Shy-Drager Syndrome, also known as Multiple System Atrophy, is a
rare degenerative disorder which causes progressive damage to the
central and autonomic nervous systems. Its
cause is unknown; thus, there is no known way to prevent the disorder, and no known cure. It develops gradually and is most often diagnosed
in men over 60 years old. The disease is characterized by postural (orthostatic) hypotension, which is an excessive drop in blood
pressure when you stand up, resulting in dizziness or fainting.
There are three types of Multiple System Atrophy:
- the cerebellar form, which affects balance, coordination and
- the parkinsonian form (striatonigral degeneration) which causes
stiff muscles, slow movement, and mild tremors;
- and mixed cerebellar and parkinsonian.
SDS was named for neurologists Milton Shy, M.D., from the National
Institutes of Health, and Glenn Drager, M.D., from the Baylor College
of Medicine, who first described the condition in 1960.
Blood pressure is unstable in the majority of patients, often
fluctuating wildly and causing severe headaches.
Diagnosis can be difficult, especially in the early stages. There are
no specific tests to confirm this disease, so the patient must be
assessed carefully by a neurologist.
There is no known cure for Shy-Drager Syndrome, and no way to slow its
progression, so treatment is limited to controlling symptoms. The
associated hypotension is treatable. Dietary increases of salt and
fluid are often recommended, but medications (such as corticosteroids)
are often necessary to increase blood pressure. Anti-Parkinson
medications, such as L-dopa, may be helpful in controlling
Parkinson-like symptoms, but they can often lower blood pressure and
should be used with caution. An artificial feeding tube or breathing
tube may be surgically inserted for management of swallowing and
breathing difficulties. In rare cases, a pacemaker may be implanted
to correct heart irregularities.
Not good. While some patients may live for up to 20 years
after the onset of the first symptoms, most patients become severely
disabled within 7 years. Death usually occurs within 10 years after
the symptoms first appear. The most common causes of death are
pneumonia (due to swallowing difficulties) and pulmonary embolus.