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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Diagnostic Checklist
TERMINOLOGY
Definitions
- Multiple system atrophy (MSA)
- Adult-onset fatal neurodegenerative disease, unknown etiology
- Various combination of cerebellar/pyramidal/extrapyramidal/autonomic features
- 3 clinical subtypes characterized by signs and symptoms
- Predominantly cerebellar (MSA-C)
- a.k.a. sporadic olivopontocerebellar atrophy (sOPCA)
- Extrapyramidal (MSA-P)
- a.k.a. striatonigral degeneration (SND)
- Autonomic (MSA-A)
- a.k.a. Shy-Drager syndrome (SDS)
- Predominantly cerebellar (MSA-C)
- 2 distinct imaging subtypes: MSA-C and MSA-P
- Multiple system atrophy (MSA)
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Chelban V et al: An update on advances in magnetic resonance imaging of multiple system atrophy. J Neurol. 266(4):1036-45, 2019
- Fanciulli A et al: Early distinction of Parkinson-variant multiple system atrophy from Parkinson's disease. Mov Disord. 34(3):440-1, 2019
- Krismer F et al: Morphometric MRI profiles of multiple system atrophy variants and implications for differential diagnosis. Mov Disord. 34(7):1041-8, 2019
- Meissner WG et al: Multiple system atrophy: recent developments and future perspectives. Mov Disord. 34(11):1629-42, 2019
- Miki Y et al: Improving diagnostic accuracy of multiple system atrophy: a clinicopathological study. Brain. 142(9):2813-27, 2019
- Monzio Compagnoni G et al: Understanding the pathogenesis of multiple system atrophy: state of the art and future perspectives. Acta Neuropathol Commun. 7(1):113, 2019
- Booth TC et al: The role of functional dopamine-transporter SPECT imaging in parkinsonian syndromes, part 2. AJNR Am J Neuroradiol. 36(2):236-44, 2015
- Fanciulli A et al: Multiple-system atrophy. N Engl J Med. 372(3):249-63, 2015
- Ramli N et al: Differentiating multiple-system atrophy from Parkinson's disease. Clin Radiol. 70(5):555-64, 2015
- Reiter E et al: Dorsolateral nigral hyperintensity on 3.0T susceptibility-weighted imaging in neurodegenerative Parkinsonism. Mov Disord. 30(8):1068-76, 2015
- Peeraully T: Multiple system atrophy. Semin Neurol. 34(2):174-81, 2014
- Wenning GK et al: Multiple system atrophy. Handb Clin Neurol. 117:229-41, 2013
- Ito S et al: Putaminal hyperintensity on T1-weighted MR imaging in patients with the Parkinson variant of multiple system atrophy. AJNR Am J Neuroradiol. 30(4):689-92, 2009
- Lipp A et al: Prospective differentiation of multiple system atrophy from Parkinson disease, with and without autonomic failure. Arch Neurol. 66(6):742-50, 2009
- Matsusue E et al: Cerebellar lesions in multiple system atrophy: postmortem MR imaging-pathologic correlations. AJNR Am J Neuroradiol. 30(9):1725-30, 2009
- Köllensperger M et al: Red flags for multiple system atrophy. Mov Disord. 23(8):1093-9, 2008
- Minnerop M et al: Voxel-based morphometry and voxel-based relaxometry in multiple system atrophy-a comparison between clinical subtypes and correlations with clinical parameters. Neuroimage. 36(4):1086-95, 2007
- Nicoletti G et al: MR imaging of middle cerebellar peduncle width: differentiation of multiple system atrophy from Parkinson disease. Radiology. 239(3):825-30, 2006
- Mascalchi M et al: Proton MR spectroscopy of the cerebellum and pons in patients with degenerative ataxia. Radiology. 223(2):371-8, 2002
- Naka H et al: Characteristic MRI findings in multiple system atrophy: comparison of the three subtypes. Neuroradiology. 44(3):204-9, 2002
