Overview of multiple system atrophy (MSA) diagnostic criteria

Core motor features		Core autonomic features
Parkinsonism Bradykinesia plus rigidity or tremor Poor levodopa responsiveness	Cerebellar syndrome Gait ataxia Limb ataxia Cerebellar dysarthria Oculomotor dysfunction (sustained nystagmus or saccadic hypermetria)	Voiding dysfunction Unexplained voiding difficulties Postvoid residual volume (>100 mL for c.e.) Secondary causes excluded (eg, bladder outflow obstruction due to prostate enlargement)	Urinary urge incontinence Involuntary urine leakage associated with urgency Non-neurogenic causes excluded (eg, prior pelvic surgery, pelvic floor prolapse)	Neurogenic orthostatic hypotension ≥20 mmHg systolic blood pressure drop within 10 minutes of standing or head-up tilt test (within 3 minutes for c.e.)
Supportive clinical features		Biomarkers
Motor Rapid progression within 3 years of motor onset Moderate to severe postural instability within 3 years of motor onset Craniocervical dystonia induced/exacerbated by levodopa in absence of limb dyskinesia Severe speech impairment within 3 years of motor onset Severe dysphagia within 3 years of motor onset Unexplained Babinski Jerky myoclonic postural or kinetic tremor Postural deformities (eg, anterocollis, laterocollis, camptocormia)	Nonmotor Stridor Inspiratory sighs Cold discolored hands and feet Erectile dysfunction (before age 60 years for c.e.) Pathologic laughter or crying	Brain MRI Atrophy of putamen, MCP, pons, and/or cerebellum Signal decrease of putamen on iron-sensitive sequences "Hot cross bun" sign Increased diffusivity of putamen and/or MCP
Exclusionary features
Substantial and persistent beneficial response to dopaminergic medications Unexplained anosmia on olfactory testing Abnormal cardiac sympathic imaging (¹²³I-MIBG-scintigraphy) Fluctuating cognition with early decline in visuospatial abilities Recurrent visual hallucinations within 3 years of disease onset Dementia within 3 years of disease onset Downgaze supranuclear palsy Brain MRI findings suggestive of alternative diagnosis (eg, PSP, MS, vascular parkinsonism, symptomatic cerebellar disease) Alternative condition known to produce autonomic failure, ataxia, or parkinsonism

Clinically established MSA is defined as a sporadic, progressive adult (>30 years) onset disease with:

Autonomic dysfunction (≥1 feature) and
Poorly levodopa-responsive parkinsonism and/or cerebellar syndrome (≥2 features) and
≥2 supportive clinical features and
≥1 brain MRI feature and
No exclusionary criteria

Clinically probable MSA is defined as a sporadic, progressive adult (>30 years) onset disease with:

At least 2 of:
- Autonomic dysfunction (≥1 feature)
- Parkinsonism
- Cerebellar syndrome (≥1 feature)
and
≥1 supportive clinical feature and
No exclusionary criteria

c.e.: clinically established; MCP: middle cerebellar peduncle; MIBG: metaiodobenzylguanidine; MRI: magnetic resonance imaging; MS: multiple sclerosis; PSP: progressive supranuclear palsy.

Adapted from: Wenning GK, Stankovic I, Vignatelli L, et al. The Movement Disorder Society criteria for the diagnosis of multiple system atrophy. Mov Disord 2022; 37:1131. Copyright © 2022 The Authors. Available at: https://movementdisorders.onlinelibrary.wiley.com/doi/10.1002/mds.29005 (Accessed on March 13, 2023). Reproduced under the terms of the Creative Commons Attribution License 4.0.

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Overview of multiple system atrophy (MSA) diagnostic criteria