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Multiple system atrophy, parkinsonian type

MedGen UID:
1842393
•Concept ID:
C5554235
•
Disease or Syndrome
Synonyms: MSA, parkinsonian type; MSA-p; multiple system atrophy, parkinsonian type
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0020352
Orphanet: ORPHA98933

Definition

Multiple system atrophy, parkinsonian type (MSA-p) is a form of multiple system atrophy (MSA; see this term) with predominant parkinsonian features (bradykinesia, rigidity, irregular jerky postural tremor, and postural instability). [from ORDO]

Professional guidelines

PubMed

Neuroimaging in Parkinsonism: a study with magnetic resonance and spectroscopy as tools in the differential diagnosis.
Vasconcellos LF, Novis SA, Moreira DM, Rosso AL, Leite AC
Arq Neuropsiquiatr 2009 Mar;67(1):1-6. doi: 10.1590/s0004-282x2009000100002. PMID: 19330200

Recent clinical studies

Etiology

Quantitative brain stem assessment in discriminating neurodegenerative disorders from normal pressure hydrocephalus.
Georgiopoulos C, Papadimitriou S, Nyholm D, Kilander L, Löwenmark M, Fällmar D, Virhammar J
J Neuroimaging 2024 Sep-Oct;34(5):612-618. Epub 2024 Apr 26 doi: 10.1111/jon.13204. PMID: 38676300
The Vocal Flutter of Multiple System Atrophy: A Parkinsonian-Type Phenomenon?
Mir MJ, Herndon NE, Wagle Shukla A, Wheeler-Hegland K, McFarland NR
Mov Disord Clin Pract 2024 Apr;11(4):403-410. Epub 2024 Feb 5 doi: 10.1002/mdc3.13988. PMID: 38314679Free PMC Article
Functional connectome automatically differentiates multiple system atrophy (parkinsonian type) from idiopathic Parkinson's disease at early stages.
Chen B, Cui W, Wang S, Sun A, Yu H, Liu Y, He J, Fan G
Hum Brain Mapp 2023 Apr 15;44(6):2176-2190. Epub 2023 Jan 20 doi: 10.1002/hbm.26201. PMID: 36661217Free PMC Article
Dopamine transporter SPECT imaging in Parkinson's disease and atypical Parkinsonism: a study of 137 patients.
Constantinides VC, Souvatzoglou M, Paraskevas GP, Chalioti M, Stefanis L, Kapaki E
Neurol Sci 2023 May;44(5):1613-1623. Epub 2023 Jan 20 doi: 10.1007/s10072-023-06628-9. PMID: 36658411
Diagnostic performance of T2* gradient echo, susceptibility-weighted imaging, and quantitative susceptibility mapping for patients with multiple system atrophy-parkinsonian type: a systematic review and meta-analysis.
Lim SJ, Suh CH, Shim WH, Kim SJ
Eur Radiol 2022 Jan;32(1):308-318. Epub 2021 Jul 16 doi: 10.1007/s00330-021-08174-4. PMID: 34272590

