Shy Drager Syndrome
Shy Drager Syndrome
(Multiple System Atrophy)
Shy-Drager Syndrome (Multiple System Atrophy) (SDS)(MSA) is a a rare degenerative condition resulting from degeneration of certain nerve cells in the brain and spinal cord. Body functions controlled by these areas of the brain and spinal cord do not function normally in sufferers. These functions include the autonomic or involuntary nervous system (which controls blood pressure, heart rate, and bladder function) and the motor system (which controls balance and muscle movement).
The syndrome is named after the two doctors who first identified it in 1960. The cause is unknown. It affects one in 10,000 people and is most often diagnosed in men older than 60. It is two or three times more common among men than among women.
Depending upon which part of the brain is affected first, MSA may appear in different ways. Sometimes it presents with low blood pressure on standing, urinary bladder problems, or difficulties with balance and movement that resemble Parkinson's disease. Early symptoms often include impotence and urinary incontinence. Most patients complain of severe constipation and later develop rectal incontinence.
Orthostatic hypotension – an excessive drop in blood pressure when the patient stands up causing light-headedness or dizziness - is a universal feature of SDS. It is worse after meals and early in the day. When orthostatic hypotension becomes severe (i.e. blood pressure of less than 70/40) ‘blackouts’ may result. When the patient falls to a prone position, the blood pressure returns toward normal and the patient will regain consciousness.
Many patients also complain of dry mouth and dry skin, and because of abnormal sweating have problems with relation of body temperature. The hands and feet will be cold.
The autonomic nervous system is also responsible for constriction and dilation of pupils. Some patients with SDS complain of difficulties with focusing and other visual symptoms. In the later stages of the disease, as a result of autonomic dysfunction, some patients develop breathing problems including loud respiration and cessation of breathing during sleep. Irregularities in heart beat may be responsible for demise in some patients with SDS.
Non-autonomic symptoms include Parkinsonism (slowness of movement, stiffness of muscles, mild tremors, and loss of balance), difficulty moving eyes causing double vision and problems with focusing, problems controlling emotions and wasting of muscles. During the final stages of the disease, patients have trouble chewing, swallowing, speaking and breathing.
There is no cure for MSA, and there is no known means to slow progression. Treatment is aimed at controlling symptoms such as hypotension and parkinsonian movements.
Anti-parkinsonian medication, such as L-dopa may be helpful. Dietary increases of salt and fluid may be helpful. Medications to elevate blood pressure, such as salt-retaining steroids, are often necessary, but they can cause side effects and should be carefully monitored.
Sleeping in a head-up position at night reduces morning orthostatic hypotension. Male impotence may be treated with penile implants or drugs. Incontinence may be treated with medications or catheterisation. Constipation may improve with increased dietary fibre or laxatives.
The probable outcome is poor. There is a progressive loss of mental and physical functions until general debilitation develops. Shy-Drager usually ends in death 7 to 10 years after the onset of symptoms. The rate of progression differs in every case and the speed of decline may vary widely in individual patients.
Pneumonia is the most common cause of death, although irregularities in heart beat or choking may be responsible for death in some patients.
Note: Not all specialists use the term "Shy-Drager" to mean precisely the same condition, and diagnostic certainty cannot at present be achieved during life. Please check with your physician/neurologist if this information appears to conflict with what you may have been told.
Further Information and Support
National Institute of Neurological Disorders and Stroke
Worldwide Education and Awareness for Movement Disorders
Shy-Drager Syndrome Support Group
National Institute of Health - USA
Reviewed by Associate Professor James Colebatch