Shy-Drager syndrome is a rare and progressive neurological disorder. The condition has several other names, including sporadic olivopontocerebellar atrophy and striatonigral degeneration. Each of these terms has fallen out of use in favor of “multiple system atrophy.” In most cases, the disease worsens rapidly over several years. Shy-Drager syndrome primarily affects people over the age of 30, though it is most prevalent in individuals in their mid-50s. Individuals with the condition sometimes experience symptoms similar to Parkinson’s disease, though Shy-Drager syndrome tends to affect more of the body.
Physicians place Shy-Drager syndrome into one of two subdivisions, based on its symptoms. These subdivisions, MSA-C and MSA-P, use the umbrella term of multiple system atrophy rather than Shy-Drager syndrome. MSA-C refers to the cerebellar phenotype and has symptoms involving the brain and coordination. MSA-P refers to the parkinsonian phenotype and the symptoms of Shy-Drager syndrome that closely resemble those of Parkinson’s disease.
It is important to note that the classification only refers to which group of symptoms is most prominent when a doctor evaluates a patient. A person with MSA-P will likely have MSA-C symptoms and vice versa. Additionally, both types typically feature postural hypotension, a form of low blood pressure that makes a person feel dizzy when they stand up.
Around 90% of people with Shy-Drager syndrome have parkinsonian symptoms. Because of this, there is a history of doctors misdiagnosing the disease as Parkinson’s. Common symptoms of MSA-P include rigid muscles, difficulty bending the joints, and slow movements. These may also manifest as issues with posture and balance. The most common first sign of Shy-Drager syndrome is a parkinsonian symptom: akinetic-rigid syndrome. This refers to the slowness with which a person begins their movements. Rarely, a person with Shy-Drager syndrome may have Parkinson’s-like tremors.
In addition to the parkinsonian symptoms, people with Shy-Drager syndrome may have a group of symptoms known as cerebellar ataxia. These symptoms consist of a general lack of voluntary coordination of muscle movements that originates in the cerebellum. This often begins as poor coordination and unsteady walking. Some people experience slurred speech, or their eyes may make repetitive and uncontrolled movements. It is also typical for people with cerebellar symptoms to have difficulty chewing or swallowing, and they may experience blurry or double vision.
Beyond the parkinsonian and cerebellar symptoms are several other possible categories of symptoms. Though there is some overlap between them, it is still helpful for physicians to be able to refer to each group individually. Almost 97% of people with Shy-Drager syndrome have autonomic dysfunction or dysautonomia, a condition where the autonomic nervous system fails to work properly. This affects the bladder, intestines, pupils, blood vessels, sweat glands, and heart. This often appears as urinary and rectal incontinence, though it can also cause organ failure.
Beyond this are the pyramidal signs of hyperreflexia and spasticity, which refer to exaggerated reflexes and increased muscle tone, respectively. These typically contribute to stiffness, slowness, and muscle spasms. There are also seemingly separate symptoms such as inappropriate laughing or crying. Almost every male with Shy-Drager syndrome experiences erectile dysfunction and impotence.
Shy-Drager syndrome tends to progress quickly, though it is variable. However, the disease does not go into remission and may eventually cause life-threatening complications. Injuries from falls or fainting become more common, and many people develop breathing abnormalities during sleep. Progressive immobility can lead to secondary issues such as a breakdown of the skin, deep vein thrombosis, and pulmonary embolism. As the autonomic dysfunction worsens, organs may begin to fail.
Despite ongoing research, physicians and researchers are still unaware of the cause of Shy-Drager syndrome. This is partly because the disease occurs at random. What physicians do know is that the disorder features an accumulation of the protein alpha-synuclein in the glial cells that assist the brain’s nerve cells. Interestingly, these accumulations are also present in Parkinson’s disease, but in the nerve cells themselves. It is possible that the alpha-synuclein protein holds the answer to the cause of both diseases.
Shy-Drager syndrome appears to affect both males and females equally. Most people tend to develop the disease between 55 and 60 years old, though the disease has affected people from 30 to 90. Current studies suggest that around 1,900 people develop the disease each year in the United States. In comparison to similar diseases, there is one living case of multiple system atrophy for every 40 cases of Parkinson’s disease. Incidentally, this statistic is only accurate because people with Parkinson’s tend to live longer than people with multiple system atrophy.
Because Shy-Drager syndrome resembles Parkinson’s disease, it is sometimes difficult for doctors to tell the difference between the two. MRIs may be able to demonstrate changes in various brain areas. A tilt table test can confirm orthostatic hypotension. Blood tests, sweat tests, electrocardiograms, and bladder assessments may detect autonomic dysfunction. Additionally, some doctors may evaluate patients in a sleep laboratory to discover sleep abnormalities.
Unfortunately, there is no cure and no specific treatment for Shy-Drager syndrome. Instead, treatments aim to alleviate the symptoms. In some cases, medications that treat Parkinson’s are also effective in treating the parkinsonian symptoms of Shy-Drager syndrome. Increasing salt consumption can help improve orthostatic hypotension. As the disease progresses, physicians may install feeding tubes to prevent aspiration. Wheelchairs or other assistance devices allow individuals with the condition to maintain some level of mobility.
Many ongoing studies and research trials that are attempting to discover ways to combat Shy-Drager syndrome. Some of this research is focusing on the role that alpha-synuclein clumping plays in the disease. Because it is similar to other neurological conditions, some scientists are using diagnostic imaging to determine specific trait markers to help the process. Many drug trials also use a variety of medications in attempts to combat the disease. Some individuals with Shy-Drager syndrome have formed groups that allow them to stay in contact and help answer questions that others may have about the condition.
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