Shy-Drager Syndrome as described by the National Institute of Neurological Disorders and Stroke
What is Shy-Drager Syndrome?
Multiple system atrophy (MSA) with postural hypotension, also called Shy-Drager syndrome, is a progressive disorder of the central and sympathetic nervous systems. The disorder is characterized by postural (or orthostatic) hypotension’an excessive drop in blood pressure when the patient stands up, which causes dizziness or momentary blackouts. MSA has been classified clinically into three types, olivopontocerebellar atrophy (OPCA), which primarily affects balance, coordination, and speech; a parkinsonian form (striatonigral degeneration), which can resemble Parkinson’s disease because of slow movement and stiff muscles; and a mixed cerebellar and parkinsonian form. In all three forms of MSA, the patient can have orthostatic hypotension. Orthostatic hypotension and symptoms of autonomic failure such as constipation, impotence in men, and urinary incontinence usually predominate early in the course of the disease. Constipation may be unrelenting and hard to manage. Shy-Drager syndrome may be difficult to diagnose in the early stages. For the majority of patients, blood pressure is low when the patients stand up and high when the patients lie down. Other symptoms that may develop include impaired speech, difficulties with breathing and swallowing, and inability to sweat.
Is there any treatment?
Orthostatic hypotension in Shy-Drager syndrome is treatable, but there is not known effective treatment for the progression central nervous system degeneration. The general treatment course is aimed at controlling symptoms. Antiparkinsonian medication, such as L-dopa, may be helpful. To relieve low blood pressure while standing, dietary increases of salt and fluid may be beneficial. Medications to elevate blood pressure, such as salt-retaining steroids, are often necessary, but they can cause side effects and should be carefully monitored by a physician. Alpha-adrenergic medications, non-steroidal anti-inflammatory drugs, and sympathomimetic amines are sometimes used. Sleeping in a head-up position at night reduces morning orthostatic hypotension. An artificial feeding tube or breathing tube may be surgically inserted for management of swallowing and breathing difficulties.
What is the prognosis?
Shy-Drager syndrome usually ends in the patient’s death by 7 to 10 years after diagnosis. Breathing problems such as aspiration, stridor (high-pitched breathing sounds due to airway obstruction), or cardiopulmonary arrest are common causes of death.
What research is being done?
The NINDS carries out and funds research about disorders of the autonomic nervous system, including Shy-Drager syndrome. This research is aimed at discovering ways to diagnose and treat disorders of the autonomic nervous system and ultimately to cure or prevent them.
Information provided by the
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Article Created: 1999-03-20
Article Updated: 1999-03-19