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Brain
Diagnosis
Pathology-Based Diagnoses
Acquired Toxic/Metabolic/Degenerative Disorders
Dementias and Degenerative Disorders
Multiple System Atrophy
Multiple System Atrophy
Santhosh Gaddikeri, MD; Miral D. Jhaveri, MD, MBA
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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)
            • 2 distinct imaging subtypes: MSA-C and MSA-P

          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

                                          1. Chelban V et al: An update on advances in magnetic resonance imaging of multiple system atrophy. J Neurol. 266(4):1036-45, 2019
                                          2. Fanciulli A et al: Early distinction of Parkinson-variant multiple system atrophy from Parkinson's disease. Mov Disord. 34(3):440-1, 2019
                                          3. Krismer F et al: Morphometric MRI profiles of multiple system atrophy variants and implications for differential diagnosis. Mov Disord. 34(7):1041-8, 2019
                                          4. Meissner WG et al: Multiple system atrophy: recent developments and future perspectives. Mov Disord. 34(11):1629-42, 2019
                                          5. Miki Y et al: Improving diagnostic accuracy of multiple system atrophy: a clinicopathological study. Brain. 142(9):2813-27, 2019
                                          6. 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
                                          7. 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
                                          8. Fanciulli A et al: Multiple-system atrophy. N Engl J Med. 372(3):249-63, 2015
                                          9. Ramli N et al: Differentiating multiple-system atrophy from Parkinson's disease. Clin Radiol. 70(5):555-64, 2015
                                          10. Reiter E et al: Dorsolateral nigral hyperintensity on 3.0T susceptibility-weighted imaging in neurodegenerative Parkinsonism. Mov Disord. 30(8):1068-76, 2015
                                          11. Peeraully T: Multiple system atrophy. Semin Neurol. 34(2):174-81, 2014
                                          12. Wenning GK et al: Multiple system atrophy. Handb Clin Neurol. 117:229-41, 2013
                                          13. 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
                                          14. 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
                                          15. Matsusue E et al: Cerebellar lesions in multiple system atrophy: postmortem MR imaging-pathologic correlations. AJNR Am J Neuroradiol. 30(9):1725-30, 2009
                                          16. Köllensperger M et al: Red flags for multiple system atrophy. Mov Disord. 23(8):1093-9, 2008
                                          17. 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
                                          18. 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
                                          19. Mascalchi M et al: Proton MR spectroscopy of the cerebellum and pons in patients with degenerative ataxia. Radiology. 223(2):371-8, 2002
                                          20. Naka H et al: Characteristic MRI findings in multiple system atrophy: comparison of the three subtypes. Neuroradiology. 44(3):204-9, 2002
                                          Related Anatomy
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                                          Related Differential Diagnoses
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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)
                                                    • 2 distinct imaging subtypes: MSA-C and MSA-P

                                                  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

                                                                                  1. Chelban V et al: An update on advances in magnetic resonance imaging of multiple system atrophy. J Neurol. 266(4):1036-45, 2019
                                                                                  2. Fanciulli A et al: Early distinction of Parkinson-variant multiple system atrophy from Parkinson's disease. Mov Disord. 34(3):440-1, 2019
                                                                                  3. Krismer F et al: Morphometric MRI profiles of multiple system atrophy variants and implications for differential diagnosis. Mov Disord. 34(7):1041-8, 2019
                                                                                  4. Meissner WG et al: Multiple system atrophy: recent developments and future perspectives. Mov Disord. 34(11):1629-42, 2019
                                                                                  5. Miki Y et al: Improving diagnostic accuracy of multiple system atrophy: a clinicopathological study. Brain. 142(9):2813-27, 2019
                                                                                  6. 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
                                                                                  7. 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
                                                                                  8. Fanciulli A et al: Multiple-system atrophy. N Engl J Med. 372(3):249-63, 2015
                                                                                  9. Ramli N et al: Differentiating multiple-system atrophy from Parkinson's disease. Clin Radiol. 70(5):555-64, 2015
                                                                                  10. Reiter E et al: Dorsolateral nigral hyperintensity on 3.0T susceptibility-weighted imaging in neurodegenerative Parkinsonism. Mov Disord. 30(8):1068-76, 2015
                                                                                  11. Peeraully T: Multiple system atrophy. Semin Neurol. 34(2):174-81, 2014
                                                                                  12. Wenning GK et al: Multiple system atrophy. Handb Clin Neurol. 117:229-41, 2013
                                                                                  13. 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
                                                                                  14. 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
                                                                                  15. Matsusue E et al: Cerebellar lesions in multiple system atrophy: postmortem MR imaging-pathologic correlations. AJNR Am J Neuroradiol. 30(9):1725-30, 2009
                                                                                  16. Köllensperger M et al: Red flags for multiple system atrophy. Mov Disord. 23(8):1093-9, 2008
                                                                                  17. 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
                                                                                  18. 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
                                                                                  19. Mascalchi M et al: Proton MR spectroscopy of the cerebellum and pons in patients with degenerative ataxia. Radiology. 223(2):371-8, 2002
                                                                                  20. Naka H et al: Characteristic MRI findings in multiple system atrophy: comparison of the three subtypes. Neuroradiology. 44(3):204-9, 2002