Shy Drager Syndrome: Unraveling the Mysteries of a Rare Neurological Disorder

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Shy Drager Syndrome or now known by its present name, Multiple System Atrophy (MSA), is a rare neurological disorder that affects essential bodily functions such as mobility, speech, vision, and internal body functions such as blood pressure, digestive tract, and urinary tract. People will MSA may experience low blood pressure, constipation, or incontinence.

Just like other chronic illnesses, Shy Drager Syndrome or MSA is common in older adults who are above 60 and is more common in older men than women. Read on to learn more about Shy Drager Syndrome or MSA.

Renaming of Shy Drager Syndrome

Shy Drager Syndrome was discovered by a neurologist, Dr. Milton Shy from the National Institute of Health and Glen Drager of the College of Medicine wherein they first theorized that orthostatic hypotension is the culprit.

However, as researchers in the field continued to examine and investigate, They were able to come up with a deeper understanding of the disease. That’s why in 1998, Shy Drager Syndrome is changed to a more appropriate name which is Multiple System Atrophy or MSA.

SubTypes or Multiple System Atrophy

Also, with the continuous research on the disease, scientists have come up with two subtypes of Shy Drager Syndrome of MSA, namely:

  1. Cerebellar MSA (MSA-C) - This subtype usually attacks basic functions such as vision and speech difficulties.
  2. Parkinsonian MSA (MSA-P) - On the other hand, Parkinsonian MSA commonly affects muscles, movements, balance, and coordination.

What Are The Symptoms?

The two subtypes of MSA each have distinct symptoms while general symptoms are also observed. Let’s take a look first at the general symptoms which include:

  • Low Blood Pressure - Also called low blood pressure. This is characterized by a drop of blood pressure to an abnormal level such that your systolic pressure drops to 90 and diastolic pressure to 60. Dizziness or fainting when standing or lying down are the major symptoms of hypotension.
  • Sleep disorders - Sleeping disorders associated with MSA include REM sleep Behavior Disorder (RBD) where you act out dreams either by making sounds, kicking, or suddenly moving other extremities. People with MSA also experience difficulty or abnormal breathing while sleeping.
  • Sexual dysfunction - Also called impotence, refers to the loss of the ability to develop an erection. It can also include the weakening of libido and sexual drive.
  • Problems in the heart - Heart problems due to MSA include the pooling of blood on the feet and hands due to poor blood pressure or blood flow.
  • Psychiatric problems -  A recent research conducted revealed that patients with MSA have a higher frequency of showing signs of psychiatric problems such as depression, and difficulty controlling emotions.
  • Problems in the bowel and the urinary tract - Another evident symptom of MSA include problems in the digestive system and urinary tract. Examples of this can be constipation or difficulty having bowel movements, and urinary incontinence which refers to the loss of bladder control.
  • Abnormal sweating - This is characterized by the loss or the difficulty of your body to sweat which may result in intolerance to extreme heat and sometimes uncontrolled body temperature.
  • Weakness and fatigue - Aside from changes in various bodily functions, MSA is also said to cause weakness and fatigue. This is supported by a research article published in, wherein it stated that there is a high frequency of MSA patients who reported often feeling weak and fatigued.

Now that you know the general symptoms of MSA, let’s now discuss the symptoms of the two subtypes.

Cerebellar MSA

According to Healthline, 20 percent of people with MSA may experience symptoms under Cerebellar MSA which include:

  • Mobility impairment - This includes difficulty moving the joints and muscles throughout the body causing loss of balance.
  • Visual problems - This can be characterized by either blurred vision or the inability to focus.
  • Speech problems - This includes soft, slurred, and sometimes inaudible speech.
  • Mouth mobility problems - This includes difficulty chewing and swallowing affecting one’s nutrition and health.

Parkinsonian MSA

Symptoms under Parkinsonian MSA are more common and affect 90% of MSA patients. These symptoms include:

  • Muscle stiffening - This includes hardening of the muscles resulting in immobility, especially when bending the joints.
  • Muscle tremors - This refers to the involuntary shaking and shuddering of the muscles in the body that also affects mobility.
  • Posture and balance problems - Poor posture and loss of balance are also major symptoms of MSA.
  • Soft voice - Just like in Cerebellar type, parkinsonian MSA also affects the audibility of the voice.
  • Slow movements - This is due to the effects of muscle stiffness and tremors.

Note: If you will notice, Cerebellar MSA has symptoms that mainly affect coordination, speech, and vision while Parkinsonian MSA includes symptoms similar to Parkinson's that affect movements and muscles.

What Causes Shy Drager Syndrome?

The cause of Shy Drager Syndrome of MSA is still unknown and cases usually occur at random. Based on the latest research on the disease, a protein known as alpha-synuclein in high amounts is found in the damaged brain tissues of MSA patients.

In this case, researchers theorize that the unusual accumulation of these proteins is one of the causes of MSA since this protein is also present in people with Parkinson’s disease.

Though this may be an indicator of the primary cause of MSA, there’s still limited research and evidence to support this claim.

Is there a Treatment/Cure for Shy Drager Syndrome?

With the complexity and limited research about the disease, a cure for Shy Drager Syndrome is still yet to be discovered. If you are diagnosed with Shy Drager Syndrome, your doctor might prescribe medications that will alleviate the symptoms specifically in:

For Respiratory Problems

  • CPAP
  • Tracheostomy

For Hypotension

  • Midodrine

For mobility Problems

  • Ropinirole
  • Pramipexole
  • Amantadine

For Speech Problems

  • Speech therapy

For Urinary Problems

  • Oxybutynin
  • Desmopressin
  • Catheterization

For Sexual Dysfunction

  • Sildenafil
  • Tadalafil
  • Vardenafil

For Nutrient Deficiencies (caused by difficulty swallowing/chewing)

  • Food supplements
What Are the Complications of Shy Drager Syndrome?

Shy Drager Syndrome or MSA is a progressive disease, meaning the symptoms will get worse as it progresses. Though the speed of the progression varies from person to person, the patient will experience complications like:

  • Increased difficulty in chewing and swallowing
  • Difficulty breathing especially during sleep
  • Increased difficulty in maintaining balance which results in injuries from fractures.
  • Trouble accomplishing basic tasks and daily activities.
  • Paralysis in the vocal cords results in speaking difficulties.
  • Increased immobility which results in skin breakdown.

Patients with MSA typically live for 7-10 years starting from the onset of the symptoms. In most cases of MSA, patients die because of complications in their respiratory system and other infections.


Shy Drager Syndrome or now commonly referred to as MSA is a rare disease that affects various bodily functions. Though there’s still no cure for the disease, researchers and scientists are doing their best to increase their understanding of MSA. There are also treatments that you can take to slow the progression of the symptoms.

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