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Diagnostic Checklist
TERMINOLOGY
Definitions
- Multiple system atrophy (MSA)
- Adult-onset fatal neurodegenerative disease, unknown etiology
- Various combination of cerebellar/pyramidal/extrapyramidal/autonomic features
- 3 clinical subtypes characterized by signs and symptoms
- Predominantly cerebellar (MSA-C)
- a.k.a. sporadic olivopontocerebellar atrophy (sOPCA)
- Extrapyramidal (MSA-P)
- a.k.a. striatonigral degeneration (SND)
- Autonomic (MSA-A)
- a.k.a. Shy-Drager syndrome (SDS)
- Predominantly cerebellar (MSA-C)
- 2 distinct imaging subtypes: MSA-C and MSA-P
- Multiple system atrophy (MSA)
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Chelban V et al: An update on advances in magnetic resonance imaging of multiple system atrophy. J Neurol. 266(4):1036-45, 2019
- Fanciulli A et al: Early distinction of Parkinson-variant multiple system atrophy from Parkinson's disease. Mov Disord. 34(3):440-1, 2019
- Krismer F et al: Morphometric MRI profiles of multiple system atrophy variants and implications for differential diagnosis. Mov Disord. 34(7):1041-8, 2019
- Meissner WG et al: Multiple system atrophy: recent developments and future perspectives. Mov Disord. 34(11):1629-42, 2019
- Miki Y et al: Improving diagnostic accuracy of multiple system atrophy: a clinicopathological study. Brain. 142(9):2813-27, 2019
- Monzio Compagnoni G et al: Understanding the pathogenesis of multiple system atrophy: state of the art and future perspectives. Acta Neuropathol Commun. 7(1):113, 2019
- Booth TC et al: The role of functional dopamine-transporter SPECT imaging in parkinsonian syndromes, part 2. AJNR Am J Neuroradiol. 36(2):236-44, 2015
- Fanciulli A et al: Multiple-system atrophy. N Engl J Med. 372(3):249-63, 2015
- Ramli N et al: Differentiating multiple-system atrophy from Parkinson's disease. Clin Radiol. 70(5):555-64, 2015
- Reiter E et al: Dorsolateral nigral hyperintensity on 3.0T susceptibility-weighted imaging in neurodegenerative Parkinsonism. Mov Disord. 30(8):1068-76, 2015
- Peeraully T: Multiple system atrophy. Semin Neurol. 34(2):174-81, 2014
- Wenning GK et al: Multiple system atrophy. Handb Clin Neurol. 117:229-41, 2013
- Ito S et al: Putaminal hyperintensity on T1-weighted MR imaging in patients with the Parkinson variant of multiple system atrophy. AJNR Am J Neuroradiol. 30(4):689-92, 2009
- Lipp A et al: Prospective differentiation of multiple system atrophy from Parkinson disease, with and without autonomic failure. Arch Neurol. 66(6):742-50, 2009
- Matsusue E et al: Cerebellar lesions in multiple system atrophy: postmortem MR imaging-pathologic correlations. AJNR Am J Neuroradiol. 30(9):1725-30, 2009
- Köllensperger M et al: Red flags for multiple system atrophy. Mov Disord. 23(8):1093-9, 2008
- Minnerop M et al: Voxel-based morphometry and voxel-based relaxometry in multiple system atrophy-a comparison between clinical subtypes and correlations with clinical parameters. Neuroimage. 36(4):1086-95, 2007
- Nicoletti G et al: MR imaging of middle cerebellar peduncle width: differentiation of multiple system atrophy from Parkinson disease. Radiology. 239(3):825-30, 2006
- Mascalchi M et al: Proton MR spectroscopy of the cerebellum and pons in patients with degenerative ataxia. Radiology. 223(2):371-8, 2002
- Naka H et al: Characteristic MRI findings in multiple system atrophy: comparison of the three subtypes. Neuroradiology. 44(3):204-9, 2002
