Multiple system atrophy

DEFINITION

Multiple system atrophy (MSA) is a rare neurological disorder that impairs your body’s involuntary (autonomic) functions, including blood pressure, heart rate, bladder function and digestion.

Formerly called Shy-Drager syndrome, the condition shares many Parkinson’s disease-like symptoms, such as slowness of movement, muscle rigidity and poor balance.

Multiple system atrophy is a degenerative disease that develops in adulthood, usually in the 50s or 60s.

Treatment for MSA includes medications and lifestyle changes to help manage symptoms. The condition progresses gradually and eventually leads to death.

CAUSES

There’s no known cause for brain changes in multiple system atrophy (MSA). Some researchers are studying whether there’s an inherited component or environmental toxin involved in the disease process, but there’s no substantial evidence to support these theories.

MSA is associated with deterioration and shrinkage (atrophy) of portions of your brain (cerebellum, basal ganglia and brainstem) that regulate internal body functions, digestion and motor control.

Evaluation under a microscope of damaged brain tissue of people with MSA reveals nerve cells (neurons) that contain an abnormal amount of a protein called alpha-synuclein. Some research suggests that this protein may be overexpressed in multiple system atrophy.

SYMPTOMS

Multiple system atrophy (MSA) is so named because its signs and symptoms affect multiple parts of your body. Previously called Shy-Drager syndrome, MSA is classified by two types: parkinsonian and cerebellar, depending on which types of symptoms predominate at the time of evaluation.

Parkinsonian type

Predominant signs and symptoms are those of Parkinson’s disease, such as:

  • Rigid muscles and difficulty bending your arms and legs
  • Slow movement (bradykinesia)
  • Tremors (rare in MSA compared with classic Parkinson’s disease)
  • Impaired posture and balance


Cerebellar type

Predominant signs and symptoms are lack of muscle coordination (ataxia). Signs and symptoms may include:

  • Impairment of movement and coordination, such as unsteady gait and loss of balance
  • Slurred, slow or low-volume speech (dysarthria)
  • Visual disturbances, such as blurred or double vision and difficulty focusing your eyes
  • Difficulty swallowing (dysphagia) or chewing


General signs and symptoms

In addition, the primary sign of multiple system atrophy is:

  • Postural (orthostatic) hypotension, a form of low blood pressure that makes you feel dizzy or lightheaded, or even faint, when you stand up from sitting or lying down.
  • You also can develop dangerously high blood pressure levels while lying down.
  • People with multiple system atrophy may have other difficulties with body functions that occur involuntarily (autonomic), including:


Urinary and bowel dysfunction

Constipation

Loss of bladder or bowel control (incontinence)

Sweating abnormalities

A reduction in the production of perspiration, tears and saliva

Impaired control of body temperature, often causing cold hands or feet as well as heat intolerance due to impaired sweating

Sleep disorders

Agitated sleep due to “acting out” one’s dreams

Abnormal breathing at night

Sexual dysfunction

Inability to achieve or maintain an erection (impotence)

Loss of libido

Cardiovascular problems

Irregular heartbeat

Psychiatric problems

Difficulty controlling emotions

When to see a doctor

If you develop any of the signs and symptoms associated with multiple system atrophy, see your doctor for an evaluation and diagnosis. If you’ve already been diagnosed with the condition, contact your doctor if new symptoms occur or if existing symptoms worsen.