Consensus Criteria of Gilman et al for Multiple System Atrophy (MSA)

 

Clinical domains:

(1) autonomic and urinary dysfunction

(2) Parkinsonism

(3) cerebellar dysfunction

(4) corticospinal dysfunction

 

Autonomic or urinary dysfunction - at least one of the following:

(1) orthostatic hypotension

(1a) as feature: drop in systolic >= 20 mm Hg OR in diastolic = 10 mm Hg

(1b) as criterion: drop in systolic >= 30 mm Hg OR in diastolic = 15 mm Hg

(2) urinary incontinence (involuntary partial or complete bladder emptying and/or erectile dysfunction in male)

 

Parkinsonism - both of the following:

(1) bradykinesia (slowness of voluntary movement with progressive decrease in speed and amplitude during repetitive actions)

(2) one or more of the following:

(2a) rigidity

(2b) postural instability (not due to primary visual, vestibular, cerebellar or proprioceptive dysfunction)

(2c) tremor (resting and/or postural)

 

Cerebellar dysfunction - both of the following:

(1) gait ataxia

(2) one or more of the following:

(2a) ataxic dysarthria

(2b) limb ataxia

(2c) sustained gaze-evoked nystagmus

 

Corticospinal dysfunction:

(1) extensor plantar response with hyper-reflexia

Number of Clinical Domains Met	Additional Findings	Diagnostic Category
1	poor response to levodopa and a feature from another domain	possible
1	at least 2 features from 2 other domains	possible
autonomic failure or urinary dysfunction	poor response to levodopa	probable
autonomic failure or urinary dysfunction	cerebellar dysfunction	probable
	pathologic features present	definite

 

where:

• Pathologic features = presence of a high density of glial cytoplasmic inclusions AND degeneration in the nigrostriatal and olivopontocerebellar pathways. If a brain exam fails to find these then I would assume the diagnosis is excluded.

• In the note to corticospinal tract dysfunction it says that it is not used in defining the diagnosis of MSA, which implies that it cannot be used as a clinical domain for the "possible" category. It might be useable as an additional feature for the "possible" category.