WHAT IS MULTIPLE SYSTEM ATROPHY?

MSA is a rare, rapidly progressing neurodegenerative disorder that affects the brain. MSA impairs the systems that regulate blood pressure, heart rate and the bladder – many of the basic bodily functions that people take for granted every day. People with MSA suffer from dangerously low blood pressure, speech and swallowing difficulties, sleep disturbances, breathing problems, rigidity and tremors. The life expectancy for those with MSA is typically 5 to 10 years. There is no remission of the disease. Almost 80% of patients are disabled within five years of the onset of the motor symptoms, and less than 20% of the cases survive beyond 10 years. Rate of progression and the speed of decline may vary widely from case to case. Patients with advanced MSA often become bed-bound, unable to speak and immobile. At present, there is no cure for MSA, no genetic tests to detect it, no therapy to slow it and very few treatments to manage its debilitating effects. Currently, diagnosis is clinical in nature with no certainty until an autopsy is performed or posthumous pathology examination. Learn More About MSA from the Mayo Clinic.

Doctor or scientist holding a model of a human brain

MSA 101

Multiple System Atrophy (MSA) is a rare, neurodegenerative disorder affecting multiple body systems. Major symptoms can occur in any combination including ataxia (loss of balance and coordination), severe low blood pressure leading to dizziness or fainting when standing, bladder retention or incontinence, constipation, male impotence, speech and swallowing difficulties, sleep disorders, breathing problems, and rigidity and tremor similar to Parkinson Disease. MSA can actually be considered three disorders in one.

  • Sporadic Cerebellar Ataxia
  • Atypical Parkinsonism (Parkinson Plus)
  • Autonomic Dysfunction (Dysautonomia)

In the past these terms were used to describe what were once thought to be three separate disorders:

  • Sporadic Olivopontocerebellar Atrophy (OPCA)
  • Striatonigral Degeneration (SND)
  • Shy-Drager Syndrome (SDS)

The currently recognized terminology includes only two types of MSA:

  • MSA-C when cerebellar symptoms are the first prominent symptoms
  • MSA-P when parkinson symptoms are the first prominent symptoms

The term MSA-A was once used to denote autonomic difficulties, however the most recent consensus guidelines on the diagnosis of Multiple System Atrophy specify that autonomic and/or urinary dysfunction is included in both MSA-C and MSA-P. Multiple System Atrophy is not known to be hereditary, but occurs sporadically with no known family history. It affects both males and females primarily in middle age. Statistical data gives the average age of onset at 50 and the average course of the disease at about 10 years. With knowledge and vigilant medical care, the most troubling symptoms can be managed however and life expectancy can be extended. For More on MSA, Visit the Mayo Clinic MSA Page.

LEARN MORE ABOUT MSA

MANAGING MSA

The cause of multiple system atrophy (MSA) remains unknown, and no current therapy can reverse or halt progression of the disease. The extra-pyramidal and cerebellar aspects of the disease are debilitating and difficult to treat.

NONPHARMACOLOGIC TREATMENT
  • Constipation – A high-fiber diet, bulk laxative, lactulose, and suppositories can prevent constipation
  • Stridor- Speech therapy is often useful to improve swallowing and communication
  • Deconditioning- Physical therapy and an aquatic exercise program (hypotension does not occur while patients are in water) prevent physical deconditioning of the patient unless the movement disorder aspect of the illness so impairs balance that this is not advisable
  • Urinary incontinence – Intermittent self catheterization or suprapubic or urethral catheterization can improve symptoms of urinary incontinence
  • Falls – As the disease progresses, the risk of falls increases; proper gait instruction and precautions are critical to prevent falls and resultant injury
PHARMACOLOGIC TREATMENT

Drug therapy is directed mainly toward alleviation of symptoms of the movement disorder and orthostatic hypotension. Urinary incontinence, constipation, erectile dysfunction, and supine hypertension can also be addressed through pharmacologic therapy.

CONSULTATIONS

Physical therapists, occupational therapists, speech therapists, psychologists, nutritionists and social workers can offer considerable practical help.

DIET

An essentially normal diet is recommended, with the following guidelines:

  • Increased salt and fluid intake maintains plasma volume
  • Small, frequent meals may help patients for whom postprandial hypotension is a significant problem
  • A high – fiber diet, bulk laxatives, and suppositories prevent constipation
ACTIVITY

Exercise of muscles of the lower extremities and abdomen, water aerobics at hip level (not swimming, as it causes polyuria), and postural training, in combination with drug therapy, are useful. Inpatient evaluation and tailoring of therapy are often important, However, if patients are restricted to bed rest, their functional mobility can decrease rapidly. Therefore, extensive physical therapy is strongly encouraged. [Reference: www.emedicine.medscape.com]