Parkinson’s plus syndrome

Alternative names
Shy-Drager syndrome; Neurologic orthostatic hypotension; Shy-McGee-Drager syndrome; Multiple system atrophy

Definition

Multiple system atrophy is a degenerative disorder characterized by the following:

     
  • Progressive damage to the autonomic nervous system (the portion of the nervous system that controls involuntary functions such as blood pressure, heart rate, digestion, and sexual function)  
  • Muscle tremor and rigidity  
  • Slow movement

Causes, incidence, and risk factors

Multiple system atrophy (MSA) is a rare degenerative condition. Its symptoms are similar to those of Parkinson’s disease in that patients may move slowly, be tremulous, and have a shuffling gait.

However, in MSA there is more widespread neurologic damage and damage to the autonomic nervous system (the portion of the nervous system that controls involuntary functions). There is progressive degeneration of the nervous system, with damage to all parts of the nervous system, including the brain.

The cause is unknown. MSA develops gradually and is most often diagnosed in men older than 60.

Symptoms

     
  • Dizziness or fainting when arising or after standing still  
  • Impotence  
  • Loss of sweating of any part of the body  
  • Loss of control of bowels or bladder  
  • Vision changes, decreased or blurred vision  
  • Muscle rigidity       o Stiffness       o Difficulty bending arms or legs  
  • Posture difficulties: may be unstable, stooped, or slumped over  
  • Movement difficulties       o Loss of balance       o Gait (walking pattern) changes       o Shuffling  
  • Slow movements       o Difficulty beginning to walk or initiating any voluntary movement       o Small steps followed by the need to run to maintain balance       o Freezing of movement when the movement is stopped, unable to resume movement  
  • Muscle aches and pains (myalgia)  
  • Tremors: may be present in varying degrees or may not be present       o May occur at rest or at any time       o May become severe enough to interfere with activities       o May be worse when tired, excited, or stressed       o Finger-thumb rubbing (pill rolling tremor): may be present       o May occur with any action such as holding a cup or other eating utensils  
  • Changes in facial expression       o Reduced ability to show facial expressions       o “Mask” appearance to face       o Staring       o May be unable to close mouth  
  • Voice and/or speech changes       o Slow speaking       o Voice is low-volume       o Monotone       o Difficulty speaking  
  • Difficulty chewing or swallowing (occasionally)  
  • Loss of fine motor skills       o Difficulty writing: may be small and illegible       o Difficulty eating       o Difficulty with any activity that requires small movements  
  • Frequent falls  
  • Mild decline in intellectual function (may occur)

Additional symptoms that may be associated with this disease:

     
  • Depression  
  • Confusion  
  • Dementia  
  • Seborrheic dermatitis  
  • Breathing difficulties due to vocal cord paralysis

Signs and tests

The health care provider may perform the following:

     
  • Eye examination: may reveal atrophy of the iris and paralysis of eye muscles.  
  • Neuromuscular examination:       o Shows abnormal reflexes       o May show severe muscle wasting (atrophy)  
  • Blood pressure measurement: may reveal postural hypotension (drop in blood pressure associated with change in position)

Parkinsonian movements (tremor, rigidity, and slow movements similar to Parkinson’s disease) are common.

There are no specific tests to confirm this disease. A neurologist can make the diagnosis based on the history of symptoms, the findings during physical examination, and ruling out other causes of symptoms.

Testing to help confirm the diagnosis may include the following:

     
  • Plasma norepinephrine levels  
  • Urine examination for norepinephrine breakdown products (urine catecholamines)  
  • MRI of head to rule out other pathology. There are no specific abnormalities on imaging associated with MSA

Treatment

There is no cure for MSA, and there is no known means to slow progression. Treatment is aimed at controlling symptoms such as postural hypotension and parkinsonian movements.

Anticholinergic medications may be used to reduce early or mild tremors. Levodopa may improve movement and balance.

Carbidopa may reduce the side effects of Levodopa and make it work better. However, the response to medications may be disappointing. Many affected individuals respond poorly to treatment with anticholinergics or Levodopa.

Medications that may be used to treat Low Blood pressure include the following:

     
  • Sympathomimetics  
  • Vasoconstrictors  
  • Beta-blockers  
  • MAO inhibitors  
  • Vasopressin  
  • 9-fluohydrocortisone

A pacemaker programmed to stimulate the heart to beat at a rapid rate (faster than 100 beats per minute) may increase blood pressure for some people.

Expectations (prognosis)

The probable outcome is poor. There is a progressive loss of mental and physical functions until general debilitation develops. Early death is likely.

Rate of progression differs in every case and speed of decline may vary widely in individual patients.

Complications

     
  • Progressive loss of ability to walk or care for self  
  • Difficulty performing daily activities  
  • Injuries from falls/fainting  
  • Side effects of medications

Calling your health care provider

Call your health care provider if you develop symptoms of this disorder.

Call your health care provider if you have been diagnosed with MSA and your symptoms recur, worsen, or progress. Also call if new symptoms appear, including possible side effects of medications:

     
  • Involuntary movements  
  • nausea/Vomiting  
  • Dizziness  
  • Changes in alertness/behavior/mood  
  • Severe confusion or disorientation  
  • Delusional behavior  
  • Hallucinations  
  • Loss of mental functioning

See also the specific medication for possible side effects.

Consult with your health care provider if you have a family member with this disorder and his or her condition deteriorates to the point that you are unable to care for the person at home.

Johns Hopkins patient information

Last revised: December 4, 2012
by Janet G. Derge, M.D.

Medical Encyclopedia

  A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z | 0-9

All ArmMed Media material is provided for information only and is neither advice nor a substitute for proper medical care. Consult a qualified healthcare professional who understands your particular history for individual concerns.