein, is a potential risk factor for MSA. It is not yet clear whether or not environmental factors play a role.

Recent estimates suggest that the prevalence of MSA is about 5 people per 100,000, which translates to almost 3,000 individuals living with the condition in the United Kingdom at any time. Parkinson’s disease is much more common, affecting around 200 per 100,000 people in the UK.

MSA usually starts to present with symptoms between the ages of 50 and 60 years and then advances rapidly over the course of the subsequent 5 to 10 years.

Symptoms

The first symptoms of MSA are often difficult to distinguish from those of Parkinson’s disease and include:

  • Slowed movement, rigidity, and tremor
  • Impaired speech and quivering voice
  • Clumsiness and lack of coordination
  • Problems controlling the bladder, with urgency to urinate or difficulty emptying the bladder
  • Orthostatic hypotension, where blood pressure drops when standing up from a sitting or lying position
What is Multiple System Atrophy (MSA)?

Types of MSA

MSA is divided into two types, depending on what the most prominent symptoms are.

The main features of the Parkinson type of MSA include those that resemble the symptoms seen in Parkinson’s disease such as slowed movement, stiffness, tremor, poor coordination, problems with balance, and autonomic nervous system dysfunction.

The cerebellar type of MSA indicates that the cerebellum is involved, which is a brain structure that is involved in coordination. The primary symptoms of this type include ataxia, abnormal speech or quivering voice, difficulty swallowing, and abnormal eye movements.

Additional general symptoms of MSA include:

  • Contractures in the hands or limbs
  • Involuntary gasping or sighing
  • Antecollis, where the neck bends and the head droops
  • Constipation, incontinence
  • Reduced perspiration and reduced production of saliva and tears
  • Impaired temperature regulation causing cold feet and abnormal sweating
  • Agitated sleep and abnormal breathing pattern during sleep
  • Impotence, lack of libido
  • Irregular heart rhythm
  • Depression, anxiety

Diagnosis

The diagnosis of MSA can be challenging due to symptoms of the condition being shared with other disorders. After checking a patient’s medical history and performing a neurological examination, a doctor may order tests to assess autonomic function such as control of heart rate, blood pressure, and bladder function.

Treating clinicians may also arrange a magnetic resonance imaging (MRI) scan to check for brain lesions that may be triggering MSA symptoms. Patients with MSA do not experience continued improvement when taking a drug for Parkinson’s disease called levodopa, which is a finding that can support an MSA diagnosis.

Treatment

Although there are currently no drugs that can cure MSA, several different medications are available that can alleviate symptoms and help to manage the condition.

Fludrocortisone or midodrine, which are often used to correct orthostatic hypotension, can increase the risk of fainting and injury. A drug called dihydroxyphenylserine can help to replace the loss of neurotransmitters in the autonomic nervous system. A high-fiber diet and laxatives may be recommended to help constipated patients, whereas anticholinergic treatment can help improve poor bladder control.

Other treatments and approaches that may be used to manage the symptoms of MSA include:

  • Melatonin, clonazepam, or antidepressants to help with sleep problems
  • Speech therapy to address speech problems and swallowing difficulties
  • Physiotherapy to sustain mobility, reduce contractures, and decrease abnormal posture and spasms
  • Walking aids or wheelchairs to aid mobility
  • Occupational therapy to develop safe ways of carrying out day-to-day activities such as washing, dressing, and cooking.

References

Further Reading

Last Updated: May 4, 2021

Sally Robertson

Written by

Sally Robertson

Sally first developed an interest in medical communications when she took on the role of Journal Development Editor for BioMed Central (BMC), after having graduated with a degree in biomedical science from Greenwich University.

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Comments

  1. Leandro Abreu Leandro Abreu Brazil says:

    Dr. Sally Robertson, Is there any new treatment for the disease?  Here in Brazil we have few studies and suggestions?  Thanks for listening

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