Difference between revisions of "Rapidly progressing dementias"
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* In addition to cognitive decline, they often have behavioral problems, pyramidal or extrapyramidal symptoms, EEG changes, and myoclonus. | * In addition to cognitive decline, they often have behavioral problems, pyramidal or extrapyramidal symptoms, EEG changes, and myoclonus. | ||
| − | |||
'''Categories''' (Salardini 2019) | '''Categories''' (Salardini 2019) | ||
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#Prion disease – misfolded prions are infectious proteins which cause devastating neurodegenerative illness | #Prion disease – misfolded prions are infectious proteins which cause devastating neurodegenerative illness | ||
##Creutzfeldt-Jakob disease (CJD) | ##Creutzfeldt-Jakob disease (CJD) | ||
| − | ###Most common form of prion disease | + | ###Most common form of prion disease |
| − | ###Presents with cognitive symptoms, pyramidal / extrapyramidal motor symptoms, ataxia, myoclonus, and psychiatric symptoms | + | ###Presents with cognitive symptoms, pyramidal / extrapyramidal motor symptoms, ataxia, myoclonus, and psychiatric symptoms |
##Fatal familial insomnia (FFI) | ##Fatal familial insomnia (FFI) | ||
| − | ###Presents with insomnia, dysautonomia, cognitive problems | + | ###Presents with insomnia, dysautonomia, cognitive problems |
##Gerstmann-Straussler-Schinker syndrome (GSS) | ##Gerstmann-Straussler-Schinker syndrome (GSS) | ||
| − | ###Cerebellar and/or extrapyramidal symptoms | + | ###Cerebellar and/or extrapyramidal symptoms |
| − | ###Slow progression over 10-20 years | + | ###Slow progression over 10-20 years |
##Kuru | ##Kuru | ||
##Variant CJD | ##Variant CJD | ||
| − | ###Caused by eating contaminated meat of livestock with bovine spongiform encephalopathy | + | ###Caused by eating contaminated meat of livestock with bovine spongiform encephalopathy |
#Atypical presentation of other primary dementias | #Atypical presentation of other primary dementias | ||
#Autoimmune diseases including autoimmune encephalitis | #Autoimmune diseases including autoimmune encephalitis | ||
Revision as of 08:04, 17 April 2022
Dementias which progress from normal cognition to severe dementia in less than 2 years (unofficial definition) (Salardini 2019).
- In addition to cognitive decline, they often have behavioral problems, pyramidal or extrapyramidal symptoms, EEG changes, and myoclonus.
Categories (Salardini 2019)
- Prion disease – misfolded prions are infectious proteins which cause devastating neurodegenerative illness
- Creutzfeldt-Jakob disease (CJD)
- Most common form of prion disease
- Presents with cognitive symptoms, pyramidal / extrapyramidal motor symptoms, ataxia, myoclonus, and psychiatric symptoms
- Fatal familial insomnia (FFI)
- Presents with insomnia, dysautonomia, cognitive problems
- Gerstmann-Straussler-Schinker syndrome (GSS)
- Cerebellar and/or extrapyramidal symptoms
- Slow progression over 10-20 years
- Kuru
- Variant CJD
- Caused by eating contaminated meat of livestock with bovine spongiform encephalopathy
- Creutzfeldt-Jakob disease (CJD)
- Atypical presentation of other primary dementias
- Autoimmune diseases including autoimmune encephalitis
- Secondary encephalopathies (toxic metabolic encephalopathy)
Imaging
DWI images of 2 patients with suspected CJD (Salardini 2019)
- In axial view of patient A there is characteristic increased cortical signal (cortical ribboning) suggesting cortical cytotoxic edema
- In axial view of patient B there is characteristic increased signal in caudate and mesial thalamus (hockey stick)
References
Salardini, A. An Overview of Primary Dementias as Clinicopathological Entities. Semin. Neurol. 39, 153–166 (2019). https://pubmed.ncbi.nlm.nih.gov/30925609/
