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Multiple System Atrophy

Author: RxPG, Posted on Thursday, June 12 @ 11:13:33 IST by RxPG  

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Medicine

Alternative names

Shy-Drager syndrome; Neurologic orthostatic hypotension; Shy-McGee-Drager syndrome; Parkinson's plus syndrome
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
Stiffness
Difficulty bending arms or legs
Posture difficulties: may be unstable, stooped, or slumped over
Movement difficulties
Loss of balance
Gait (walking pattern) changes
Shuffling
Slow movements
Difficulty beginning to walk or initiating any voluntary movement
Small steps followed by the need to run to maintain balance
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
May occur at rest or at any time
May become severe enough to interfere with activities
May be worse when tired, excited, or stressed
Finger-thumb rubbing (pill rolling tremor): may be present
May occur with any action such as holding a cup or other eating utensils
Changes in facial expression
Reduced ability to show facial expressions
"Mask" appearance to face
Staring
May be unable to close mouth
Voice and/or speech changes
Slow speaking
Voice is low-volume
Monotone
Difficulty speaking
Difficulty chewing or swallowing (occasionally)
Loss of fine motor skills
Difficulty writing: may be small and illegible
Difficulty eating
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:
Shows abnormal reflexes
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

Involuntary movements
Nausea/vomiting
Dizziness
Changes in alertness/behavior/mood
Severe confusion or disorientation
Delusional behavior
Hallucinations
Loss of mental functioning



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