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Diagnosing multiple system atrophy at the prodromal stage.

Chenjie Xia1, Ronald B Postuma2

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|April 2, 2020
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Summary

Developing prodromal criteria for multiple system atrophy (MSA) is essential for early diagnosis and treatment. Key features like REM sleep behavior disorder and autonomic dysfunction aid in identifying potential MSA cases, distinguishing them from Parkinson

Keywords:
Alpha-synucleinopathiesMultiple system atrophyProdromal diagnostic criteria developmentPure autonomic failure

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Area of Science:

  • Neuroscience
  • Neurology
  • Clinical Medicine

Background:

  • Early diagnosis of neurodegenerative disorders is critical for developing disease-modifying treatments.
  • Prodromal diagnostic criteria exist for Parkinson's disease (PD) and are developing for dementia with Lewy bodies (DLB).
  • Current diagnostic criteria for multiple system atrophy (MSA) improve accuracy but lack formal prodromal guidelines.

Purpose of the Study:

  • To propose features for identifying prodromal multiple system atrophy (MSA).
  • To differentiate prodromal MSA from Parkinson's disease (PD) and dementia with Lewy bodies (DLB).
  • To inform the development of future prodromal criteria for MSA.

Main Methods:

  • Reviewing clinical history, examination findings, and ancillary testing.
  • Identifying key clinical hallmarks of MSA, including REM sleep behavior disorder (RBD) and autonomic dysfunction.
  • Analyzing features that distinguish MSA from PD and DLB, such as olfaction, cognition, and respiratory symptoms.

Main Results:

  • REM sleep behavior disorder (RBD) and autonomic dysfunction are primary indicators for potential prodromal MSA.
  • Preserved olfaction, minimal cognitive deficits, and specific respiratory symptoms help differentiate MSA from PD and DLB.
  • Ancillary tests like neuroimaging and biomarkers can quantify the likelihood of phenoconversion to MSA.

Conclusions:

  • Prodromal criteria for MSA should leverage clinical features like RBD and autonomic dysfunction.
  • Distinguishing features are crucial for differentiating MSA from other synucleinopathies.
  • Future criteria may require an umbrella approach for prodromal alpha-synucleinopathies, specifying MSA later.