Multiple System Atrophy

1 What is Multiple System Atrophy (MSA)?

A rare neurological disorder that impairs your body’s involuntary (autonomic) functions including heart rate, blood pressure, digestion and bladder function is called multiple system atrophy (MSA). It is formerly called Shy-Drager syndrome.

The condition is just like Parkinson’s disease with symptoms such as poor balance, muscle rigidity and slowness of movement. This disease usually develops in adulthood, in 50's or 60's that is a degenerative disease.

Medications and lifestyle changes are the treatment to help manage symptoms and this can progress and may lead to death.

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2 Symptoms

Because of the signs and symptoms affect multiple parts of your body that’s why it is called multiple system atrophy.

This disease is classified by two parts:

  • Parkinsonian,
  • Cerebellar.

In parkinsonian, the signs and symptoms are like the Parkinson disease such as:

  • slow movement (bradykinesia),
  • difficulty bending your arms and legs and rigid muscles,
  • impaired balance and posture,
  • tremors (rare in MSA).

While in cerebellar, the symptoms are:

  • lack of muscle coordination (ataxia),
  • loss of balance,
  • slurred, slow or low volume speech (dysarthria),
  • unsteady gait,
  • double vision,
  • blurred vision,
  • difficulty focusing your eyes,
  • difficulty swallowing and chewing,
  • postural (orthostatic) hypotension which makes you feel dizzy or you might even faint especially when you are sitting down or lying down,
  • dangerous high blood pressure when lying down,
  • difficulty with body function that occurs involuntarily (autonomic) such as bowel dysfunction and urine dysfunction,
  • loss of bowel control (incontinence),
  • loss of bladder,
  • constipation,
  • sweating abnormalities,
  • there is a reduce in the production of perspiration, saliva and tears,
  • impaired control of body temperature,
  • sleep disorders,
  • sexual dysfunction, abnormal breathing at night,
  • impotence,
  • loss of libido,
  • irregular heart beat,
  • cardiovascular problem,
  • psychiatric problems,
  • difficulty controlling emotions.

Consult your doctor if you are having these symptoms.

3 Causes

There is no cause for brain changes in multiple system atrophy. Some researchers are studying if it is environmental toxin or inherited component involved but there is still no such evidence to support these theories.

MSA is linked with shrinkage (atrophy) and deterioration of portions of your brain (basal ganglia, cerebellum and brainstem) that regulate internal body functions, motor control and digestion.

When the damage brain tissue is evaluated under a microscope, it will reveal nerve cells (neurons) that contain an abnormal amount of a protein called alpha-synuclein.

This protein may be over expressed in multiple system atrophy according to some research.

4 Making a Diagnosis

Consult your doctor and he may refer you to a neurologist that specializes in brain and nervous system to receive a diagnosis of multiple system atrophy (MSA). You may bring a family member or a close friend in order for them to help you with relevant information and to support you.

Bring a notebook so that you can list all the things that you want to ask the doctor or things that he will tell you. You can also list down the symptoms that you are experiencing and the medications, supplements or vitamins that you are taking every day.

Write down the medical conditions that you had and family history. Write down the changes in your mood and emotional well being. You can also write down the changes in your sex life.

Here are some of the questions that you may ask your doctor:

  • What is causing my symptoms?
  • Is this Parkinson’s disease?
  • How will you make a diagnosis?
  • What test do I need?
  • Are there treatments?
  • What are the side effects of these treatments?
  • How will you monitor my health overtime?
  • Will this progress?

Your doctor will likely ask:

  • What are your symptoms?
  • Do you feel dizzy when you stand up?
  • Have you ever fainted?
  • Do you have emotional change?
  • Did your voice changed?
  • Do you have trouble sleeping?
  • Are you constipated?
  • Have you had any sexual problems?
  • Do you have difficulty swallowing?
  • Do you have a family history of Parkinson’s disease?

