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Understanding the Symptoms and Causes of Multiple System Atrophy

Medically reviewed by Evelyn O. Berman, M.D.
Written by Brooke Dulka, Ph.D.
Posted on April 6, 2022

Multiple system atrophy (MSA) is a rare neurodegenerative disorder that shares several characteristics with Parkinson’s disease. The two conditions have similar symptoms, and they are diagnosed in similar ways. However, MSA — first known as Shy-Drager syndrome — is its own disease.

MSA is characterized by symptoms that affect both the autonomic nervous system (the part of the nervous system that controls involuntary actions such as digestion) and the movement system. The estimated prevalence of MSA is 4.4 cases per 100,000, and the condition typically affects people over 40.

Multiple System Atrophy Causes and Risk Factors

The causes of MSA are not well understood. MSA is generally considered to be sporadic — it develops randomly. The environment does not appear to influence MSA development. However, genetic factors can play a role in some families.

One case study considered two Japanese siblings with different forms of MSA. Because the siblings share genes, this suggests a genetic cause. However, a genetic analysis failed to identify any mutations in the siblings’ DNA. A review of current studies on the genetics of MSA suggests there is a genetic component in the development of MSA, but it hasn’t been found yet. More research is needed to understand the genetics of MSA.

Symptoms of Multiple System Atrophy

There are many signs and symptoms of MSA. The first symptoms that appear are often hard to tell apart from early Parkinson’s disease symptoms. These include:

  • Slow movement, rigidity (stiffness), or tremor
  • Clumsiness
  • Impaired speech
  • Fainting or lightheadedness
  • Problems with bladder control

Doctors divide MSA into two types depending on which symptoms are most prominent. These two types are:

  • Parkinsonian type (MSA-P) — Symptoms similar to Parkinson’s disease (slow movement, tremor, and stiffness), along with problems with balance, coordination, and the autonomic nervous system
  • Cerebellar type (MSA-C) — Symptoms include problems with balance and coordination, swallowing, and speaking, as well as abnormal eye movements

Other symptoms of MSA include:

  • Contractures in the limbs or hands (shortened tendons or muscles around joints that prevent them from moving freely)
  • Antecollis (bending of the neck that causes the head to drop down)
  • Pisa syndrome (abnormal posture that causes a person’s body to lean to one side)
  • Involuntary gasping or sighing
  • Anxiety or depression
  • Sleep disorders

Diagnosing Multiple System Atrophy

The steps to diagnose MSA are similar to those used to diagnose Parkinson’s disease. A doctor will need to conduct a physical examination and a neurological examination. Other tests might include bladder function assessments and imaging of the brain, such as MRI imaging or positron emission tomography (PET) scans.

An MRI scan of the brain may identify changes consistent with MSA or rule out other causes. A PET scan looks at brain metabolism. In cases of suspected MSA, doctors will look for the metabolism of the neurotransmitter dopamine. Dopamine is critical for movement; the destruction of dopamine brain cells is a hallmark of MSA and Parkinson’s disease.

Unfortunately, some of these techniques — including PET imaging — do not distinguish MSA from Parkinson’s disease. This makes diagnosis difficult and can lead to misdiagnosis.

Treatment Options

There is no cure yet for MSA, or any treatments that can slow disease progression. Current treatments help reduce symptoms associated with MSA.

For instance, if a person’s symptoms include a sleep disorder, an anxiety medication such as clonazepam (Klonopin) may be prescribed. If someone is experiencing depression, antidepressants might be used to alleviate depression-associated symptoms.

Outlook for Multiple System Atrophy

Research shows that the average survival from the onset of MSA symptoms is about 10 years. Some factors — like having MSA-P specifically, early bladder problems, and laboratory evidence of generalized autonomic failure — are associated with shorter survival.

However, research into Parkinson’s disease is growing, which could lead to more MSA research as well. People living with MSA may also benefit from advances in Parkinson’s research and therapies.

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Posted on April 6, 2022
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Evelyn O. Berman, M.D. is a neurology and pediatric specialist and treats disorders of the brain in children. Review provided by VeriMed Healthcare Network. Learn more about her here.
Brooke Dulka, Ph.D. is a freelance science writer and editor. She received her doctoral training in biological psychology at the University of Tennessee. Learn more about her here.

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