Multiple System Atrophy for clinicians

Multiple System Atrophy (MSA) is a rapidly degenerative neurological disorder also known as Oliviopontocerebellar Atrophy or Shy-Drager Syndrome.

Symptoms

Symptoms may also include:

  • autonomic difficulties - postural hypotension (causing severe trauma from falls from fainting), bladder problems including frequent UTIs, chronic constipation, erectile dysfunction, cold hands/feet and difficulties controlling sweating
  • mobility difficulties - slow movements, stiff muscles, poor balance and coordination
  • swallowing difficulties - dysphagia, impulsive feeding, excess saliva/drooling, needing PEG Feeding (late stages)
  • communication difficulties - speech problems, voice difficulties.
  • cognition/mood difficulties - apathy, depression, anxiety, irritability, emotional lability, executive dysfunction, slowness of thought
  • sleep disturbances - insomnia, REM sleep behaviour disorder, excessive daytime sleepiness, restless legs syndrome, nocturnal stridor (needing CPAP, tracheostomy in severe cases).

Prevalence

  • The prevalence of MSA is 4.4 per 100,000
  • average age of onset is around 55
  • average life expectancy is seven to 10 years from onset of symptoms.

Cause

  • The underlying cause of MSA is not known
  • collections of an abnormal protein in the brain, called alpha-synuclein, cause damage to cells
  • regions of the brain that control movement and autonomic functions are affected.

Diagnosis

MSA is difficult to diagnose.

Patients with MSA present with parkinsonian symptoms of slowness of movement, difficulty initiating movement, and rigidity with or without resting tremor.

Patients with MSA differ from Parkinson’s Disease with rapid progression of their disorder, absent/poor/waning response to levodopa, early instability/falls, early dysphagia and/or dysarthria.

Cures or treatments

Possible cures for MSA are being researched. Currently, there are a number of symptomatic therapies available which can help to improve quality of life.

Medications

  • Appropriate medications for symptomatic management continue to evolve and it may be beneficial to consult with a Neurologist and/or Geriatrician
  • high levodopa in some circumstances has limited effect, and may worsen autonomic problems
  • atropine eye drops or atrovent nasal spray administered under the tongue may assist in drying excess secretions/assist with drooling, medications to assist with constipation
  • medications may assist with constipation.

Non-pharmacological treatments

  • Orthostatic hypotension - compression stockings, custom-made elastic body garments, sufficient fluid intake, high-salt diet, spreading of total carbohydrate intake and head-up tilt during sleep
  • constipation - daily movement, sufficient fluid and fibre intake.

Management of symptoms

For a list of management symptoms, see the Multiple System Atrophy fact sheet for GPs and Healthcare Providers (PDF 325KB). Some of these include:

  • allied health care
  • psychology/counselling
  • and a number of specialised clinics.

Further information

For further information, contact:

Speech Pathology
Repatriation General Hospital
Daws Road, Daw Park SA 5041
Telephone: (08) 8275 1861