Shy-Drager Syndrome

Definition

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 speech;
• 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.

Symptoms

• Dizziness or fainting when standing up
• Constipation
• Impotence
• Loss of the ability to sweat
• Incontinence
• Vision changes, decreased or blurred vision
• Muscle rigidity
• Stiffness
• Movement difficulties
• Loss of balance
• Change in gait
• Shuffling
• Slow movements
• Muscle aches and pains (myalgia)
• Tremors
• Voice and/or speech changes
• Breathing and swallowing difficulties

Blood pressure is unstable in the majority of patients, often fluctuating wildly and causing severe headaches.

Diagnosis

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.

Prognosis

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.