Multiple system atrophy

Multiple system atrophy (MSA) is a degenerative neurological disorder characterized by a combination of the following: more...

• Progressive damage to the nervous system, leading to low blood pressure when standing, difficulty urinating, and abnormal breathing during sleep
• Muscle tremor and rigidity (Parkinsonism)
• Slow movement (ataxia)

When autonomic failure predominates, the term Shy-Drager Syndrome is often used.

MSA is associated with the degeneration of nerve cells in specific areas of the brain. This cell degeneration causes problems with movement, balance and automatic functions of the body such as bladder control.

Nerve cells in the affected areas of the brain shrink (atrophy). When brain tissue of a person with MSA is examined under a microscope, structures called glial inclusion bodies can be seen. It is the presence of these inclusion bodies in the movement, balance and automatic control centres of the brain that confirms a diagnosis of MSA.

Symptoms

For men, the first sign is often erectile dysfunction (unable to achieve or sustain an erection). Both men and women often experience problems with their bladders including urgency, frequency, incomplete bladder emptying or an inability to pass urine.

As the disease progresses three groups of symptoms predominate. These are:

• parkinsonism (slow, stiff movement, writing becomes small and spidery)
• cerebellar (co-ordinating movement and balance)
• autonomic (automatic body functions) including:

— postural or orthostatic hypotension, resulting in dizziness or fainting when standing up

— urinary incontinence

— impotence

— dry mouth and skin

— trouble regulating body temperature due to abnormal sweating

— breathing difficulties

Not all patients experience all of these symptoms.

Prognosis

MSA usually progresses more quicly than Parkinsons disease (Bower, 1997). 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.

Treatment

There is no cure for MSA, so treatment involves treating the symptoms.

One particularly serious problem (the drop in blood pressure on standing up) often responds to fludrocortisone, a synthetic mineralocorticoid.

Levdopa (L-DOPA) does not alleviate the symtops of most MSA patients. In fact, poor response to Levdopa has been suggested as a possible element in the differential diagnosis of MSA from Parkinson's Disease.

Terminology

Other terms have been used to refer to this disorder, based on the predominant systems presented. These terms and their distinctions have been dropped in recent (1996 onwards) medical usage, but are helpful to understanding the older literature about this disease:

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