Synucleinopathy
Citation, DOI, disclosures and article data
At the time the article was created Frank Gaillard had no recorded disclosures.
View Frank Gaillard's current disclosuresAt the time the article was last revised Frank Gaillard had the following disclosures:
- Radiopaedia Australia Pty Ltd and Radiopaedia Events Pty Ltd, Director, Founder and CEO (Radiopaedia) (ongoing)
- Biogen Australia Pty Ltd, Investigator-Initiated Research Grant for CAD software in multiple sclerosis: finished Oct 2021 (past)
These were assessed during peer review and were determined to not be relevant to the changes that were made.
View Frank Gaillard's current disclosures- Synucleinopathies
Synucleinopathies are a subgroup of neurodegenerative diseases, characterized by impairment of alpha-synuclein metabolism, resulting in abnormal intracellular deposits. They can further be divided into those with and those without the formation of Lewy bodies, although it should be noted that some overlap does exist 1,2,4:
- synucleinopathies with Lewy bodies
- Parkinson disease
- Dementia with Lewy bodies (aka Lewy body disease)
- synucleinopathies without Lewy bodies
Pathology
α-synuclein is a neuronal protein of unclear function which accounts for approximately 1% of brain weight 1.
In addition to cerebral accumulation, α-synuclein also accumulates in the gastrointestinal tract resulting in symptoms 3.
Editor: more detail is required here pertaining to the genetic and biochemical underpinnings of these diseases, as well as any commonalities in presentation/demographics/ radiographic appearances.
Quiz questions
References
- 1. Kornienko VN, Pronin IN. Diagnostic Neuroradiology. Springer Verlag. (2008) ISBN:3540756523. Read it at Google Books - Find it at Amazon
- 2. Taylor JP, O'Brien J. Neuroimaging of dementia with Lewy bodies. Neuroimaging Clin. N. Am. 2012;22 (1): 67-81, viii. doi:10.1016/j.nic.2011.11.001 - Pubmed citation
- 3. Kunal P. Patel, David T. Wymer, Vinay K. Bhatia, Ranjan Duara, Chetan D. Rajadhyaksha. Multimodality Imaging of Dementia: Clinical Importance and Role of Integrated Anatomic and Molecular Imaging. (2020) RadioGraphics. 40 (1): 200-222. doi:10.1148/rg.2020190070 - Pubmed
- 4. Jellinger K. More Frequent Lewy Bodies but Less Frequent Alzheimer-Type Lesions in Multiple System Atrophy as Compared to Age-Matched Control Brains. Acta Neuropathol. 2007;114(3):299-303. doi:10.1007/s00401-007-0227-4 - Pubmed
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Related articles: Neurodegenerative diseases
Neurodegenerative diseases are legion and their classification just as protean. A useful approach is to divide them according to underlying pathological process, although even using this schema, there is much overlap and thus resulting confusion.
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neurodegenerative MRI brain (an approach)
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- scoring systems
- measurements and ratios
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neurodegenerative diseases
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synucleinopathies
- diseases with Lewy bodies
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multiple systemic atrophy (MSA)
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tauopathies
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Alzheimer disease
- amyloidoses
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- spinocerebellar ataxias
- Huntington disease
- hereditary spastic paraplegia
- clinically unclassifiable parkinsonism (CUP)
- Unverricht-Lundborg disease
-
prion diseases (not always included as neurodegenerative)
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synucleinopathies