Multiple System Atrophy Treatment & Management

Updated: Oct 17, 2018
  • Author: André Diedrich, MD, PhD; Chief Editor: Selim R Benbadis, MD  more...
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Treatment

Approach Considerations

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

Nonpharmacologic treatment

See the list below:

  • 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. (See Table 9.)

Surgical care

An atrial pacemaker may be used in patients with profound bradycardia in addition to orthostatic hypotension as a means of preventing the hypotension. However, this treatment is rarely undertaken and is rarely helpful.

Consider tracheostomy with the utmost care for intermittent respiratory stridor. Cricopharyngeal myotomy or gastrostomy has been used in patients with severe dysphagia, but its value is uncertain.

Consultations

Physical therapists, occupational therapists, speech therapists, 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 bedrest, their functional mobility can decrease rapidly. Therefore, initiate physical therapy if the patient must remain in the hospital for longer than 2 days.

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Nonpharmacologic Treatment of Hypotension and Hypertension

Orthostatic hypotension

The earliest symptom that brings patients to medical attention usually is orthostatic hypotension. Orthostatic hypotension leads to curtailing of physical activity, with all of the problems of deconditioning that consequently occur. Without an adequate upright BP, keeping patients active and on an exercise regimen is extremely difficult; therefore, management of orthostatic hypotension is one of the major tasks in the treatment of patients with MSA.

Mechanical maneuvers, such as leg-crossing, squatting, abdominal compression, bending forward, and placing 1 foot on a chair, can be effective in preventing episodes of orthostatic hypotension. Wearing an external support garment that comes to the waist improves venous return and preload to the heart during standing but loses effectiveness if the patient also wears it while supine. Increased salt and fluid intake and tilted sleeping with the head elevated increase the circulatory plasma volume.

Postprandial hypotension

Small, frequent meals attenuate BP drop after eating. Intake of water half an hour before meals or drinking coffee can counteract postprandial hypotension.

Supine hypertension

The management of patients with orthostatic hypotension and supine hypertension can be challenging, but adequate BP control is often achieved with the following treatment strategy:

  • Use of over-the-counter medication with pressor effects

  • Avoidance of fluid intake at bedtime

  • Not using elastic stockings when supine

  • Not using pressor agents before bedtime

  • Raising the head of the bed 6-9 inches

  • Resting on a semirecumbent chair with feet on the floor during the day

  • Snacking before bedtime

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