Gilman et al developed criteria for multiple system atrophy (MSA) developed at a consensus conference. Participants were from multiple institutions in North America and Europe.
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.
Specialty: Neurology