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1Department of Neurology, Fujita Health University, School of Medicine, Toyoake, Japan
2Department of Neurology, Fujita Health University Okazaki Medical Center, Okazaki, Japan
3Department of Neurology, Fujita Health University Bantane Hospital, Nagoya, Japan
Copyright © 2023 The Korean Movement Disorder Society
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Conflicts of Interest
The authors have no financial conflicts of interest.
Funding Statement
This work was supported by Grants-in-Aid from the Research Committee of Ataxia, Health Labour Sciences Research Grant, the Ministry of Health, Labour and Welfare, Japan (20317603).
Author contributions
Conceptualization: Hirohisa Watanabe. Data curation: Yasuaki Mizutani, Mizuki Ito. Formal analysis: Hirohisa Watanabe. Funding acquisition: Hirohisa Watanabe. Investigation: Hirohisa Watanabe. Methodology: Hirohisa Watanabe. Supervision: Akihiro Ueda. Validation: Sayuri Shima. Visualization: Hirohisa Watanabe. Writing—original draft: Hirohisa Watanabe. Writing—review & editing: Sayuri Shima, Yasuaki Mizutani, Akihiro Ueda, Mizuki Ito.
MDS criteria for the diagnosis of MSA | Second consensus statement on the diagnosis of MSA | ||||
---|---|---|---|---|---|
Supportive motor features | Additional features of possible MSA | ||||
1. Rapid progression within 3 years of motor onset | For possible MSA-P or MSA-C | ||||
2. Moderate to severe postural instability within 3 years of motor onset | 1. Babinski sign with hyperreflexia | ||||
3. Craniocervical dystonia induced or exacerbated by L-dopa in the absence of limb dyskinesia | 2. Stridor | ||||
4. Severe speech impairment within 3 years of motor onset | For possible MSA-P | ||||
5. Severe dysphagia within 3 years of motor onset | 1. Rapidly progressive parkinsonism | ||||
6. Unexplained Babinski sign | 2. Poor response to levodopa | ||||
7. Jerky myoclonic postural or kinetic tremor | 3. Postural instability within 3 years of motor onset | ||||
8. Postural deformities | 4. Gait ataxia, cerebellar dysarthria, limb ataxia, or cerebellar oculomotor dysfunction | ||||
Supportive nonmotor features | 5. Dysphagia within 5 years of motor onset | ||||
1. Stridor | 6. Atrophy on MRI of putamen, middle cerebellar peduncle, pons, or cerebellum | ||||
2. Inspiratory sighs | 7. Hypometabolism on FDG-PET in putamen, brainstem, or cerebellum* | ||||
3. Cold, discolored hands and feet | For possible MSA-C | ||||
4. Erectile dysfunction (below 60 years of age for clinically probable MSA) | 1. Parkinsonism (bradykinesia and rigidity) | ||||
5. Pathologic laughter or crying | 2. Atrophy on MRI of the putamen, middle cerebellar peduncle, or pons | ||||
MRI markers of clinically established MSA | 3. Hypometabolism on FDG-PET in the putamen* | ||||
For MSA-P | 4. Presynaptic nigrostriatal dopaminergic denervation on SPECT or PET* | ||||
1. Atrophy of: | Supportive features | ||||
● Putamen (and a signal decrease on iron-sensitive sequences) | 1. Orofacial dystonia | ||||
● Middle cerebellar peduncle | 2. Disproportionate antecollis | ||||
● Pons | 3. Camptocormia and/or Pisa syndrome | ||||
● Cerebellum | 4. Contractures of hands or feet | ||||
2. “Hot cross bun” sign | 5. Inspiratory sighs | ||||
3. Increased diffusivity of: | 6. Severe dysphonia | ||||
● Putamen | 7. Severe dysarthria | ||||
● Middle cerebellar peduncle | 8. New or increased snoring* | ||||
For MSA-C | 9. Cold hands and feet | ||||
1. Atrophy of: | 10. Pathologic laughter or crying | ||||
● Putamen (and a signal decrease on iron-sensitive sequences) | 11. Jerky, myoclonic postural/action tremor | ||||
● Infratentorial structures (pons and middle cerebellar peduncle) | Nonsupporting features | ||||
“Hot cross bun” sign | 1. Classic pill-rolling rest tremor* | ||||
2. Increased diffusivity of: | 2. Clinically significant neuropathy* | ||||
● Putamen | 3. Hallucinations not induced by drugs | ||||
Exclusion criteria | 4. Onset after age 75 yr* | ||||
1. Substantial and persistent beneficial response to dopaminergic medications | 5. Family history of ataxia or parkinonsinsm | ||||
2. Unexplained anosmia on olfactory testing | 6. Dementia (on DSM-IV) | ||||
3. Fluctuating cognition with pronounced variation in attention and alertness and early decline in visuoperceptual abilities | 7. White matter lesions suggesting multiple sclerossis | ||||
4. Recurrent visual hallucinations not induced by drugs within 3 years of disease onset | |||||
5. Dementia according to DSM-V within 3 years of disease onset | |||||
6. Downgaze supranuclear palsy or slowing of vertical saccades | |||||
7. Brain MRI findings suggestive of an alternative diagnosis (e.g., PSP, multiple sclerosis, vascular parkinsonism, symptomatic cerebellar disease, etc.) | |||||
8. Documentation of an alternative condition (conditions mimicking MSA, including genetic or symptomatic ataxia and parkinsonism) known to produce autonomic failure, ataxia, or parkinsonism and plausibly connected to the patient’s symptoms |
* not included in the new diagnostic criteria.
MDS, Movement Disorder Society; MSA, multiple system atrophy; MSA-P, MSA-parkinsonian type; MSAC, MSA-cerebellar type; DSM, Diagnostic & Statistical Manual of Mental Disorders; PSP, progressive supranuclear palsy; FDG, [18F]Fluorodeoxyglucose; PET, positron emission tomography; SPECT, single photon emission computed tomography.