What is Shy-Drager Syndrome?
Shy-Drager Syndrome which is also referred to as Multiple System Atrophy is an extremely rare neurological condition in which the autonomic functions of the body get impaired. This includes blood pressure, heart rate, bladder function, and digestive system. Other characteristic features of Shy-Drager Syndrome resemble that of Parkinson Syndrome like slow movement, balance difficulties, and stiff muscles. Shy-Drager Syndrome usually affects people who are above the age of 60 years.
Treatment for Shy-Drager Syndrome is basically supportive as there is no definite cure and medications and lifestyle modifications are done to control symptoms. Eventually, this disease gradually takes its toll on the patient and eventually the patient succumbs to Shy-Drager Syndrome.
What Causes Shy-Drager Syndrome?
What causes Shy-Drager Syndrome is not known as of yet. A genetic component to Shy-Drager Syndrome is being studied to see whether this condition is inherited in any form. Any type of environmental toxin responsible for the development of Shy-Drager Syndrome is also being studied but as of now there has been no proof of such. In Shy-Drager Syndrome, there is gradual deterioration and atrophy of some portions of the brain which are responsible for functioning of vital internal functions of the body like the blood pressure, digestion, and movement. When a damaged brain tissue of an individual with Shy-Drager Syndrome is examined under a microscope it reveals abnormally high amount of a protein called alpha-synuclein. Some studies suggest that may be responsible for development of Shy-Drager Syndrome.
What are the Symptoms of Shy-Drager Syndrome?
Shy-Drager Syndrome has primarily two types of symptoms of which one is parkinsonian type symptoms and the other is cerebellar type symptoms. The symptoms of parkinsonian type of Shy-Drager Syndrome are:
- Muscle stiffness with problems with bending the arms and legs
- Slowness of movement
- Balance difficulties.
The symptoms of cerebellar type of Shy-Drager Syndrome are:
Additionally, some of the other signs and symptoms of Shy-Drager Syndrome are:
- Orthostatic hypotension with development of abnormally high blood pressure when lying down
- Urinary and bowel dysfunction
- Urinary and bowel incontinence
- Abnormal sweating
- Sleep abnormalities
- Loss of libido
- Irregular heartbeat.
How is Shy-Drager Syndrome Diagnosed?
Diagnosing Shy-Drager Syndrome is a difficult task to do as the symptoms caused by it are similar to many other degenerative disorders like Parkinson Disease and hence it is very difficult to definitively diagnose Shy-Drager Syndrome. This is the reason that there are many cases which remain undiagnosed or are misdiagnosed as something else, although doctors these days are more aware of this condition and are more likely to identify this condition than before. If Shy-Drager Syndrome is suspected in an individual, then the doctor will take a detailed history of the patient inquiring as to when the symptoms started and how much severe the symptoms are.
A physical examination will also be performed to check for any muscle stiffness. Additionally, radiological studies in the form of CT or MRI scan of the brain will be ordered which will clearly show lesions in the brain which may be damaging the brain tissues and causing the symptoms pointing towards a diagnosis of Shy-Drager Syndrome. Additionally other tests to monitor the irregularities in the blood pressure such as a tilt table test may also be done.
The patient may also undergo tests to look for any autonomic dysfunction like testing the bowel and bladder function of the patient, blood pressure monitoring, ophthalmologic examination to check the status of the eyes, EMG and nerve conduction studies to check the status of the functioning of the muscles and nerves.
These tests will be confirmatory enough for the doctor to diagnose Shy-Drager Syndrome.
How is Shy-Drager Syndrome Treated?
As of now there is no cure for Shy-Drager Syndrome and treatment is basically symptomatic and supportive aimed at controlling the symptoms and the make the patient as comfortable as possible and allow the patient to be as independent as he or she can be. Medications may be prescribed for blood pressure control. A medication called mestinon is quite effective in controlling the blood pressure both while standing and lying down. Medications to reduce parkinsonian symptoms are also recommended like sinemet to control muscle stiffness, balance difficulties, and slowness of movement, although not everyone with Shy-Drager Syndrome responds positively to these medications and moreover the efficacy of these medications decrease with time.
Symptoms like irregular heartbeat may require placement of a pacemaker while symptoms of decreased libido may be treated with medications like Viagra. Speech therapy will be recommended for the patient to deal with dysphagia. For bladder control problems this may be controlled with medications initially but as the disease process advances with time the patient may require catheter insertion for draining the bladder. Muscle strength and coordination can be managed by a physical therapist.
What is the Prognosis & Life Expectancy of Shy-Drager Syndrome?
The prognosis for Shy-Drager Syndrome is not that good as this condition is a disease process that progresses gradually with time. The maximum that a patient with known diagnosis of Shy-Drager Syndrome lives is around 10 years from the time of the diagnosis, with the patient becoming more and more dependent with time as there is progression of Shy-Drager Syndrome.
- National Organization for Rare Disorders (NORD). (2022). Multiple System Atrophy. https://rarediseases.org/rare-diseases/multiple-system-atrophy/
- National Institute of Neurological Disorders and Stroke (NINDS). (2022). Multiple System Atrophy Fact Sheet. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Multiple-System-Atrophy