Toward a better understanding of MSA

I have Multiple System Atrophy (MSA). This is a progressive, degenerative, and fatal disease of the brain. At this time it affects multiple areas of my brain including my left cerebellum, my midbrain, my left frontal lobe, and my left temporal lobe. The autonomic system that controls blood pressure, balance, and speech, if the most noticeable.

Some of my symptoms include:

• Balance problems: Falling frequently and unexpectedly
• Vertigo and/or dizziness when standing or walking
• Unable to walk or stand for more than a short period of time
• Hyperventilation during movement
• Muscle weakness
• Inability to look up or to the side when walking, resulting in bumping my head on anything that projects out just above my eyes
• Speech problems, stuttering, unable to speak the words I am thinking
• Hesitation when asked a question as I translate the words
• Choking and swallowing problems, especially when drinking liquids
• Taste and smell differences
• Inco-ordination – knock things over and drop things frequently, writing is difficult
• Muscle stiffness after sitting for more than a short time
• Breathing difficulties

What is the progression of MSA?

Disease progression in MSA is quicker than in Parkinsonism. Almost 80% of patients are disabled within 5 years of onset of the motor symptoms, and only 20% survive past 12 years. The mean survival is roughly 6 years. Rate of progression differs in every case and speed of decline may vary widely in individual patients.

Patients usually have autonomic nervous system dysfunction first. When MSA begins with non-autonomic features, imbalance is the most common feature. This difficulty in maintaining balance may be due to either cerebellar or Parkinsonian abnormalities. Some patients complain of stiffness, clumsiness, or a change in handwriting at the onset of MSA.

MSA progresses over the course of several years to cause more widespread and severe symptoms. Orthostatic hypotension can cause fainting and falls. Loss of coordination, slowed movements, and rigidity can interfere with activities of daily living.

Some patients with MSA have mild loss of cognitive abilities, with impairments in attention and speed of thinking.

Complications include:

Progressive loss of ability to walk or care for self

Difficulty performing daily activities

Injuries from falls/fainting

Side effects of medications

Orthostatic hypotension (a large drop in blood pressure upon standing) is common and may cause dizziness, dimming of vision, head or neck pain, yawning, temporary confusion, slurred speech, and if the hypotension is severe, the patient may "faint" upon arising from a recumbent position.

In spite of low blood pressure while standing, it is common for MSA patients to have high blood pressure when lying down. A fall in blood pressure following meals or in hot weather or following infection is quite common.

The concurrent involvement in MSA of multiple brain systems subserving movement, including the striatum, cerebellum, and cortex, leads to the movement disorder as often being the most profound disability. Hoarseness or even vocal paralysis is relatively common, as are sleep disturbances, including snoring and sleep apnea. The ability to swallow foods and liquids may be impaired.

How long can I expect to live?

The probable outcome is poor. There is a progressive loss of mental and physical functions until general debilitation develops. Early death is likely. The mean survival is roughly 6 years. Most people who are diagnosed with Shy-Drager syndrome die within seven to 10 years after symptoms begin. It is rare for a patient to survive 10 years.

The autonomic abnormalities are seldom the direct cause of death. A significant number of patients develop laryngeal stridor and difficulty swallowing, which can lead to pneumonia.

Pneumonia is the most common cause of death, although irregularities in heartbeat or choking may be responsible for death in some patients. Breathing problems such as aspiration, stridor (high-pitched breathing sounds due to airway obstruction), or cardiopulmonary arrest are common causes of death.

In addition, many patients with MSA experience periodic respiration and in some cases this may lead to a critical loss of respiratory drive, so called Ondine's curse.

Pulmonary hypertension may occur during apnea. The most common causes of death in patients with MSA are pulmonary embolus, apnea, and intercurrent infection.

Treatment

Currently there is no cure for MSA and no known means to slow progression of the disease. A number of drugs can be used to treat the various symptoms of MSA, although they become less effective as the disease progresses.