Heidenhain variant of sporadic Creutzfeldt-Jakob disease
Last revised by Dr Rohit Sharma ◉ on 08 Feb 2020
Citation, DOI & article data
Citation:
Gaillard, F., Sharma, R. Heidenhain variant of sporadic Creutzfeldt-Jakob disease. Reference article, Radiopaedia.org. (accessed on 28 May 2022) https://doi.org/10.53347/rID-63885
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rID:
63885
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3 times by 3 users - see full revision history
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Synonyms:
- Creutzfeldt-Jakob disease Heidenhain variant
- CJD Heidenhain variant
The Heidenhain variant of sporadic Creutzfeldt-Jakob disease is a distinct clinical manifestation of a number of molecular subtypes (MM1 and MM2C) dominated by pure visual disturbances early in the course of the disease 1. This is predictably correlated with prominent occipital involvement on MRI 1.
References
- 1. Baiardi S, Capellari S, Ladogana A, Strumia S, Santangelo M, Pocchiari M, Parchi P. Revisiting the Heidenhain Variant of Creutzfeldt-Jakob Disease: Evidence for Prion Type Variability Influencing Clinical Course and Laboratory Findings. (2016) Journal of Alzheimer's disease : JAD. 50 (2): 465-76. doi:10.3233/JAD-150668 - Pubmed
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
- midbrain to pons area ratio (for PSP)
- Magnetic Resonance Parkinsonism Index (MRPI) (for PSP)
- frontal horn width to intercaudate distance ratio (FH/CC) (for Huntington disease)
- intercaudate distance to inner table width ratio (CC/IT) (for Huntington disease)
- signs
- hummingbird sign (of PSP)
- Mickey Mouse sign (of PSP)
- 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
-
synucleinopathies
- diseases with Lewy bodies
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multiple systemic atrophy (MSA)
- Shy-Drager syndrome
- MSA-P (striatonigral degeneration)
- olivopontocerebellar atrophy (MSA-C)
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tauopathies
-
Alzheimer disease
- typical/classical Alzheimer disease
- variant (e.g. posterior cortical atrophy)
- chronic traumatic encephalopathy (CTE)
- corticobasal degeneration
- frontotemporal lobar degeneration (FTLD) (not all are tau)
- Pick disease
- progressive supranuclear palsy (PSP)
-
Alzheimer disease
- amyloidoses
- TDP-43 proteinopathies
- spinocerebellar ataxias
- Huntington disease
- hereditary spastic paraplegia
- clinically unclassifiable parkinsonism (CUP)
- Unverricht-Lundborg disease
-
prion diseases (not always included as neurodegenerative)
- Creutzfeldt-Jakob disease (sporadic, variant, familial, and iatrogenic)
- fatal familial insomnia
- Gerstmann-Straussler-Scheinker disease
- kuru
- variably protease-sensitive prionopathy
-
synucleinopathies
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