Diagnosis

Quantitative brain stem assessment in discriminating neurodegenerative disorders from normal pressure hydrocephalus.
Georgiopoulos C, Papadimitriou S, Nyholm D, Kilander L, Löwenmark M, Fällmar D, Virhammar J
J Neuroimaging 2024 Sep-Oct;34(5):612-618. Epub 2024 Apr 26 doi: 10.1111/jon.13204. PMID: 38676300
The Vocal Flutter of Multiple System Atrophy: A Parkinsonian-Type Phenomenon?
Mir MJ, Herndon NE, Wagle Shukla A, Wheeler-Hegland K, McFarland NR
Mov Disord Clin Pract 2024 Apr;11(4):403-410. Epub 2024 Feb 5 doi: 10.1002/mdc3.13988. PMID: 38314679Free PMC Article
Transcranial magnetic stimulation neurophysiology in patients with non-Alzheimer's neurodegenerative diseases: A systematic review and meta-analysis.
Mimura Y, Tobari Y, Nakahara K, Nakajima S, Yoshida K, Mimura M, Noda Y
Neurosci Biobehav Rev 2023 Dec;155:105451. Epub 2023 Nov 4 doi: 10.1016/j.neubiorev.2023.105451. PMID: 37926239
Functional connectome automatically differentiates multiple system atrophy (parkinsonian type) from idiopathic Parkinson's disease at early stages.
Chen B, Cui W, Wang S, Sun A, Yu H, Liu Y, He J, Fan G
Hum Brain Mapp 2023 Apr 15;44(6):2176-2190. Epub 2023 Jan 20 doi: 10.1002/hbm.26201. PMID: 36661217Free PMC Article
Dopamine transporter SPECT imaging in Parkinson's disease and atypical Parkinsonism: a study of 137 patients.
Constantinides VC, Souvatzoglou M, Paraskevas GP, Chalioti M, Stefanis L, Kapaki E
Neurol Sci 2023 May;44(5):1613-1623. Epub 2023 Jan 20 doi: 10.1007/s10072-023-06628-9. PMID: 36658411

Therapy

The recovery of sympathetic skin responses with levodopa in a patient with multiple system atrophy-parkinsonian type.
Onder H, Yurtdas M, Comoglu S
Neurocase 2024 Aug;30(3):121-123. Epub 2024 Jul 29 doi: 10.1080/13554794.2024.2381855. PMID: 39072635
Functional connectome automatically differentiates multiple system atrophy (parkinsonian type) from idiopathic Parkinson's disease at early stages.
Chen B, Cui W, Wang S, Sun A, Yu H, Liu Y, He J, Fan G
Hum Brain Mapp 2023 Apr 15;44(6):2176-2190. Epub 2023 Jan 20 doi: 10.1002/hbm.26201. PMID: 36661217Free PMC Article
Stacked Model-Based Classification of Parkinson's Disease Patients Using Imaging Biomarker Data.
Hathaliya J, Modi H, Gupta R, Tanwar S, Alqahtani F, Elghatwary M, Neagu BC, Raboaca MS
Biosensors (Basel) 2022 Jul 29;12(8) doi: 10.3390/bios12080579. PMID: 36004975Free PMC Article
Diagnostic performance of T2* gradient echo, susceptibility-weighted imaging, and quantitative susceptibility mapping for patients with multiple system atrophy-parkinsonian type: a systematic review and meta-analysis.
Lim SJ, Suh CH, Shim WH, Kim SJ
Eur Radiol 2022 Jan;32(1):308-318. Epub 2021 Jul 16 doi: 10.1007/s00330-021-08174-4. PMID: 34272590
Post-mortem assessment of the short and long-term effects of the trophic factor neurturin in patients with α-synucleinopathies.
Bartus RT, Kordower JH, Johnson EM Jr, Brown L, Kruegel BR, Chu Y, Baumann TL, Lang AE, Olanow CW, Herzog CD
Neurobiol Dis 2015 Jun;78:162-71. Epub 2015 Apr 2 doi: 10.1016/j.nbd.2015.03.023. PMID: 25841760

