What is Multiple System Atrophy (Shy Drager Syndrome)

Multiple System Atrophy (MSA) is an adult-onset disease with features of parkinsonism, autonomic dysfunction, urinary dysfunction and cerebellar ataxia. There is progressive loss of neuronal and oligodendroglial cells in the central nervous system.
MSA is also known as Shy-Drager syndrome (when autonomic dysfunction is the predominant feature), striatonigral degeneration (Parkinsonsim predominates) and olivo-ponto-cerebellar atrophy (cerebekllar features predominate).
The diagnosis of MSA can be very difficult as it can be clinically very similar to other disorders such as Parkinson’s disease and pure autonomic failure; the diagnosis is confirmed only on autopsy.

Statistics on Multiple System Atrophy (Shy Drager Syndrome)

The prevalence of MSA is 2-15 per 100,000, however, this is most likely an underestimation as due to the difficultly of clinically differentiating MSA from other disorders, many patients with MSA are not correctly diagnosed during their lifetime.
The onset is usually in middle-age, with men and women being equally affected.

Risk Factors for Multiple System Atrophy (Shy Drager Syndrome)

The cause of MSA is unknown and there is no evidence that MSA runs in families.

Progression of Multiple System Atrophy (Shy Drager Syndrome)

Multiple system atrophy (MSA) with postural hypotension, also called Shy-Drager syndrome, is a progressive disorder of the central and sympathetic nervous systems.
The disorder is characterized by postural hypotension – an excessive drop in blood pressure when the patient stands up, which causes dizziness or momentary blackouts.
MSA has been classified clinically into three types, the first of which primarily affects balance, coordination, and speech; a form which can resemble Parkinson’s disease because of slow movement and stiff muscles; and a mixed cerebellar and parkinsonian form. In all three forms of MSA, the patient can experience postural hypotension.

How is Multiple System Atrophy (Shy Drager Syndrome) Diagnosed?

The diagnosis of MSA is based mainly on the clinical features. Tests done can include an MRI or CT scan of the brain, as well as other more specialised tests.

Prognosis of Multiple System Atrophy (Shy Drager Syndrome)

Prognosis is poor and on average patients will survive about 6 years after the inital presentation of MSA. There is no treatment currently available that will cure or slow the rate of progression of MSA. Cause of death is often related to breathing problems or heart problems.

How is Multiple System Atrophy (Shy Drager Syndrome) Treated?

Currently there is no treatment that cures or slows the rate of progression of MSA, treatment is aiming at controlling symtpoms. A multidisiplinary approach is needed with medical managment, physiotherapy, occupational therapy and speech therapy.
Postural hypotension:
 

  • Mechanical maneuvers such as leg-crossing, squatting and bending forward can prevent episodes of postural hypotension as can waering external support garments.
  • Sleeping with the head of the bed elevated.
  • Small and more frequent meals.
  • Medications are used to treat hypotension.
  • Parkinsonian symptoms:
  • Treated with medication, although this becomes less effective with time.
  • Urinary incontinence:
  • Catheterisation can help to prevent incontinence
  • If the urinary incontinence is due to detrusor, muscle instability anticholinergic agents can be helpful.
  • Impotence:
  • Treated with medications or injections.
  • Constipation:
  • High fibre diet, laxatives and suppositories can help to relieve constipation.

    Multiple System Atrophy (Shy Drager Syndrome) References

    [1] Emedicine, Multiple System Atrophy, 2004
    [2] Hankey G., Wardlaw J. Clinical Neurology. Demos Medical Publishing, United Kingdom, 2002.
    [3] Kumar and Clarke, Clinical Medicine, 2002 pg 1186