Multiple system atrophy (MSA) is a slowly progressive neurodegenerative disease that afflicts approximately 25,000-75,000 Americans, with approximately 10,000 new cases diagnosed each year. The number of cases is hard to estimate because MSA is similar to other disorders and many patients do not get the correct diagnosis during their lifetime.
MSA is one of the parkinsonian disorders that starts with non-motor symptoms and sleep disturbances.
The autonomic nervous system is the body's internal system that controls most of the involuntary functions of the body. Examples of this include: controlling urinary bladder function, maintaining blood pressure, and controlling breath and heart rate, and bowel motion. The failure of the function of this system is called "dysautonomia."
Studies have shown that blood pressure, urinary bladder and genital organs are affected early by MSA. The autonomic nervous system normally works to increase blood pressure when standing up quickly from a sitting or lying position to maintain blood supply to the brain. But
orthostatic hypotension occurs when the autonomic system fails, which can result in feeling about to faint, fatigue, tiredness or a partial or complete loss of consciousness. The main danger is the risk of falls and fall-related consequences.
Other examples of dysautonomia affecting the urinary bladder include: increased urinary frequency, urinary urgency, and feeling of incomplete urinary bladder emptying. The inability to maintain erection during sexual intercourse or losing morning erection could also occur as early manifestation of the disease.
Sleep disorders in MSA include rapid eye movement sleep behavior disorder (RBD), fragmented sleep, restless leg syndrome (RLS), and excessive daytime sleepiness. RBD is the most common among these symptoms, which usually precedes motor symptoms or may occur with its onset. It involves abnormal behavior during the sleep phase with rapid eye movement (REM sleep) in which the content of the dreams can be vivid, violent, and frightening and patients may act out their dreams. Obstructive sleep apnea (OSA), a frequent cessation of breath during sleep, may also occur.
MSA is one of the atypical parkinsonian syndromes. As in Parkinson's disease, MSA is characterized by slowness of movement that may lead to an increase in the amount of time needed to accomplish the activities of daily living (bradykinesia), decrease in amplitude of movement that may lead to decrease associated movements when walking, lower tone of voice (hypophonia), and stiffness of the muscles (rigidity). People with MSA may develop tremor, clumsiness, early shuffling gait, and frequent falls.
MSA has two main forms, depending on which symptoms are most prominent at the time we examine a person:
- The parkinsonian type (MSA-P), has primary characteristics similar to Parkinson's disease (such as moving slowly, stiffness, and tremor) along with problems of balance, coordination, and autonomic nervous system dysfunction
- The cerebellar type (MSA-C), features ataxia (problems with balance and coordination), difficulty swallowing, speech abnormalities or a quavering voice, and abnormal eye movements. Cerebellar relates to the part of the brain that controls coordination.
Both forms of MSA have similar survival rates, but MSA-P may affect quality of life more. Commonly, patients may end up having both parkinsonism and cerebellar features.
Although the cause of MSA is currently unknown, there is evidence that the primary defect occurs in glial cells, a type of cells in the nervous system that support and protect neurons in the brain, and help maintain physiological balance. Abnormal aggregation (clustering) of multiple types of protein in glial cells causes these cells to malfunction or die.
While there is no cure for MSA, we work to control and alleviate symptoms of the disease to avoid complications and to improve the quality of patients' lives.
At UC San Diego Health, we use a multidisciplinary management approach for MSA. Along with a movement disorder specialist, a urologist, physical, speech, and occupational therapists and dietitians are involved in the management plan.
Treatment for Dysautonomia (Dysregulation of the Autonomic Nervous System)
orthostatic hypotension, measures can be taken to prevent it, including: rising slowly from supine to sitting position, raising the head of the bed 20 to 30 degrees with a furniture raiser, and increasing daily salt and water intake. Using custom-fitted elastic stockings that extend to the waist or readjusting medication can help maintain blood pressure and prevent significant drops. Abdominal bands are also beneficial.
When these measures are insufficient, medications such as fludrocortisone that retain salt, or midodrine that increase the blood pressure may be needed.
Bladder function should be evaluated by a urologist, usually requiring a urodynamic study that allows the physician to better manage the "neurogenic bladder." If the bladder tends to be hyperactive with increased frequency of urination, medications (such as oxybutynin and tolterodine) can help to decrease frequency and urgency symptoms and improve bladder control.
Sleep and Breathing Disorders
REM sleep behavioral disorder symptoms can be treated by melatonin, a natural hormone in the human body that is available over the counter. When melatonin is not effective, other therapies could be tried such as gabapentin or clonazepam.
To improve obstructive sleep apnea, a machine that provides positive pressure ventilation with either a Continuous Positive Airway Pressure (CPAP) or Bilevel Positive Airway Pressure (BiPAP) system may be needed.
In at least one-third of patients, the parkinson's type symptoms may improve with antiparkinsonian medications. The best treatment is carbidopa-levodopa or other formulations of levodopa.
Physical therapy is also very important to prevent falls and maintain and improve gait. Our physical therapist helps determine the best walking aid when needed. A cane or a weighted walker (pictured right) with laser light may be very useful when there is freezing. Special programs like LSVT-BIG, an exercise treatment program for people with Parkinson's disease, can significantly improve motor symptoms.
Dystonia may improve with
botulinum toxin injections, usually administered by the movement disorder specialist. Stridor may benefit from botulinum toxin injections, but at times is so severe that it may require a tracheostomy.
Speech therapists assess the speech and swallowing functions. The LSVT-LOUD program for Parkinson's disease can improve voice projection and speech quality. A modified barium swallowing test helps determine if patients can tolerate all types of consistencies of food
Occupational therapists help improve the patient's performance of daily living activities, which promotes longer independence and better quality of life.
MSA is a disabling disease in its advanced stage. Social support through local, regional, and national associations can play an integral role in improving the lives of patients, family members, and caregivers.
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