What are levels of certainty in the diagnosis of multiple system atrophy (MSA)?

Updated: Oct 17, 2018
  • Author: André Diedrich, MD, PhD; Chief Editor: Selim R Benbadis, MD  more...
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Answer

MSA can be ascertained as possible, probable, or definite MSA (see Table 4, below), based on autonomic and urogenital features, on the presence of parkinsonism, and on cerebellar dysfunction, as well as on additional features (see tables 2a and 2b, above).

Only pathologic findings of high density of alpha-synuclein-positive glial cytoplasmic inclusions (GCIs) and degenerative changes in the striatonigral or olivopontocerebellar pathways can definitively confirm the diagnosis of MSA. (See Workup.)

Table 4. Diagnostic Categories of MSA (Open Table in a new window)

Category

Definition

Possible MSA

A sporadic, progressive, adult (>30y) with onset disease* characterized by the following:

  • Parkinsonism or cerebellar syndrome

  • At least 1 feature of autonomic or urogenital dysfunction

  • At least 1 of the additional features from Table 2b

Probable MSA

A sporadic, progressive, adult (>30y) with onset disease* characterized by the following:

  • Autonomic failure involving urinary dysfunction

  • Poorly levodopa-responsive parkinsonism or cerebellar dysfunction

Definitive MSA

A sporadic, progressive, adult (>30y) with onset disease pathologically confirmed by presence of high density GCIs in association with degenerative changes in striatonigral and olivopontocerebellar pathways

*Disease onset is defined as the initial presentation of any parkinsonian or cerebellar motor problems or autonomic features (except erectile dysfunction). 


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