Multiple system atrophy is not inherited. Diagnosis of multiple system atrophy can be difficult for doctors because it has the same symptoms like the Parkinson’s disease. Some people are not properly diagnosed because of this. Your doctor will likely do a physical examination, blood tests and imaging test such as MRI to see if there are brain lesions that are present. Tilt table test to determine if you have a problem with blood pressure control. In this procedure, you will be strapped in a table, then it will be tilted upward and they will monitor your blood pressure and heart rate.

Doctors may also do other tests such as:

  • blood pressure measurement,
  • a sweat test to see perspiration,
  • a lying down and standing up test,
  • eye exam,
  • test for bladder and bowel function,
  • nerve and muscular examination,
  • electrocardiogram to track the electrical signs of your heart,
  • evaluation in sleep laboratory to see if you have sleep apnea.

5 Treatment

As of today, there is still no cure or treatment for multiple system atrophy.

Follow these to treat signs and symptoms of multiple system atrophy:

  • Medications to raise blood pressure such as corticosteroid fludrocortisones by leading more retention of salt and water to increase your blood pressure, this drug which is a steroid is taken twice a day;
  • pyridostigmine (Mestinon) that can raise your standing blood pressure without increasing your blood pressure while you are lying down;
  • midodrine (Orvaten); midodrine to elevate pressure when lying down;
  • droxidopa (Northera) for treating orthostatic hypotension that was recently approved by the FDA.
  • Medications to reduce Parkinson’s-like signs and symptoms such as combined carbidopa (Parcopa, Sinemet) and levodopa can be used for stiffness, slowness of movement and balance problems as well as overall feeling of well-being.
  • Pacemaker, to keep your heart beating at a rapid pace, your doctor may recommend implanting a heart pacemaker that can increase your blood pressure.
  • Impotence drugs such as sildenafil (Revatio, Viagra) to manage erectile dysfunction.
  • Bladder care medicines can help in the early stages of the symptom but if the disease becomes advance you might need to use a soft tube or catheter to allow you to drain your bladder;
  • eat small and soft foods if you are having a hard time swallowing or chewing, if disease becomes advance you might need a feeding tube or breathing tube (breathing difficulties), and if the disease is severe already you might need a gastrostomy tube to deliver the food directly into your stomach;
  • to improve or maintain your speaking ability, you may need a speech language pathologist;
  • to maintain your motor and muscle capacity you may need a physical therapist to help you.

6 Prevention

There is no prevention of multiple system atrophy (MSA).

As of today, doctors and researchers are still researching on how to prevent or even lessen the symptoms of multiple system atrophy.

7 Alternative and Homeopathic Remedies

There are no homeopathic or alternative remedies to cure multiple system atrophy.

8 Lifestyle and Coping

Lifestyle modifications are necessary in order to cope with multiple system atrophy (MSA).

To help minimize symptoms associated with multiple system atrophy, follow these self-care measures:

  • take steps to raise your blood pressure such as add a little salt to your salt and drinks,
  • drink a lot of fluid,
  • drink coffee to raise your blood pressure,
  • elevate the head of your bed to about 30-degree angle to minimize increase of blood pressure while you sleep,
  • get up slowly from a reclining position,
  • add more fiber to your diet to ease constipation,
  • avoid alcohol,
  • eat small meals instead of 3 heavy meals,
  • stay in cool places,
  • avoid excess of heat in bathing,
  • wear elastic support stockings up to your waist so you will not have low blood pressure.

9 Risks and Complications

There are several complications associated with multiple system atrophy (MSA).

The progress of multiple system atrophy varies but does not go into remission. Daily activities become difficult as the disease progress.

You may experience these complications:

  • injuries from falls because of impaired walking or fainting,
  • breathing abnormalities during sleep,
  • loss of ability to take care of yourself in daily activities such as brushing teeth,
  • progressive immobility that can lead to secondary problems such as breakdown of your skin,
  • vocal cord paralysis,
  • difficulty swallowing.

About seven to nine years is the life expectancy of a person diagnosed with multiple system atrophy, ten year survival is rare and respiratory problems is often the cause of death.