Multiple system atrophy (MSA) (also sometimes referred to as Neurolical orthostatic hypotension; Shy-McGee-Drager syndrome or Parkinson Plus syndrome) is a rare neurodegenerative disorder characterized by progressive damage to the autonomic nervous system (the portion of the nervous system that controls involuntary functions such as bodd pressure, heart rate, digestion and sexual function), muscle tremor and regidity, movement and other body functions; hence the label "multiple system" atrophy.
MSA often appears to be similar to Parkinson's Disease and/or other nuerological conditions such as Progressive Supranuclear Palsy and is often misdiagnosed. Diagnosis is largely clinical as there are no specific tests during life to confirm the disease. A neurologist makes the diagnosis based on the history of symptoms and the findings on physical examination as well as ruling out other causes.
An eye examination may reveal atropy of the iris and paralysis of eye muscles. Postural hypotension (drop in blood pressure associated with change in position) is evident. A neuromuscular examination shows abnormal reflexes and may show severe muscle wasting (atropy). Parkinsonian movements (tremor, rigidity, and slow movements similar to Parkinson's disease) are common.
The cause of MSA is unknown. There is progressive damage (degeneration) of the nervous system, with damage to all parts of the nervous system. The disorder develops gradually. It is most often diagnosed in men over 60 years old. There is no known prevention for this rare disorder.
As the term "multiple system atrophy" implies, MSA can vary in its affect on the nervous system, resulting in a wide range of symptoms that may differ in various patients.
MSA is generally separated into three main categories depending on the predominant symptoms. These are Shy-Drager syndrome, striatonigral degeneration, and olivopontocerebellar atrophy - all are now considered forms of MSA.
In Shy-Drager syndrome, the most prominent symptoms are those involving the autonomic system. The autonomic system strongly influences and regulates blood pressure, pulse rate, sweating, intestinal motility, bladder and sexual function - functions not involving conscious control.
Striatonigral degeneration causes parkinsonian symptoms such as slowed movements, rigidity, and impaired balance. Speech and swallowing may also be affected.
Olivopontocerebellar atrophy principally affects balance, coordination, and speech. It is the term applied to the MSA variant manifesting prominent "ataxia" along with parkinsonism.
MSA can cause a wide range of symptoms, including:
MSA progresses over the course of several years to cause more widespread and severe symptoms. Orthostatic hypotension can cause fainting and falls. Loss of coordination, slowed movements, and rigidity can interfere with activities of daily living. Some patients with MSA have mild loss of cognitive abilities, with impairments in attention and speed of thinking.
There is no cure for the disorder and no known means to slow progression. The treatment is aimed at controlling symptoms such as postural hypotension and parkinsonian movements.
Pharmacological treatment is much the same as Parkinson's disease, although as the disease progresses the benefits of these drugs diminish and often complicate the autonomic dysfunction, particularly orthostatic hypotension (low blood pressure).
Anticholinergic medications may be used to reduce early and mild tremors. If tremors are not present they should be used only after considering the possible side-effects. If there is a shortage of the neurotransmitter acetylcholine (such as there is with PSP and Alzheimer's Disease) they may worsen the patients condition.
Levodopa and dopamine agonists used to treat Parkinson's disease are also effective for the slowness and rigidity of MSA and may improve movement and balance. Carbidopa may reduce the side effects of Levodopa and make the Levodopa work better. Many affected individuals respond poorly to treatment with anticholinergis or Levodopa.
Sympathomimetics, vascoconstrictors, beta-blockers, MAO inhibitors, vasopressin, fludrocortisone (Florinef), midodrine (ProAmatine), or other medications may be used to treat low blood pressure (postural hypotension). They may also tend to limit the delivery of nutrition to the brain. The only way nutrition is provided to the brain is via the bloodstream.
Male impotence may be treated with penile implants or drugs.
Incontinence may be treated with medications or catheterization.
Constipation may improve with increased dietary fiber or laxatives.
A speech-language pathologist may be able to offer strategies for improving swallowing and speaking.
Use of softer foods may improve the ability to swallow.
A gastrostomy tube, which delivers foods directly to the stomach, may be needed later in the disease course.
Other complications include: progressive loss of ability to walk or care for self, difficulty performing daily activities, injuries from falling and side effects of medications.
The probable outcome is poor. There is a progressive loss of mental and physical functions until general debilitation develops. Early death is likely. The rate of progression differs in every case and speed of decline may vary widely in individual patients.
Call your health care provider if:
Advocacy/Support Organizations can help the MSA patient and caregiver learn coping strategies and offer support for the difficulties encountered in dealing with MSA.