Prognosis

Additional Role of Midbrain F-18 FP-CIT Uptake on PET in Evaluation of Essential Tremor and Parkinsonism.
Lee H, Sung YH, Hwang KH
Curr Med Imaging 2023;19(9):1041-1051. doi: 10.2174/1573405619666230124142725. PMID: 36734891
Stacked Model-Based Classification of Parkinson's Disease Patients Using Imaging Biomarker Data.
Hathaliya J, Modi H, Gupta R, Tanwar S, Alqahtani F, Elghatwary M, Neagu BC, Raboaca MS
Biosensors (Basel) 2022 Jul 29;12(8) doi: 10.3390/bios12080579. PMID: 36004975Free PMC Article
Postganglionic Sudomotor Assessment in Early Stage of Multiple System Atrophy and Parkinson Disease: A Morpho-functional Study.
Provitera V, Iodice V, Manganelli F, Mozzillo S, Caporaso G, Stancanelli A, Borreca I, Esposito M, Dubbioso R, Iodice R, Vitale F, Koay S, Vichayanrat E, Valerio F, Santoro L, Nolano M
Neurology 2022 Mar 22;98(12):e1282-e1291. Epub 2022 Jan 11 doi: 10.1212/WNL.0000000000013300. PMID: 35017309Free PMC Article
Non-ergot dopamine agonist rotigotine as a promising therapeutic tool in atypical parkinsonism syndromes: a 24 months pilot observational open-label study.
Moretti DV, Binetti G, Zanetti O, Frisoni GB
Neuropharmacology 2014 Oct;85:284-9. Epub 2014 Jun 7 doi: 10.1016/j.neuropharm.2014.05.028. PMID: 24915072
Comparison of cerebral glucose metabolism between multiple system atrophy Parkinsonian type and Parkinson's disease.
Feng T, Wang Y, Ouyang Q, Duan Z, Li W, Lu L, Xiang W
Neurol Res 2008 May;30(4):377-82. doi: 10.1179/174313208X300396. PMID: 18544255

Clinical prediction guides

The Vocal Flutter of Multiple System Atrophy: A Parkinsonian-Type Phenomenon?
Mir MJ, Herndon NE, Wagle Shukla A, Wheeler-Hegland K, McFarland NR
Mov Disord Clin Pract 2024 Apr;11(4):403-410. Epub 2024 Feb 5 doi: 10.1002/mdc3.13988. PMID: 38314679Free PMC Article
Stacked Model-Based Classification of Parkinson's Disease Patients Using Imaging Biomarker Data.
Hathaliya J, Modi H, Gupta R, Tanwar S, Alqahtani F, Elghatwary M, Neagu BC, Raboaca MS
Biosensors (Basel) 2022 Jul 29;12(8) doi: 10.3390/bios12080579. PMID: 36004975Free PMC Article
Thalamic and cerebellar hypoperfusion in single photon emission computed tomography may differentiate multiple system atrophy and progressive supranuclear palsy.
Alster P, Nieciecki M, Koziorowski DM, Cacko A, Charzyńska I, Królicki L, Friedman A
Medicine (Baltimore) 2019 Jul;98(30):e16603. doi: 10.1097/MD.0000000000016603. PMID: 31348305Free PMC Article
3-Hz postural tremor in multiple system atrophy cerebellar type (MSA-C)-a static posturography study.
Li X, Wang Y, Wang Z, Xu Y, Zheng W
Neurol Sci 2018 Jan;39(1):71-77. Epub 2017 Oct 12 doi: 10.1007/s10072-017-3130-3. PMID: 29027029
Comparison of cerebral glucose metabolism between multiple system atrophy Parkinsonian type and Parkinson's disease.
Feng T, Wang Y, Ouyang Q, Duan Z, Li W, Lu L, Xiang W
Neurol Res 2008 May;30(4):377-82. doi: 10.1179/174313208X300396. PMID: 18544255

Recent systematic reviews

Transcranial magnetic stimulation neurophysiology in patients with non-Alzheimer's neurodegenerative diseases: A systematic review and meta-analysis.
Mimura Y, Tobari Y, Nakahara K, Nakajima S, Yoshida K, Mimura M, Noda Y
Neurosci Biobehav Rev 2023 Dec;155:105451. Epub 2023 Nov 4 doi: 10.1016/j.neubiorev.2023.105451. PMID: 37926239
Diagnostic performance of T2* gradient echo, susceptibility-weighted imaging, and quantitative susceptibility mapping for patients with multiple system atrophy-parkinsonian type: a systematic review and meta-analysis.
Lim SJ, Suh CH, Shim WH, Kim SJ
Eur Radiol 2022 Jan;32(1):308-318. Epub 2021 Jul 16 doi: 10.1007/s00330-021-08174-4. PMID: 34272590

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