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Multiple System Atrophy / MSA

What is multiple system atrophy?

Multiple system atrophy (MSA) is caused by the progressive degeneration of nerves in the brain and spinal cord. MSA attacks the autonomic nervous system, which controls involuntary action such as breathing, blood pressure, and digestion.

MSA affects nearly 15,000 to 50,000 Americans and generally manifests in midlife, mimicking Parkinson’s disease. In rare cases, MSA also affects children.

The cause of MSA is unknown, but it seems related to the build-up of a protein (alpha-synuclein) in glial cells that protect neurons in the central nervous system.

What are the symptoms of multiple system atrophy?

Initial symptoms include

  • clumsiness and incoordination
  • fainting or lightheadedness due to orthostatic hypotension (blood pressure drops when rising from a seated or lying position)
  • impaired speech
  • loss of bladder control
  • slowness of movement, tremors, or rigidity (stiff muscles)

Additional symptoms may be:

  • antecollis, when the neck bends forward and the head droops
  • anxiety or depression
  • contractures, the chronic shortening of muscles or tendons around the joints, affecting hands or limbs
  • involuntary or uncontrollable sighing or gasping
  • Pisa syndrome, when the body appears to lean to one side
  • sleep disorders, including a tendency to act out dreams

How is multiple system atrophy diagnosed?

We take a clinical history and perform a brief neurological examination. The following tests may also be ordered:

  • Assessment of bladder function
  • Autonomic testing evaluates control of blood pressure and heart rate.
  • DaTscan is an imaging test to assess the dopamine transporter in a part of the brain called the striatum.
  • Magnetic resonance imaging (MRI) scans identify changes related to MSA or rule out other causes. MRIs use a combination of large magnets, radio frequencies, and a computer to produce detailed images of organs and anatomical structures.
  • Positron emission tomography (PET) scan shows whether metabolic function is reduced in specific areas of the brain. We put a small amount of radioactive dye into the body to illuminate tissues for the scanner.

How is multiple system atrophy treated?

While there is no cure for MSA or way to delay its progression, treatments can help children to cope with their symptoms.

  • Assistive devices such as walkers and wheelchairs may be designed to custom-fit children.
  • Bladder control issues may be treated with anticholinergic drugs, such as oxybutynin or tolteridine, reducing the sudden urge to urinate.
  • Difficulty swallowing affects some children with MSA and they may need a feeding tube or nutritional support. Speech therapists also provide strategies to address swallowing problems.
  • Dihydroxyphenylserine is a medication that helps to replenish neurotransmitters damaged by MSA.
  • Fainting and lightheadedness (caused by orthostatic hypotension) may be relieved by wearing compression stockings, adding extra salt and/or water to the diet, and avoiding heavy meals. Hypotension can be alleviated by sleeping with the head raised. Medications may also be prescribed for this condition.
  • Levodopa is a medication used to improve motor function, though it may become less effective over time.
  • Occupational therapists advise parents about home safety and new ways to address routine activities like dressing and eating.
  • Physical therapy may be prescribed to maintain mobility, reduce contractures (chronic shortening of muscles or tendons around joints), decrease muscle spasms, and address abnormal posture.
  • Rigid postures (dystonia) may be helped by injections of botulinum toxin.
  • Sleep problems are treated with medicines such as clonazepam, melatonin, or some antidepressants.