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Multiple System Atrophy (MSA)

Definition


Disease: Multiple System Atrophy (MSA) Multiple System Atrophy (MSA)
Category: Neurological diseases
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Disease Definition:

The rare neurological disorder that impairs the body's involuntary (autonomic) functions such as digestion, heart rate, blood pressure and bladder function is known as multiple system atrophy (MSA), formerly called Shy-Drager syndrome. This condition shares many Parkinson's disease-like symptoms, like poor balance, slowness of movement and muscle rigidity.

Multiple system atrophy affects more men than women, and it is a degenerative disease that develops in adulthood, usually in the 50s. This condition, which eventually leads to death, is gradually progressive.

To help manage symptoms, lifestyle changes and medications are included in the treatment of MSA.

Work Group:


Prepared by: Scientific Section

Symptoms, Causes

Symptoms:

As its name indicates, the signs and symptoms of multiple system atrophy affect multiple parts of the body.

A dysfunction in blood pressure control mechanisms is found in people with multiple system atrophy, which may result in problems such as:

Extreme high blood pressure:
While lying down, one can develop dangerously high blood pressure.

Extreme low blood pressure:
Postural (orthostatic) hypotension, a form of low blood pressure that makes one feel lightheaded or dizzy, or even faint, when he/she stands up from sitting or lying down, is the primary sign of multiple system atrophy.

The following may be included in the signs and symptoms of multiple system atrophy:

 

  • In men, impotence and loss of libido
  • Agitated sleep due to "acting out" one's dreams
  • Loss of bladder or bowel control (incontinence)
  • Irregular heartbeat
  • Difficulty controlling emotions
  • Rigid muscles and difficulty bending the arms and legs
  • High blood pressure while lying down
  • Low blood pressure when standing up
  • Impaired ability to swallow
  • Impaired movement and coordination (unsteady gait, loss of balance)
  • Abnormal breathing at night
  • Impaired control of body temperature, often causing cold hands or feet as well as heat intolerance
  • A reduction in the production of perspiration, tears and saliva
  • Slurred speech; slow or low-volume speech
  • Constipation
  • Visual disturbances such as blurred vision or difficulty focusing the eyes


A person should see the doctor for an evaluation and diagnosis in case he/she develops any of the signs and symptoms associated with multiple system atrophy. In case a person has already been diagnosed with multiple system atrophy and starts experiencing symptoms or if existing symptoms worsen, he/she should see a doctor.

Causes:

Deterioration and shrinkage (atrophy) of portions of the brain (brainstem, cerebellum and basal ganglia) that regulate internal body functions, motor control and digestion, are associated with multiple system atrophy.

What exactly causes the brain changes in MSA is still not known. There is no substantial evidence to support the theories that there's an environmental toxin or inherited component involved in the disease process, but these theories are being studied.

Nerve cells (neurons) that contain an abnormal amount of a protein called alpha-synuclein can be seen when damaged brain tissue of people with multiple system atrophy is evaluated under a microscope. In multiple system atrophy, this protein is suggested to be overexpressed.

Complications

Complications:

Multiple system atrophy does not go into remission, and its rate of progression varies from one person to another. Daily activities become increasingly difficult as the disorder progresses.

The following complications may be caused by multiple system atrophy:

 

  • Vocal cord paralysis, which makes speech and breathing difficult
  • Injuries from falls associated with impaired walking
  • Breathing abnormalities during sleep
  • Progressive immobility that can lead to secondary problems such as a breakdown of the skin
  • Increased difficulty swallowing
  • Loss of ability to care for one's self in day-to-day activities, from bathing to brushing teeth.


Although some people with this condition have lived 15 years or longer, after the symptoms of the disease first appeared, usually people live about six to nine year. Respiratory problems are often the cause of death.

Treatments:

Multiple system atrophy has no cure. Treating signs and symptoms to make the patient maintain his/her body functions and capabilities and to make him/her as comfortable as possible are involved in the management of the disease.

The following may be recommended to treat specific signs and symptoms:

Medications to raise blood pressure:
By leading to more retention of salt and water, blood pressure may be increased by medications such as the corticosteroid fludrocortisone. This steroid drug is prescribed on a twice-a-day regimen. Without increasing the blood pressure of the patient while lying down, the drug pyridostigmine can raise his/her standing blood pressure.

Midodrine is one of the medications that can quickly increase standing pressure. However, in some people, the risk of stroke can be increased by elevated pressure while lying down, which can be caused by midodrine.

Pacemaker:
To keep the heart of the patient beating at a rapid pace, which can increase the blood pressure, implanting a heart pacemaker may be suggested.

Medications to reduce Parkinson's-like signs and symptoms:
To reduce Parkinson's-like signs and symptoms such as slowness of movement, stiffness and balance problems, and to improve an overall feeling of well-being, certain anti-Parkinson's medications such as combined levodopa and carbidopa, can be used.

After a few years of therapy, the effectiveness of Parkinson's drugs may decline, and not everyone with multiple system atrophy responds to these medications.

Bladder care:
Medications can help in the earlier stages if the patient is experiencing bladder control problems. To allow the patient drain his/her bladder, he/she may need to have a soft tube (catheter) inserted permanently, and this happens eventually when the disease becomes advanced.

Impotence drugs:
With a variety of drugs, such as sildenafil , designed to manage erectile dysfunction, impotence can be treated.

Swallowing and breathing difficulties:
One should try eating softer foods if he/she experiences difficulty swallowing. To manage the problem, a feeding or breathing tube may need to be surgically inserted in case swallowing or breathing becomes increasingly problematic. A tube (gastrostomy tube) that delivers food directly into the stomach may be required later in the disease process.

As the disorder progresses, a physical therapist can help the patient maintain as much of his/her motor and muscle capacity as possible.  Additionally, a speech-language pathologist can help the patient learn to improve or maintain his/her speaking ability.

Prognosis:

Not Available

Expert's opinion

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