What is the definition of Multiple System Atrophy?

Multiple system atrophy- parkinsonian type (MSA-P) is a rare condition that causes symptoms similar to Parkinson disease. However, people with MSA-P have more widespread damage to the part of the nervous system that controls important functions such as heart rate, blood pressure, and sweating.

The other subtype of MSA is MSA-cerebellar. It mainly affects areas deep in the brain, just above the spinal cord.

What are the alternative names for Multiple System Atrophy?

Shy-Drager syndrome; Neurologic orthostatic hypotension; Shy-McGee-Drager syndrome; Parkinson plus syndrome; MSA-P; MSA-C

What are the causes for Multiple System Atrophy?

The cause of MSA-P is unknown. The affected areas of the brain overlap with areas affected by Parkinson disease, with similar symptoms. For this reason, this subtype of MSA is called parkinsonian.

MSA-P is most often diagnosed in men older than 60.

What are the symptoms for Multiple System Atrophy?

MSA damages the nervous system. The disease tends to progress rapidly. About one half of people with MSA-P have lost most of their motor skills within 5 years of onset of the disease.

Symptoms may include:

  • Tremors
  • Movement difficulties, such as slowness, loss of balance, shuffling when walking
  • Frequent falls
  • Muscle aches and pains (myalgia), and stiffness
  • Face changes, such as a masklike appearance to the face and staring
  • Difficulty chewing or swallowing (occasionally), not able to close the mouth
  • Disrupted sleep patterns (often during rapid eye movement [REM] sleep late at night)
  • Dizziness or fainting when standing up or after standing still
  • Erection problems
  • Loss of control over bowels or bladder
  • Problems with activity that requires small movements (loss of fine motor skills), such as writing that is small and hard to read
  • Loss of sweating in any part of the body
  • Decline in mental function
  • Nausea and problems with digestion
  • Posture problems, such as unstable, stooped, or slumped over
  • Vision changes, decreased or blurred vision
  • Voice and speech changes

Other symptoms that may occur with this disease:

  • Confusion
  • Dementia
  • Depression
  • Sleep-related breathing difficulties, including sleep apnea or a blockage in the air passage that leads to a harsh vibrating sound

What are the current treatments for Multiple System Atrophy?

There is no cure for MSA-P. There is no known way to prevent the disease from getting worse. The goal of treatment is to control symptoms.

Dopaminergic medicines, such as levodopa and carbidopa, may be used to reduce early or mild tremors.

But, for many people with MSA-P, these medicines do not work well.

Medicines may be used to treat low blood pressure.

A pacemaker that is programmed to stimulate the heart to beat at a rapid rate (faster than 100 beats per minute) may increase blood pressure for some people.

Constipation can be treated with a high-fiber diet and laxatives. Medicines are available to treat erection problems.

What are the support groups for Multiple System Atrophy?

The following groups can provide more information on MSA-P:

  • National Organization for Rare Disorders -- rarediseases.org/rare-diseases/multiple-system-atrophy
  • The MSA Coalition -- www.multiplesystematrophy.org/about-msa

What is the outlook (prognosis) for Multiple System Atrophy?

Outcome for MSA is poor. Loss of mental and physical functions slowly get worse. Early death is likely. People typically live 7 to 9 years after diagnosis.

When should I contact a medical professional for Multiple System Atrophy?

Call your provider if you develop symptoms of this disorder.

Call your provider if you have been diagnosed with MSA and your symptoms return or get worse. Also call if new symptoms appear, including possible side effects of medicines, such as:

  • Changes in alertness/behavior/mood
  • Delusional behavior
  • Dizziness
  • Hallucinations
  • Involuntary movements
  • Loss of mental functioning
  • Nausea or vomiting
  • Severe confusion or disorientation

If you have a family member with MSA and their condition declines to the point that you are unable to care for the person at home, seek advice from your family member's provider.

Central

REFERENCES

Fanciulli A, Wenning GK. Multiple system atrophy. N Engl J Med. 2015;372(3):249-263. PMID: 25587949 www.ncbi.nlm.nih.gov/pubmed/25587949.

Jankovic J. Parkinson disease and other movement disorders. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology in Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016:chap 96.

Romero-Ortuno R, Wilson KJ, Hampton JL. Disorders of the autonomic nervous system. In: Fillit HM, Rockwood K, Young J, eds. Brocklehurst's Textbook of Geriatric Medicine and Gerontology. 8th ed. Philadelphia, PA: Elsevier; 2017:chap 63.

  • Condition: Neurogenic Orthostatic Hypotension (NOH)
  • Journal: American journal of hypertension
  • Treatment Used: Avoiding Triggers, Diet, Compression Garments, Fludocortisone, Desmopressin, Pyridostigmine, Atomoxetine, Midorodrine, and Droxidopa
  • Number of Patients: 0
  • Published —
This article discusses the treatment of neurogenic orthostatic hypotension (postural low blood pressure; NOH).
  • Condition: Long-term Neurological Conditions
  • Journal: JAMA network open
  • Treatment Used: Short-term Integrated Palliative Care
  • Number of Patients: 350
  • Published —
In this study, researchers evaluated the outcomes of short-term integrated palliative care for patients with long-term neurological conditions.
Clinical Trial
  • Status: Not yet recruiting
  • Phase: N/A
  • Intervention Type: Behavioral
  • Participants: 60
  • Start Date: July 1, 2021
Optimizing BCI-FIT: Brain Computer Interface - Functional Implementation Toolkit
Clinical Trial
  • Status: Not yet recruiting
  • Phase: N/A
  • Intervention Type: Other
  • Participants: 24
  • Start Date: March 22, 2021
Effect of Comprehensive Swallowing Rehabilitation in Patients With Multiple System Atrophy: A Randomized Controlled Trial