Table 1

Multiple system atrophy: diagnostic criteria

SND type (predominantly parkinsonism)OPCA type (predominantly cerebellar)
Sporadic adult onset non or poorly levodopa responsive parkinsonismPossibleSporadic adult onset cerebellar syndrome with parkinsonism
Above,1-150 plus severe symptomatic autonomic failure1-152 or cerebellar
signs or pyramidal signs or pathological sphincter EMG
ProbableSporadic adult onset cerebellar syndrome1-151 (with or without parkinsonism  or pyramidal signs), plus severe symptomatic autonomic failure1-152 or  pathological sphincter EMG
Postmortem confirmedDefinitePostmortem confirmed
  • 1-150 Moderate or good, but often waning, response to levodopa may occur, in which case multiple atypical features need to be present.

  • 1-151 Without DSM III-R dementia, generalised tendon areflexia, prominent supranuclear palsy for downgaze or other identifiable cause.

  • 1-152 Postural syncope or marked urinary incontinence or retention not due to other causes.

  • Sporadic = no other case of MSA among first or second degree relatives. Adult onset = onset age ⩾30 y.

  • Modified from Quinn,15 with permission of the BMJ Publishing Group.