Stern-Garcin variant of sporadic Creutzfeldt-Jakob disease
Citation, DOI, disclosures and article data
At the time the article was created Rohit Sharma had no financial relationships to ineligible companies to disclose.
View Rohit Sharma's current disclosuresAt the time the article was last revised Rohit Sharma had no financial relationships to ineligible companies to disclose.
View Rohit Sharma's current disclosuresThe Stern-Garcin variant of sporadic Creutzfeldt-Jakob disease (CJD) is a phenotypical variant characterized by prominent early extrapyramidal symptoms, such as parkinsonism 1.
Radiographic features
MRI
MRI shows early involvement of the basal ganglia (striatum) and thalamus, in addition to other typical radiographic features of Creutzfeldt-Jakob disease 1,2. Some authors dichotomise this variant depending on if predominantly involving the basal ganglia (Garcin variant) or if predominantly involving the thalamus (Stern variant) 2, although the clinical implications of this differentiation are uncertain.
History and etymology
The phenotype was first described by K Stern in 1939 and then again by R Garcin and colleagues in 1962 3,4.
References
- 1. Chen T. Stern-Garcin Variant of Creutzfeldt-Jakob Disease. Neurohospitalist. 2021;11(3):270-1. doi:10.1177/1941874420977604 - Pubmed
- 2. Fragoso D, Gonçalves Filho A, Pacheco F et al. Imaging of Creutzfeldt-Jakob Disease: Imaging Patterns and Their Differential Diagnosis. Radiographics. 2017;37(1):234-57. doi:10.1148/rg.2017160075 - Pubmed
- 3. Stern K. Severe Dementia Associated with Bilateral Symmetrical Degeneration of the Thalamus. Brain. 1939;62(2):157-71. doi:10.1093/brain/62.2.157
- 4. Garcin R, Brion S & Khochneuis A. Le syndrome de Creutzfeldt-Jakob et lcs syndromes cortico-striks du presenium (a l’occasion de cinq observations anatomo-cliniques). RBv. Neurol. (Paris). 1962;106, 506-508.
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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)
- measurements and ratios
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- signs
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- morning glory sign (of PSP)
- hot cross bun sign (of MSA-C)
- hockey stick sign (of Creutzfeldt-Jakob disease)
- pulvinar sign (of Creutzfeldt-Jakob disease)
- 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
- typical/classical Alzheimer disease
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- Pick disease
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Alzheimer disease
- amyloidoses
- TDP-43 proteinopathies
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- Huntington disease
- hereditary spastic paraplegia
- clinically unclassifiable parkinsonism (CUP)
- Unverricht-Lundborg disease
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prion diseases (not always included as neurodegenerative)
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