Multiple System Atrophy

Summary

Multiple system atrophy, previously known as Shy Drager Syndrome, is a condition characterized by widespread neurodegeneration affecting several systems in the brain and spinal cord. Patients with this condition typically present with autonomic instability, which is characterized by postural instability, syncope, and hypotension. Autonomic instability may also cause incontinence. Finally, patients can also present with parkinsonism.

Key Points

• Multiple System Atrophy
  • Formerly known as Shy-Drager Syndrome
  • Pathophysiology
    • Unclear etiology
    • Neurodegeneration affecting multiple systems in CNS
  • Presentation
    • Autonomic instability
      • postural instability, with syncope and hypotension is classic
      • Impotence and incontinence may be seen
    • Parkinsonism
    • Widespread neurological deficits/neuropathy
    • May be fatal if phrenic nerve or other nerves controlling respiration are affected
  • Treatment
    • Fluids
    • Fludrocortisone
    • Alpha agonists